INTRODUCTION

Soft tissue is defined as a complex of non-epithelial extra-skeletal structures of the

body exclusive of the reticulo-endothelial system glia and supportive tissue of the various
parenchymal organs.

Soft tissue tumors are highly heterogenous group of tumors that are classified on a
histogenetic basis they are broadly divided in to 2 types Benign and malignant types.

Benign tumors far outnumber the malignant ones.1

For any soft tissue tumor initial diagnostic procedure is to obtain material through fine
needle aspiration. For a definitive histopathological diagnosis excision biopsy is mandatory
FNAC has a definite role in forming the initial diagnosis of soft tissue tumors, while
histopathology with the aid of immuno markers provides the final diagnosis.2

Embryologically soft tissue tumors is derived principally from mesoderm with some
contrbution form neuroectoderm.1

Soft tissue sarcomas compared with other carcinomas are relatively rare constitute
less than1% of all cancer.3

They present most commonly as an asymptomatic mass originating in an extremity

but can occur anywhere in the body, particularly trunk, retroperitoneum and the head and
neck.4

Benign tumors more closely resembles normal tissue, have limited capacity for
autonomous growth. Most common type of benign tumor is lipoma followed by
hemangiomas, benign peripheral nerve sheath tumor and fibroma. Benign tumor far
outnumber the malignant ones by 100:1.
Most common malignant soft tissue tumors are undifferentiated pleomorphic sarcoma
followed by leiomyosarcoma, liposarcoma, synovial sarcoma, and malignant peripheral
nerve sheath tumour.4

The most common age for beningn tumors are 4 th and 5th decades and for malignant tumors lst
and 2nd. Maximum number of tissue tumor occurs in extremities. Benzabih M also fund lower
extremity as the most common site soft tissue tumor followed by upper extremity.5

Soft tissue tumors occur more commonly in males but gender and age related incidence vary
among the histologic types. Rhabdomyosarcoma is the most common soft tissue sarcoma of
6
children and adolescent while undifferentiated pleomorphic sarcomas is predominantly
tumor of old age.

Benign tumors are locally invasive while malignant soft tissue tumors present most
common site of metastasis is lungs.4

High grade tumors are treated with radical surgery or chemotherapy and radiation,
whereas low grade sarcomas are usually treated whit surgical excision alone.

Hence the present study was undertaken to assess the histopatholoical pattern of soft
tissue tumors using routine, special and wherever warranted immunohistochemical stains and
to classify soft tissue tumors according to new world health Organization classification 2013.
 AIMS AND OBJECTIVES OF THE STUDY

The aim of this study is to know the following features of soft tissue tumors.

 Age incidence
 Sex incidence
 Site-specific distribution
 Most common type of soft tissue tumor
 Various spectrum of soft tissue tumor both malignant and benign.
 Grading and Staging of malignant soft tissue tumors
 REVIEW OF LITERATURE

HISTORICAL ASPECTS

Soft tissue lesions have been recognized since the time of the Egyptians.

Hippocrates (460-375BC) documented superficial and deep-seated tumors in the

extremities and Galen (AD 131-200) described sarcoma as fleshy excrescence reminiscent of
raw meat ‘sarkos’7

Virchow established ‘cellular pathology’ and coined the phrase Omnis cellula e cellula, that
is, ‘each cell stems from another cell’, and he distinguished sarcomas from carcinomas. 7

The term “Soft cancer” was defined by wardrop for haematodes in 1782.7

Abernethy suggested that tumor should be named according to their anatomic site and
offered the first classification of sarcomas in 1809.7

Weber in 1854, was the first person to describe “Rhabdomyosarcoma” 8

In1845 Lebert published an atlas illustrating, the first time microscopic appearance of
sarcomas of soft tissues7

Stout and Murray in 1942 described “Haemangiopericytoma” for the first time.9

At the Rockefeller institute in new York city, Rous and his associates produced
sarcoma from cell-free infiltrate established the same “Rous and his associates long term
tissue culture, and succeeded in growing the first human sarcoma in vitro7

In the 20th century the surgical pathology of SST underwent further development in
the USA. Ewing first described a peculiar bone tumor, now called Ewing sarcoma, as an
‘endothelioma of bone’ or ‘endothelial myeloma’ ,because he believed that it arose from the
blood vessels of bone tissue. In addition, Ewing recognized that sarcomas were derived from
mesenchymal tissues.10
Stout and Lattes described the clinicopathological characteristics of many STT in a
monograph published by the Armed Forces Institute of Pathology (AFIP). Enzinger and
Weiss performed comprehensive studies of STT and establish the histological pattern
recognition for the diagnosis of soft tissue tumours.10

Soft tissue tumor have been are a large and heterogenous group of neoplasm.
Traditionally tumor have been classified to histogenetic features. The explosion of
cytogenetics and molecular genetics information in this field over the past 10-15 years had
significant impact on soft tissue tumors classification and also on our understanding of their
biology.11

Benign tumors have been treated with surgical excision alone.

In case of malignant tumors prior to the 1970s, surgery was the primary treatment,
and this was associated with a poor prognosis and high mortality rates in most patients.
However, since the mid-1970s, a multimodal approach comprising of radiation therapy,
chemotherapy and surgery have helped in improving prognosis in these patients by increasing
long-term survival.11

EMBRYOLOGY

Embryologically, soft tissue tumors are derived from mesoderm with some
contribution from neuroectoderm.

Fertilized Ovum
AETIO PATHOGENESIS

Like many other malignant tumors, the pathogenesis of most tissue tumors is still unknown.

Various physical and chemical factors, exposure to ionizing radiations, inherited or

acquired immunologic defects, trauma, fractured site, vicinity of plastic or metallic implant
usually after a latent period of several years.12

External radiation therapy is a well established risk factors for tissue sarcoma.

Post irradiation sarcomas are seen in about 0.1%of cancer patients who survive 5
years. The criteria defined are:

1) Sarcoma should develop in the irradiated field

2) A period of latency of at least 3years
3) Histological confirmation of diagnosis.

Wingren et al.,14 studied the association between soft tissue sarcoma and occupational
exposure in 96 cases. They have observed increased risk for STS in gardeners railroad
workers, construction works with exposure to asbestos and unspecified chemical workers.
They concluded that a strong effect is seen in gardeners.

Human herpes virus 8 plays a key role in the development of Kaposi sarcoma and the
clinical course is dependent on the immune status of the patient.15
 Several types of benign soft tissue have been reported to occur on a familial or
inherited basis.15 The genetic alteration that allow a clone of cells to acquire the hallmark
properties of cancer affect three broad types of cancer genes, oncogenes tumor suppressor
gene and caretaker genes. Activation of oncogenes occurs by mutation that alter the gene
sequence of protein which results in aberrant enhanced function by an increase in number of
copies (gene amplification),by translocation or by combination of examples of mesenchymal
tumour include KIT and PDGFRA both activated by mutations, MYCN and MDM2 activated
by gene amplification. PLAG1and HMGA1 activated by translocation (also known as fusion
oncogenes)

INCIDENCE

Benign soft tissue tumors such as lipomas and benign fibrous histiocytomas are the most
common neoplasms in human beings.

The annual incidence of soft tissue sarcoma is around 30/million which is less than 1% all
malignant tumors. Kransdorf17,18 in 1995 citing record from AFIP reported a ratio of 1:5:1 and
pointed out at inherent bias in a referral population is because of the consultative and difficult
nature of case material.

AGE INCIDENCE

Soft tissue tumors can occur at any age. It has been noted that the histological distribution of
soft tissue tumors are rather specific for a particular age group at a particular anatomical site.

In myhre jenson the age group varied from 0-80 years. The age range for benin tumors was
from 20-69 years while that for malignant tumors was 20-79 year with exception of
embryonal and alveolar rhabdomyosacroma majority of which is less than 10 years of age19.

SEX INCIDENCE

There seem to be a slight male preponderance especially in malignant soft tissue tumors. In
myhre jenson series the M.F ratio was 2:119. In kransdorf’s studies it was 1.29:1.1718.

Lipomas were the commonest off all the benign tumors in adults in myhre jenson 19 and
kransdorf’s series. Haemangiomas were the commonest benign STT in childhood.
SITE SPECIFIC DISTRIBUTION

According to Kransdorf’s study, the incidence of various tumors at various anatomical

locations varies with age. For example, haemangiomas are the commonest tumors in the hand
and wrist region, while giant cell tumors of tendon sheath were common in the same site in
adults17.

PATHOPHYSIOLOGY

Generally, soft tissue tumors grow centripetally, although some benign tumors, such as
fibrous lesions, may grow longitudinally along tissue planes. Most respect fascial
boundaries, remaining confined to the compartment of origin until the later stages of
development when it is likely to breach compartmental boundaries.

Major neurovascular structures usually are displaced as opposed to being enveloped or

invaded by tumor. Tumors arising in extra compartmental locations, such as the popliteal
fossa, may expand more quickly because of a lack of fascial boundaries; they are also more
likely to involve neurovascular structures. The peripheral portion of the tumor compresses
surrounding normal soft tissue because of centripetal expansile growth. These results in the
formation of a relatively well defined zone of compressed fibrous tissue potentially
containing scattered tumor cells, inflammatory cells and demonstrate neovascularity. A thin
layer of tissue called the reactive zone surrounds the compression zone and together with the
reactive zone forms a pseudocapsule that encloses the tumor.20

A. Local Recurrence

Soft tissue sacromas have the propensity to recur locally and most tumors destined to

recur do so within the first 2-3 years. The pseudocapsule provides surgeons with more or less
obvious plane of dissection; however, such an excision can leave behind microscopic or
occasionally gross tumor. This may lead local recurrences in up to 80% of patients.21

B.Distant Metastatis

Dominant path of metastatic of sarcoma is through haematogenous route. The first step in the
sequence leading to the formation of blood-borne metastatic is intravasation. This is followed
by embolism and then by arrest of the malignant emboli. The lung is by far the most common
 site of metastasis, involved in up to 52% of patients with high-grade lesions, followed by
liver, bone and brain.22

CLINICAL FEATURES

Most benign tumors are located in superficial (dermal of subcutaneous) soft tissue. The most
frequent benign STT is lipoma, which often goes untreated. Most soft tissue sarcomas of the
extremities and trunk present as painless accidently observed mass, which usually do not
influence general health of limb function. However, it may cause pain or neurologic
symptoms by compressing or stretching nerves or by irritating overlying bursae. Superficial
soft tissue lesions larger than 5 cm in size and all deep seated tumors (irrespective of size)
should arouse the suspicion of a malignancy. A rapid rate of increase in the size of a mass
should also arouse suspicion that the lesion is malignant.

DIAGNOSIS OF SOFT TISSUE TUMORS

Both benign and malignant soft tissue tumors commonly present as a painless mass.

When a soft tissue mass arises in a patient with no history of trauma or when a mass persisting even
after 6 weeks after a local trauma a biopsy is indicated. Fine needle aspiration cytology has a
role play in the diagnosis of soft tissue lesions guided by CT Scans in intra-abdominal and
retroperitoneal lesions. FNAC is very useful to document local resources of metastatsis in a
previously diagnosis soft tissue tumors. 24

A core biopsy, an excisional biopsy and an incisional biopsy are the other technique used for
diagnosing most soft tissue masses.

Radiological evaluation of soft tissue tumors utilizes the trial of

1) Conventional radiography
2) CT Scan
3) MRI

H & E stained sections, ancillary methods such as special stains, IHC and EM studies are
helpful for making accurate diagnosis.

The combination of clinical, histopathological, cytogenetics and molecular analysis will not
only facilities the precise diagnosis of soft tissue tumor but also promote the development of
targeted therapy for specific types of sarcoma. 25
The advent of multimodal therapy has made it possible to avoid radical surgery and decrease
the morbidity while substantially improving the 5 year survival rates in malignant soft tissue
tumors. 26

The WHO Classification of Soft Tissue Tumors 201327

Adipocytic tumors

Benign

Lipoma

Lipomatosis

Lipomatosis of Nerve

Lipoblastoma / Lipoblastomatosis

Angiolipoma

Myolipoma of soft tissue

Chondroid lipoma

Extra-renal angiomyolipoma

Extra-Adrenal myolipoma

Spindle cell / pleomorphic lipoma

Hibernoma

Intermediate (Locally aggressive)

Atypical lipomatous tumor / well differentiated liposarcoma

Malignant

Dedifferentiate liposarcoma

Myxoid Liposarcoma

Pleomorphic Liposarcoma
Liposarcoma, not otherwise specified

General Changes

The current classification no longer includes mixed-type liposarcoma. Cases previously

diagnosed as mixed-type liposarcoma were almost always discovered to fall within a specific
type of Liposarcoma when subjected to molecular and genetic testing.

Atypical lipomatous tumour (ALT)

ALT is divided into 3 main subgroups (rather than 4 as done previously):

 Adipocytic (Lipoma-Like)
 Sclerosing
 Inflammatory types

A description of spindle cell liposarcoma still appears in the text. The lack of MDM2
immunopositive or 12q15 amplification in this tumor type suggests spindle cell liposarcoma
represents a separate group.

Fibroblastic / Myofibroblastic tumors

Benign

Nodular fasciitis

Proliferative fasciitis

Proliferative myositis

Myositis ossifficans

Fibro-osseous pseudotumor of digits

Ischemic fasciitis

Elastofibroma

Fibrous hamartoma of infancy

Fibromatosis colli

Juvenile hyaline fibromatosis

Inclusion body fibromatosis

Fibroma of tendon sheath

Desmoplastic fibroblastoma

Mammary-type myofiroblastoma

Calcifying aponeurotic fibroma

Angiomyofibroblastoma

Cellular angiofibroma

Nuchal-type fibroma

Gardner fibroma

Calcifying fibrous tumour

Intermediate (Iocally aggressive)

Palmar/plantar fibromatosis

Desmoids-type fibromatosis

Lipofibromatosis

Giant cell fibroblastoma

Intermediate (rarely metastasizing)

Dermatofibrosarcoma protuberans

Fibrosarcomatous dermatofibrosarcoma protuberans

Pigmented dermatofibrosarcoma protuberans

Solitary fibrous tumor

Solitary fibrous tumor, malignant

Solitory fibrous tumor,malignant

Inflammatory myofibroblastic tumour

Low grade myofibroblastic sarcoma

Myxoinflammatory fibroblastic sarcoma\Atypical myxoinflammatory

Fibroblastic tumour

Infantile fibrosarcoma

Malignant

Adult fibrosarcoma

Myxofibrosarcoma

Low-grade fibromyxoid sarcoma

Sclerosing epithelioid fibrosarcoma

Extrapleural solitary fibrous tumour

The term “heamangiopericyrtoma” was abandoned it is used only describe a morphological

pattern that is shared by different pattern that is shared by different entities. Currently, solitary
fibrous tumour, heamangiopericytoma, lopomatous heamongiopericytoma and giant cell
angiofibroma are all lumped under the “extra pleural solitary fibrous tumour” category.

So-called fibrohistocytic tumours

Benign

Tenosynovial giant cell tumour

Localized type

Diffuse type

Malignant

Deep benign fibrous histiocytoma

Intermediate(rarely metastasizing)
Plexoform fibrohistiocytic tumour

Giant cell tumour of soft tissue

Smooth-muscle tumours

Benign

Leiomyoma of deep soft tissue

Malignant

Leiomyosarcoma(excluding skin)

Angioleiomyoma was reclassified under pericytic (perivascular) tumours.

Pericytic ( perivascular)tumours

 Glomus tumour(and variants)

 Glomangiomatosis
 Malignant glomus tumour
 Myopericytoma
 Myofibroma
 Myofibromatosis

Angioleiomyma

Myofibroma and myofibromatosis were reclassified under myopericytoma instead of

fibroblastic\myofibroblastic tumours.

Skeletal-muscle tumours

 Rhabdomyoma
 Embryonal rhabdomyosarcoma
 Alveolar rhabdomyosarcoma
 Plemorphic rhabdomyosarcoma
 Spindle cell\sclerosing rhabdomtysarcoma
 Spindle cell\ sclerosing rhabdomyosarcoma was felt to now be well enough recognized and
defined to be added to this group.
Vascular tomours

Benign

Heamangioma

Synovial

Venous

Arteriovenous heamangioma\malformation

Epithelioid heamangioma

Angiomatosis

Lymphangioma

Intermediate (locally aggressive)

Kaposiform heamongioendothelioma

Intermediate (rarely metastasizing)

Retiform heamongiondothelioma

Papillary intralymphatic angioendo thelioma

Composite hemangeoendothelioma

Pseudomyogenic(epithelolioid sarcoma-like) heamangioendotheliioma

Kapsoi sarcoma

Malignant

Pseudomyogenic (epitheloid sarcoma-like) heamongioendothelioma was added to the

intermediate (rarely metastasizing) subgroup.

Gastrointestinal stromal tumours

  Benign gastrointestinal stromal tumour
 Gastrointestinal stromal tumour, uncertain malignant potential
 Gastrointenstinal stromal tumour,malignant

This is a major and important addition to the new 2013 classificataion.

Nerve sheath tumours

Benign

Schwannoma(including variants)

Melonotic schwannoma

Neurofibroma(including variants)

Plexiform neurofibroma

Perineurioma

Malignant perineurioma

Granular cell tumour

Dermal nerves sheath myxoma

Solitary circumscribed neuroma

Ectopic meningioma

Nasal glial heterotopias

Benign triton tumour

Hybrid nerve sheath tumours

Malignant

peripheral nerve sheath tumour

Epitheliloid malignant nerve sheath tumour

Malignant triton tumour

Malignant granular cell tumour

Ectomesenchymoma

It was previously included with the 2007 WHO classification of tumours of the central nervous
system. The current WHO classification of tumours previously classified under cranial and
peripheral nerves, head and neck and skin tumours. This represents an important initiative to
present the diverse family of mesenchymal tumours into a single reference source. The 2007
classification did not include the following:

 Granular cell tumour

 Dermal nerve sheath myxoma
 Solitary circumscribed neuroma
 Ectopic meningioma\meningiothelial hamartoma
 Nasal glial heterotopias
 Benign triton tumour
 Hybrid nerve sheath tumours
 Malignant triton tumour
 Malignant granular cell tumour
 Ectomesenchymoma

Tumours of uncertain differentiation

Benign

Acral fibromyxoma

Intramuscular myxoma(including cellular variant)

Juxta-articular myxoma

Deep(“aggressive”) angiomyxoma

Pleomorphic hyalinizing angiectatic tumour

Ectopic harmartomatous thymoma

Intermediate(locally aggressive)

Heamosiderotic fibropomatous tumour

Intermediate(rarely metastizing)

Atypical fibroxanthoma

Angiomatoid fibrous histocytoma

Ossifying fibromixoid tumour

Ossifying fibromyxoid tumour, malignant

Mixed tumour NOS

Mixed tumour NOS, malignant

Myoepithelioma

Myoepithicalcarcinoma

Phosphatoric mesenchymal tumour, benign

Phosphaturic mesenchymal tomour, malignant

Malignant

Synovial sarcoma NOS

Synovial sarcoma, spindle cell

Synovial sarcoma ,bhipasic

Epitheloid sarcoma

Alveolar soft-part sarcoma

Clear cell sarcoma of soft tissue

Extraaskeletal myoxoid chonadrosarcoma

Extraaskeletal ewing sarcoma

Desmoplastic small round cell tumour

Extra-renal rhabdoid tumour

Neoplasms with perivascular epithelilod cell differentiation(PEComa)

PEComa NOs,benign

PEComa NOS,malignant

Intimal sarcoma

Undifferentiated\unclassified sarcomas

 Undifferentiated spindle cell sarcoma

 Undifferentiated round cell sarcoma
 Undifferentiated pleomorphic sarcoma
 Undifferentiated epitheliloid sarcoma
 Undifferentiated sarcoma NOS

This category was added to encompass a group of malignant tumours that were previously included in
the fibrohistiocytic tumours, namely “malignant fibrous histiocytomas”. These tumours lack a
specifically indentified line of differentiation when analyzed by presently available technology.
Dedifferentiated types of specific sarcomas are not included in this category. Undifferentiated \
unclassified sarcomas account for up to 20% of all sarcomas and about a quarter of these are radiation
–associated tumours.

Histologic grading

Histologic grade indicates the probability of distant metastasis and overall survival, but is of poor
value for predicting local recurrences which is mainly related to the quality of surgical margins.

The two most widely used grading systems are NCI(united states national cancer inistitute)
System and the FNCLCC (French Federation National edes Centres de lute control cancer)system.

The NCI system uses a combination of histological type, cellularity, pleomorphism,

tumor necrosis and mitotic rate for +attributing grade 1 to 3.

The FNCLCC system is based on a score obtained by evaluating three parameters:

tumor differentiation, mitotic rate and amount of tumor necrosis. A score of 1 to 3 is
attributed independently to each parameter and the grade is obtained by adding the three
scores.

FNCLCC grading system: definition of parameters

Tumor differentiation

Score 1 :Sarcomas closely resembling normal adult mesenchymal tissue (e.g., low grade
leiomyosarcoma).

Score 2 :

Sarcomas for which histological typing is certain (e.g., myxoid liposarcoma).

Score 3 :

Embryonal and undifferentiated sarcomas, sarcomas of doubtful type, synoial sarcomas,

osteosarcomas, PNET.

Mitotic count

Score 1 : 0-9 mitoses per 10 HPF*

Score 2 : 10-19 mitoses per 10 HPF

Score 3 : 20 mitoses per HPF

Tumour necrosis

Score 0 : no necrosis

Score 1 : <50% tumour necrosis

Score 2 : > 50% tumour necrosis

Histological grade

Grade 1 : total score -2,3

 Grade 2 : total score – 4,5

Grade 3 : total score – 6,7,8

The major staging system used for STS was developed by the International Union against
Cancer (UICC) and the America Joint Committee on Cancer (AJCC) and appears to be
clinically useful and of prognostic value. This TNM system incorporates histological grade
as well as tumour size and depth, regional node involvement and distant metastasis. It
accommodates 2, 3,4-tiered grading systems.

AJCC staging of soft tissue sarcomas

Primary tumour (T) TX : Primary tumour cannot be assessed

T0 : no evidence of primary tumour

T1 : Tumour >= 5cm in greatest dimension

T1a : Superficial tumour*

T2b : Deep tumour

T2 : Tumour >5cm in greatest dimension

T2a : Superficial tumour

T2b : Deep tumour

Regional lymph nodes (N) NX: Regional lymph nodes cannot be

assessed

N0 : No regional lymph node metastasis

N1 : Regional lymph node metastasis

Note: Regional node involvement is rare and cases in which nodal status is not assessed either clinically or
pathologically could be considered N0 instead of NX or pNX.

Distant metastasis (M) M0: no distant metastasis

M1: distant metastasis

Histopathological Grading

Translation table for three and four grade to two grade (low vs. high grade) system

TNM two grade system Three grade system Four grade systems

Low grade Grade 1 Grade 1

High grade Grade 2 Grade 2
Grade 3 Grade 3
Grade 4
Stage IA T1a N0, NX Low grade

M0 Low grade

Stage IB T1b N0, NX Low grade

M0 Low grade

Stage IIA T2a N0, NX High grade

M0 High grade

Stage IIB T2b N0, NX High grade

Stage III M0 High grade

Stage IV T1a N0, NX Any grade

M0 Any grade

T1b N0, NX

M0

T2a N0, NX

M0
 T2b N0, NX

M0

Any T N1

M0

Any T Any N

M1

LIMITATIONS

Despite the fact the there is widespread use of some form of grading system in the
diagnosis and management of sarcomas experts believe that no grading system.
Performs well on every type of sarcoma. There are sarcomas in which histologic subtypes
essentially defines behavior and therefore grade becomes redundant, and it is best illustrated
by well differentiated liposarcoma , and inherently low grade, non metastasizing lesion and
the majority of round cell sarcomas (example alveolar rhabdomyosarcoma) which are
inherently high grade.30

Grading furthermore plays no role in distinguishing benign and malignant lesions and
this distinction should always be made first, some benign and reactive lesions may posses
certain features like mitotic activity that are also prevalent in sarcomas.

Prognostic value

Kettan and colleagues from the memorial sloan, Kettering cancer centre (MSKCC)
have utilized a database of over 200 prospectively followed adult patients with soft tissue
sarcoma to predict the probability of sarcoma-specific death by 12 years. It considers the size
of tumour, depth, site, histology and age of the patient.

The overall relative 5-years survival rate of people with soft tissue sarcomas is around
50% according to statistics from the National Cancer Institute (NCI). These statistics
include people with Kaposi sarcoma, which has a poorer outlook than many sarcomas. The
NCI doesn’t use the AJCC staging system. Instead, they group sarcomas only by whether
they are still confined to the primary site (called localized) have spread to nearby lymph
nodes or tissues “(called regional); or have spread (metastasized) to sites away from the main
tumor (called distant).31
FIBROUS TUMORS

Fibrous tumors are categorized in 3 broad groups.

I. Benign tumors

Nodular fasciitis

Clinically, these tumors mimic like malignant tumors. They are benign proliferation of
fibroblasts, which have an ability to simulate malignant process. Trauma has been implicated
as a possible causative factor.32

It is more common in adults between 20-40 years of age, without any sex predilection.
There is a distinct predilection for the upper extremities, especially for the flexor aspect of
forearm.

Macroscopically, it is a well-circumscribed mass measuring less the 2 mm. on

microscopy, tumor is composed of plump fibroblasts, which are arranged in short bundles.
The cells have pale nuclei and prominent nucleoli. The intervening matrix is rich in
mucopolysaccaride.33

Bernstein et al., (1982)34 published an article on nodular fasciitis after clinic

pathological study of 134 cases and described 4 types . the reactive type, densely cellular
type, those with osteoid or cartilaginous metaplasia and proliferative fasciitis.

Fibroma of tendon sheath

These tumors are commonly seen in males with a predilection for extremities,
affected chiefly the tendons and tendon sheaths of the fingers (49%), hands (21%), and wrist
(12%). The main presenting symptom was an insidiously growing mass causing mild
tenderness or pain in about one-third of the patients.35

Nuchal fibroma

It is a fibrous growth occurring in the interscapular and Para spinal region.

Myositis ossificans

It is a localized self-limiting lesionthat follows mechanical trauma in 50% of the

cases.

It affects young, active adolescents with no sex predilection.

The favoured sites are the llimbs, quadriceps and gluteal muscles in the lower limbs.

On gross, they are well circumscribed mass measuring 3-6 cm with a stony sensation.

Microscopy reveals innermost portion consisting of richly vascular fibroblastic tissue

admixed with macrophages and multinucleate giant cells.

The intermediate zone shows admixture of fibroblasts, osteoblasts and variable

osteoid.

The periphery shows calcification and mature lamellar bone’

It is a benign self-limiting lesion.

Desmoplastic fibroblastoma

It is also known as collagenous fibroma occurring in adults, showing no tendency for local
recurrence. It is characterized by a hypocelular proliferation of bipolar or stellate fibroblasts.

Angiomyo fibroblastoma

This tumor shows a predilection fro the vulvovaginal region during the reproductive
years but which may also occur in the inguinoscrotal region in men. It is characterized by
greater cellularity, vascularity and virtual absence of recurrent potential.

Cellular angiofibroma

This tumor occurs in adults of either sex, principally in vulvovaginal and inguinoscrotal
regions.\

Cellular tumor with fascicles or haphazard pattern, Bland spindle cells with scant,
lightly eosinophilic cytoplasm with ill defined borders, oval to fuisform nucleus.37
Fibromatosis

These are benign fibrous tissue proliferations. They are intermediate in between that
of benign fuibroblstic lesions and fibrosarcoma, which is known for local recurrences but
never metastasize.38

Hereditary, trauma and hormones play a major role.

Superficial fibromatosis are small, slowly growing and of small size and arise from
the fascia or aponeurosis. Deep fibromatiosis are large, more rapidly growing tumours.

Superficial fibromatosis is seen in adults between 30 and 60 years with a male

predilection.

Intermediate (rarely metastasizing)

Haemangiopericytoma

This tumor was believed as tumor of capillary pericytes. Instead they are fibroblastic
in nature and hence the name ‘solitary fibrous tumor’. They from 1.1% of malignant soft
tissue tumors in Kransdorf’s et al., (1995) series.

These are more common in adult life with no sex predilection and are seen more
commonly in the lower extremities.39

On gross, the tumor appears as a well-circumscribed mass measuring 4 to 8 cm in

diameter.

Microscopy reveals thin walled endothelial lined vascular channels ranging from
small capillary sized vessels to large gaping sinusoidal spaces. The cells are round to oval
and are tightly packed around blood vessels. The number of mitotic figures Is a helpful
criteria in prediciting the behavior. 4 or more / 10 HPF is usually indicative of rapid growth.
Mac Master et al., reported 60 patients with Haemangiopericytoma and observed metastasis
in more than 50% of cases.40

III. Malignant Fibrous tumors

Adult fibrosarcoma
After reviewing earlier diagnosis, it ranked 3 rd following liposarcoma and
rhabdomyosarcoma. Radiation therapy, thermal, injury, plastic repair and prosthetic
implantation are the known causative factors.

It is more common between 30 and 55 years with a slight male preponderance.

Proximal extremities especially, thigh, trunk and distal extremities are the commonest sites.

On gross examination, the tumor appears as a solitary, encapsulated fleshy or

lobulated mass.

Microscopy reveals uniform spindle cells with pale nucleus and scanty cytoplasm
arranged in herringbone pattern.

Fibrosarcoma metastasize through blood stream commonly to the hung.

On gross, it is a multinodular grey white mass.

Microscopy reveals pleomorphic cellular areas with abundant myxomatous matrix

accentuating the thick blood vessels.43

Microscopy reveals small rounded immature looking cells with focal fibroblastic
differentiation.

Low-grade fibromyxoid sarcoma

These tumors are common in younger patients, most often in the limbs and which,
despite bland swirling morphology, have a significant long time risk of distant metastasis.

FIBROHISITOCYTIC TUMORS

Benign tumors

Tenosynovial giant cell tumour

Localized type

The term “Giant cell tumour of tendon sheath “encompasses a family of lesions most
often arising from the synovium of joints, bursae and tendon sheaths.44

The localized from is frequent and the most common subset of giant cell tumours.44
Tumours are lobulated, well circumscribed and at atleast partially covered by a fibrous
capsule. Their microscopic appearance is variable, depending on the proportion of
mononuclear cells, multinucleate giant cells, foamy macrophages, siderophages and the
amount of stroma.45

Deep benign fibrous histiocytoma

Benign fibrous histiocytoma usually affects adults. The tumors arise from the skin and
subcutaneous tissue of the face, neck, trunk, and lower limbs. They are painless, slowly
growing, small lumps, firm in consistency and occasionally vascular.47

Grossly the tumors are yellowish in color with few cystic areas.

Microscopy reveals a mixture of fibroblasts and histiocyte like cells arranged in vague
storiform pattern accompanied by variable number of inflammatory cells.

Plexiform Fibrohistiocytic Tumors

Plexiform fibrohistiocytic tumour is a rarely metastasizing dermal-subcutaneous

neoplasm composed of fibroblast and histiocyte-like cells.48

Plexiform fibrohistiocytic tumour most commonly involves the upper extremities

especially forearm, followed by the lower extremity, trunk, and head and neck.48

Giant cell tumors of soft tissue

Giant cell tumour of soft tissue is a primary soft tissue neoplasm that is clinically and
histologically similar to giant cell tumor of bone, it very rarely metastasizes. 49

GCT-ST occurs predominantly in the fifth decade of life.49

GCT-ST usually occurs in superficial soft tissues of the upper and lower extremities
(70% of tumors.) GCT-ST displays a striking multinodular architecture (85%), with nodules
ranging in size up to 15mm. the nodules are composed of a mixture of round to oval
mononuclear cells and osteoclast-like giant cells, with both cell type immersed in a richly
vascularized stroma.

Pleomorphic sarcoma is the alternate name advocated by the WHO to replace the
current name as it gives a more accurate description of the tumor and does not imply the
origin of the tumor cells.50
LIPOMATOUS TUMORS

I. Benign

Lipoma

Lipomas are adipose tumors that are often located in the subcutaneous tissues of the
head, neck, shoulders, and back. Lipomas have been indentified in all age groups but usually
first appear between 40 and 60 years of age. These slow-growing, nearly always benign,
tumors usually present as nonpainful, round, mobile masses with a characteristic soft, doughy
feel.51

Rarely, lipomas can be associated with syndromes such as hereditary multiple

lopoomatosis, adiposis dolorosa, Gardner’s syndrome, and Madelung’s disease. There are
also variants such as angiolipomas, pleomorphic lipomas, spindle cell lipomas, and
chondrolipomas.51

Clinically, the tumour presents as a well circumscribed, encapsulated slowly growing,

soft, mobile tumour

The tumour varies in size from a few millimeters to 20 cms or more in diameter.

Hibernoma

Hibernoma is a rare tumour composed of brown adipose tissue, and represents less
than 2% of benign lipomatous tumours. This tumor was first described by Merkel in 1906 as
a “pseudolipoma” and it was later named “hibernoma” in 1914.52

Macroscopically, it is a well-circumscribed, lobulated mass. Microscopy reveal

variable amounts of mature, white adipocytes as well as three characteristic brown fat cell
types including a granular eosinophilic cell with or without lipid vacuoles.52

Extrarenal angiomyolipoma

These are benign neoplasms of the kidney composed of fat, smooth muscle and thick
walled blood vessels in varying proportions.
Lipomatosis

It is a rare condition, characterized by diffuse over growth f fatty tissue, which affects
mainly adult males in one of the four following forms

Multiple symmetric lipomatosis

Asymmetric lipomatosis

Pelvic lipomatosis

Mediastinoabdominal lipomatosis

They are nonecapsulated fatty tumors, they infiltrate adjacent muscles and soft tissues, there
is an absence of lipoblasts and malignant characteristics, fibrous elements are present, and
there is hypertrophy of subjacent bone.55

II. Intermediate (locally aggressive)

Well-differentiated liposarcoma

Liposarcoma is arguably the most common malignant mesenchymal neoplasm

accounting for 20% of all soft tissue sarcomas in adults.56

Currently, liposarcoma is classified into several subtypes based on morphology and

malignant behavior.

The World Health Organization describes five subtypes of liposarcoma, including

well-defferentiated, dedifferentiated, myxoid, pleomorphic (these four representing
histologically distinct entities) and mixed liposarcoma (a combination of pleomorphic and
myxoid or of dedifferentiated and myxoid subtypes). It is helpful to think of liposarcomas in
three main groups based on a biologic continuum which include; well differentiated to
dedifferentiated liposarcomas, myxoid to round cell liposarcomas, and pleomorphic
liposarcoma.56

III. Malignant tumors of adipose tissue

These tumors are one of the commonest soft tissue sarcomas of adult life.

It occurs most commonly between 40 and 60 years, although rare in childhood.

It has a marked predilection for lower extremity followed by retro peritoneum.

Radiographically, liposarcoma shows distinct translucency and myxoid type stands

out by increased density. CT and MRI are valuable for detection of recurrent neoplasms.

On gross examination, most of them measure between 5 and 10 mm, well

circumscribed and yellowish on cut section well-differentiated and myxoid iposarcomas were
more commonly found within the primary tumor, however, dedifferentiated liposarcomas
were more common in recurrent tumors.57

Myxoid liposarcoma

The tumor consists of Lipoblasts in varying stages- from primitive mesenchymal

stellate cells, multivacuolated lipoblasts to signet ring lipoblasts. These tumor cells are
admixed with plexiform capillary network and abundant myxoid matrix.

Round cell Liposarcoma

This tumor consists of poorly differentiated myxoid liposarcomatous areas with

excessive proliferation of small uniform round cells.

This tumor reveals large giant cells or numerous giant cells.

Recurrence is common in deep-seated liposarcomas. Dedifferentiation is seen in

tumors at all sites with majority in the retroperitoneum.

SMOOTH MUSCLE TUMORS

I. Benign tumors

Cutaneous leiomyoma

These occur as cutaneous nodules over the extremities in the early adult life.
Microscopy shows bundles of smooth muscles blending with dermal collagen.

Angiomyoma

It occurs as a painful solitary nodule, common in 4 th to 6th decades of life, with

predominance in females over the lower extremities.58
II. Malignant tumors

It accounts for 5-10% of all soft tissue sarcomas seen in elderly females. Commonest
site is retro peritoneum. Radiation exposure is a possible etiological factor. On gross
examination, the tumor appears as a fleshy large mass. Microscopy reveals well-
differentiated tumor cells arranged in fascicular pattern.

In 1977, Ramchod m61 studied 100 cases of G1 Tract and retroperitoneal

leiomyosarcomas and found frequency of mitosis as the most useful criteria for the diagnosis
of leiomyosarcoma.

SKELETAL MUSCLE TUMORS

I. Benign (Rhabdomyoma)

Rhabdomyoma are benign tumors of striated muscle cells and generally divided into
the following categories: cardiac rhabdomyomas, which are relatively common, and
extracardiac rhabdomyomas, which are rare (comprising only 2% of al tumors with striated
muscle differentiation ) adult rhabdomyoma occurs in the head and neck area, the fetal type
occurs in both adults and children and the genital type occurs in the vagina and vulva of
middle-aged women.62

II. Malignant tumors

Rhabdomyosarcoma (RMS)

There are classified into 3 histological types:

a) Embryonal rhabdomyosarcoma
b) Alveolar rhabdomyosarcoma
c) Pleomorphic rhabdomyosarcoma

Clinical presentation according to the histological type

Clinical presentation

According to the histological Incidence Age Sex Site

Type: Histological type

 Embryonal 50-60% of RMS Birth – 15 Yrs M>F
More Common In Extremities

Alveolar 15-20% of RMS 10-25 yrs M=F Head, Neck and

genitourinary

Pleomorphic <5% of RMS <40 YEARS M>F Large muscles of

extremities

On gross, they appear as gray white mass measuring less than 4 cm

A microscopic finding in embryonal RMS reveals a mixture of undifferentiated hyper

chromatic round or spindle cells to differentiated cells having eosinophilic cytoplasm
characteristic of rhabdomyoblasts and myxoid matrix.

Pleomorphic RMS is difficult to distinguish from other pleomorphic sarcomas.

Immunohistochemisty is helpful since they show positivity for desmin and myoglobin.63

The inter group rhabdomyosarcoma studies (IRS) has formed a clinical staging
system. They also defined various favourable and unfavourable factors in prediciting the
prognosis.64

TUMORS OF BLOOD VESSEL

I. Benign tumors

Haemangioma

These are benign but non-reactive process in which there is an increase in the number
of normal or abnormal appearing blood vessels.65

It is one of the common soft tissue tumors. In pramila jain e et al., study they formed
18.91% of all benign soft tissue tumors.16

There are most commonly seen in infancy and childhood.

Capillary Haemangioma

It is the most common type consisting of capillary sized blood vessels.

 Cellular hemangiomas or strawberry nevus is an immature form of capillary
haemangioma occurring in infancy at a rate of about 1 in every 20 live births.66

Cavernous Haemangioma

These are less frequent and are composed of dilated blood filled vessels lined by
flattened epithelium.]

Epithelioid haemangioma

It is a rare lesion seen in mid adult life with female predilection. Common location is
around the ear.

Lymphangioma

Lymphangioma are classified as microcystic (capillary lymphangiomas), macrocystic

(cavernous lymphangiomas) and cystic hygromas according to the size of the lymphatic
cavities incorporated.67

They form 0.9% of all benign soft tissue tumors in Kransdorf’s series and are present
at birth or by 2nd year of life with equal sex incidence.

There have predilection for head, neck and axilla.

Macroscopically, they are il-defined sponge like lesions. Microscopy reeals

lymphatic vessels lined by endothelium and filled with proteinaceous fluid containing
lymphocytes.

Though benign may cause morbidity because of large size and infection. Complete
excision is the preferred mode of treatment.68

Kaposi sarcoma

These are rare tumors with four clinical settings.

a) The chronic type occurring in elderly males, often associated with a second malignancy of
lymph reticular system.69

b) lymphadenophathic type occur in young African children

c) transplantation associated
d) AIDS related Kaposi sarcoma.

Microscopy reveals network of jagged blood vessels in the upper dermis with bland
appearing lining cells.

IV. Malignant vascular tumor

Angiosarcoma

These are malignant tumors and are the rest soft tissue tumors.

They comprise less than 1% of sarcomas.70

They formed 2.1% in Kransdorf’s (1995) series, chronic lymph edema, chronic
filariasis, radiation exposure and environmental chemical carcinogens are the known
predisposing factors.

This tumor primarily affects elderly patients with a male predilection and occurs
commonly in superficial soft tissue of the distal extremities, head and neck

Microscopy reveals vascular channels of irregular size and shape. Plump endothelial
cells having hyperchromatic nucleus in these channels.

The prognosis is poor. The tumor spreads by local extension. Commonest site of
metastasis are cervical lymph nodes, lung, liver and spleen. Radical excision is to be done to
prevent local recurrences.70

PERIPHERAL NERVE SHEATH TUMORS

These tumors of presumed peripheral nerve or nerve sheath derivation are some of the
common soft tissue neoplasms in routine practice.

Benign peripheral nerve tumors

A. Neurofibromas
They are divided into three types: localized, plexiform and diffuse neurofibroma.
1. Localized neurofibroma:

It is sporadic, usually superficial, solitary and unassociated with genetic syndromes.

Microscopically the circumscribed nodule is composed of neural bundles with wavy
nuclei and strands of collagen in a neuro fibrillary background.
B. Schwannoma (Neurilemoma)

It most often occurs in patients between 20 and 40 years and presents as a solitary
mass in the head and neck, on the flexor surface of upper and lower extremities. It very in
size, but encapsulated and cut surface shows yellow to yellow white areas with or without
areas of cystic degeneration. Microscopically it is characterized by alternating Antoni A
(hypercellular areas consisting of spindled cells with nuclei exhibiting palisading and verocay
bodies) and Antoni B areas (hypocellular areas with haphazardly arranged oval to spindle
cells in loose fibrous matrix).72

Malignant peripheral nerve tumors

A. Malignant peripheral nerve sheath tumor

It is a large fusiform neoplasm that has a fleshy mucoid cut surface with large areas of
hemorrhage and necrosis. Microscopically it is composed of interlacing fascicles of
malignant spindle cells exhibiting wavy or comma shaped nuclei.73

Gastrointestinal stromal tumours

GISTs are mesenchymal neoplasms of the gastrointestinal (GI) tract and are though to
develop from the interstitial cells of Cajal, innervated cells associated with the Auerbach
plexus. GISTs are typically defined by the expression of c-KIT (CD117) in the tumor cells,
as these activating KIT mutations are seen in 85-05% of GIST ABOUT 3-5% of the
remainder of KIT –negative GISTs contain PDGFR alpha mutations.74

Stromal tumours of the gastrointestinal tract (GIST) occur over a wide age range but
affect predominantly middle-aged and elderly individuals, with a slight female predominance.
Bleeding is the most common initial symptom, and up to 20% of patients present with
anemia. Pain represent another common complaint. Despite their large size, only a small
proportion of tumours are palpable.75

The overall 5 and 10-years survivals of malignant GISTs, have been estimated at
between 25 and 50%, although in one series, and only 10% of patients remained free of
disease after a median follow-up of 68 months.

GISTs have variable malignant potential, ranging from small lesions with a benign
behavior to fatal sarcomas. Most tumors stain positively for the mast/stem cell growth factor
receptor KIT and anoctamin 1 and harbor a kinase-activating mutation in either KIT or
PDGFRA 78. Tumors without such mutations could have alterations in genes of the
succinate dehydrogenase complex or in BRAF, or rarely as family genes. About 60% of
patients are cured by surgery. Adjuvant treatment with imatinib is recommended for patients
with a substantial risk of recurrence, if the tumor has an imatinib-sensitive mutation.
Tyrosine kinase inhibitors substantially improve survival in advanced disease, but secondary
drug resistance is common.76

Undifferentiated sarcomas

A. Undifferentiated pleomorphic sarcoma

They are the most common types of sarcoma in patients over age 40 years. The peak
incidence is in the 6th and 7th decades with a male predominanace. They are typically large
deep-seated tumors which show progressive, often rapid enlargement and have predilection
fro extremities, especially the lower limb. Microscopy reveals pleomorphic fibroblst like
cells, histocytes, inflammatory cells and bizarre cells or multinucleated giant cells with
numerous atypical mitosis.

B. Giant cell ‘MFH’/undifferentiated pleomorphic sarcoma with giant cells

In addition to pleomorphic cellular areas, microscopy reveals prominent stromal

osteoclastic giant cell reaction.

C. Inflammatory ‘MFH/undifferentiated pleomorphic sarcoma with prominent

inflammation

It is characterized by sheets of xanthomatous cells, both benign and malignant,

admixed with atypical spindle cells and inflammatory cells.77,78

TUMORS OF UNCERTAIN DIFFERENTIATION

I. Benign

Aggressive Angiomyxoma
 Aggressive angiomyxoma is a rare mesenchymal tumor that most commonly arises in
the vulvoaginal region, perineum, and pelvis of women. The term aggressive emphasizes the
often infiltrative nature of the tumor and its frequent association with local recurrence.
Patients often present with nonspecific symptoms which are frequently misdiagnosed with
more common entities, such as a Bartholin cyst, lipoma, or hernia. 79 Histlogic examination
reveals a hypocellular and highly vascular tumor with a myxoid stroma containing
cytologically bland stellate or spindled cells. The tumor cells are characteristically positive
for estrogen and progesterone receptors, suggesting a hormonal role in the development of
the tumor.79

II. Malignant tumors

Alveolar soft part sarcoma

It is an uncommon neoplasm forming only 0.5% of soft tissue sarcomas in

Kransdorf’s et al, series (1995).

They are commonly seen in adolescents and young adults, with a female
preponderance. The common sites are the lower extremities, especially the anterior portion
of thigh.

On gross, these are poorly circumscribed, soft and friable. Cut section shows areas of
necrosis and hemorrhage.80

Microscopy shows dense fibrous trabeculae dividing the tumor into compact groups
that in turn are subdivided into sharply defined nests of rounded or polygonal cells having
abundant granular cytoplasm giving rise to an organoid pattern.

It has poor prognosis with likelihood of metastasis to lung or brain.

Clear cell sarcoma

It can produce melanin but differs from conventional melanoma in that it is more
deeply located in association with tendons or aponeurosis and lacks epidermal / junctional
changes.

They mainly affect young adults between 20 and 40 years with female predilection
and occur in the extremities.
 On gross examination, the tumor appears as well circumscribed grey or white mass.
Microscopically, tumor cells, which are fusiform or cuboidal in shape, are arranged in nests
and fascicles. The cells have large nucleoli with cytoplasmic melanin.

Progrnosis is poor and may develop local recurrences and metastasis.83

Extra skeletal Ewing’s sarcoma

These are primitive neuroblastic tumors arising outside the autonomous nervous
system and have good prognosis. It is more common in females around 20 years of age,
commonly located in Para vertebral and chest wall region.

They are lobulated masses measuring less than 10cm.

Microscopy reveals sheets of small dark cells having scant cytoplasm with rosettes.85
 MATERIAL AND METHODS

The present cross-sectional study was conducted in the Department of Pathology

during the period of September 2014 to September 2017. Ethical clearance for the study was
obtained from Institutional Ethics Committee on Human Subjects Research, DECCAN
COLLEGE OF MEDICAL SCIENCES, HYDERABAD.

A total of 100 samples from patient with soft tissue tumors, including both benign and
malignant tumors, were analyzed.

Inclusion criteria

 All the tumors of soft tissue origin, benign and malignant, were included.

Exclusion criteria

 Inflammatory conditions were not included such as;

 Myositis
 Vasculitis
 Recurrent tumours

Retrospective study group (n=30)

For the period of Sep 2014 to june 2015 embedded tissue blocks of patients diagnosed
with soft tissue tumors, both benign and malignant, were retrieved from the surgical
pathology department. The clinical details of the patients were obtained from the case filed
retrieved from the Medical Records Department of the hospital.

Prospective study group (n=70)

For the period of june 2012 to sep 2015, biopsies and surgical resected specimens of
soft tissue tumors sent to the Department of Pathology were included. Thoroughly history
was taken and findings were recorded on predesigned and presteed proforma (Annexure II).
The biopsies and resected surgical specimens were sent to the Department of Pathology in
10% formalin fixative. The resected specimens were subjected to meticulous inspection of
the external and cut surface. The gross findings were noted down in the proforma (Annexure
II).
 The specimens were grossed and multiple representative bits from the tumors,
adjacent tissue, all surgical margins and any other relevant areas were submitted for
processing. Regional lymph nodes, if received, were inspected grossly for nodal involvement
and submitted either entirely if ≤ 5 mm or bisected and one half was submitted if > 5 mm in
dimension. Biopsies, whenever received fro diagnosis, were submitted entirely for
processing. Thin sections, 3-4 microns thick, were cut from the paraffin block. The slides
prepared were routinely stained by Harris; Hematoxylin and Eosin stain (Annexure III) and
evaluated by light microscopy.

Special histochemical stains, like Periodic Acid Schiff stains, Gomori”s reticulum
were used wherever required (Annexure III). Immunohistochemical staining was employed in
few cases where routine morphology and histochemistry was unsuccessful in providing a
definitive diagnosis (Annexure III)

Statistical analysis

Data obtained was coded and entered into Microsoft Excel spreadsheet (Annexure V). the
data was analyzed using rates, ratios and percentages.
 RESULTS

The Present study includes a total of 100 soft tissue tumors during sep 2014 to sep
2017 (30 cases in retrospective and 70 cases in prospective study) out of a total 1052 tumors
of all types received in the department of pathology at (DECCAN COLLEGE OF MEDICAL
SCIENCES during 3 years period.

TABEL 1 : PERCENTAGE OF SOFT TISSUE TUMORS

1 Total numbers of tumors 1052

2 Total number of soft tissue tumors 100

3 Percentage incidence of soft tissue tumors out of all tumors 9.5%

Soft tissue tumors constituted 9.5 % of all tumors.

GRAPH 1: RELATIVE INCIDENCE OF SOFT TISSUE TUMORS

9.50%

other tumors
soft tisue tumors

90.50%
DISTRIBUTION OF ALL SOFT TISSUE TUMORS ACCORDING TO ITS
CTEGORY

Adipocytic tumours

Benign

Lipoma – 65

Lipomatosis -1

Fibroblastic tumor

Benign

Fibroma – 6

Desmoids type fibromatosis – 1

Angiofibrom – 1

Dermatofibrosarcoma pertuberans – 1

Malignant

Fibrosarcoma – 1

Fibrohistiocytic tumour

Benign

Benign fibrous histiocytoma – 2

Smooth muscle tumour

Benign

Leiomyoma – 1

Vascular tumour

Benign

Haemangioma – 8
Lymphangioma – 3

Gastro – intestinal stromal tumors

Malignant – 1

Nerve sheath tumor

Benign

Neurofibroma – 8

Neurofibromatosis – 1
 TABLE 2: PERCENTAGE OF BENIGN AND MALIGNANT SOFT TISSUE
TUMORS

Type Total No % Benign Malignant

No % No % No %

All tumors 1052 100 693 65.8 350 34.2

Soft tissue tumors 100 9.5 98 98 2 2

GRAPH 2: INCIDENTAL OF BENIGN AND MALIGNANT TUMORS

1200

1000

800

600 malignant
benign

400

200

0
all tumors soft tissue tumors

Benign tumors constituted 98% of all soft tissue tumors and the malignant counterpart
formed only 2.
 TABLE 3: RELATIVE INCIDENCE OF BENIGN SOFT TISSUE TUMORS

Type No %

All benign tumors 693 100

Benign soft tissue tumors 98 14.1

Benign soft tissue tumors accounted for 14.1 of all benign tumors.

TABLE 4: RELATIVE INCIDENCE OF MALIGNANT SOFT TISSUE TUMORS

Type No %

All Malignant tumors 359 100

Malignant soft tissue tumors 2 0.5

Malignant soft tissue tumors formed only 0.5 (<1%) of all malignant tumors.

TABLE 5: INCIDENCE OF BENIGN AND MALIGNANT SOFT TISSUE TUMORS

Type No %

Soft tissue tumors - Total 100 100

Soft tissue tumors – Benign 98 98

Soft tissue tumors – Malignant 2 2

Out of all soft tissue tumors 98 are benign, where as 2 are malignant.
TABLE 6: INCIDENCE OF BENIGN AND MALIGNANT OSFT TISSUE TUMORS
ACCORDING TO CATEGORY

SL.No. Tumors Benign Malignant Total

Fibroblastic tumours 1

Fibroblastic tumours -

Adipocytic tumours -

Peripheral nerve sheath tumours 0

Smooth Muscle tumours 0

Vascular tumours 0

Gastrointestinal stromal tumours 1

 The adipocytic tumors accounts for the majority of benign soft tissue tumors (66%)
followed by vascular tumors (11%) Fibroblastic Tumors (10%). Skeletal muscle
tumors and tumours of uncertain differentiation are not encountered in the present
study/
 The gastrointestinal stromal tumours accounted for the (1%) of malignant soft tissue
tumours
TABLE 7 : SEX INCIDENCE OF ALL SOFT ITSSSUE TUMORS

SL.No. Tumors Males Females

Fibroblastic

Fibrohistiocytic

Adipocytic 36

Nerve sheath

Smooth Muscle

Vascular

Gastrointestinal stromal

Total 55

Incidence of adipocytic tumors and vascular tumors are relatively more common in
females while almost equal incidence in rest of the tumors. There are 45 males and 55
females with a slight female preponderance in general in the case of all soft tissue lesions as
highlighted above.

45%
male
female
55%
GRAPH 3: SEX DISTRIBUTION OF SOFT TISSUE TUMORS
 In case of all soft tissue tumors 55% of STT’S are encountered in females and 45% in
males. Males to female ratio is 1:1.2

TABLE 8: SEX INCIDENCE OF BENIGN SOFT TISSUE TUMORS

SL.No. Tumors Males Females

Fibroblastic

Fibrohistiocytic 1

Adipocytic 36

Nerve sheath

Smooth Muscle

Vascular

Gastrointestinal stromal

Total 54

Benign STT’S are more in females than males with male to female ratio of 1:1.1,
TABLE 9: SEX INCIDENCE OF MALIGNANT SOFT TISSUE TUMORS

SL.No. Tumors Males Females

Fibroblastic

Fibrohistiocytic

Adipocytic

Nerve sheath

Smooth Muscle

Vascular

Gastrointestinal stromal

Undifferentiated sarcoma

Total

While there was only a slight female preponderance in the case of benign tumors,
malignant soft tissue tumors showed equal to female ratio of 4:1

GRAPH 4: SEX INCIDENCE OF SOFT TISSUE TUMORS

60

50

40

30 Benign
Malignant

20

10

0
male female
AGE INCIDENCE

TABLE 10: AGE DISTRIBUTION OF BENIGN SOFT TISSUE TUMORS

SL. No Tumor Type 0-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80

Fibroblastic

Fibrohistiocytic

Adipocytic

Nerve sheath

Smooth Muscle

Vascular

Total

The youngest patient in the present study was 4 yr old while the oldest was 76 years
old. Majority of the benign tumor occurred in the second, third and fourth decade with a
peak incidence in the fourth decade.
TABLE 11: AGE DISTRIBUTION OF MALIGNANT SOFT TISSUE TUMORS

SL. No Tumor Type 0-10 11-20 21-30 31-40 41-50 51-60 61-70 71-80

1 Fibroblastic 0 0 0 0 0 0 1 0

2 Gastrointestinal stromal
0 0 0 0 0 1 0 0

Total 0 0 0 0 0 1 1 0

Majority of the malignant tumors occurred in the fifth and sixth decade.

GRAPH 5: AGE DISTRIBUTION OF BENIGN AND MALIGNANT SOFT TISSUE

TUMORS

30

25

20

15 Benign
Malignant

10

0
0-10 20-Oct 20-30 30-40 40-50 50-60 60-70 70-80
SITE DISTRIBUTION

Soft tissue tumors occurred all over the body true to its vast distribution. In our study

We divided while body in to 4 major anatomical category as-:

Upper extremity – arm + Forearm + Hand + elbow + wrist

Lower extremity – Thigh + Leg + knee + foot

Head and Neck – Head + Neck + face

Trunk – Shoulder + Abdomen + Back + Retroperitoneum + Tests

TABLE 12 : ANATOMICAL SITES OF BENIGN SOFT TISSUE TUMORS

Extremities

Upper Lower Head and Neck

SL.No Tumor type Prox Dist Prox Dist Trunk

1 Fibroblastic 4

2 Fibrohistiocytic 0

3 Adipocytic 19

4 Nerve sheath 1

5 Smooth Muscle 0

6 Vascular 6

Total 30

The benign soft tissue tumors showed predilection for upper extremity followed by head and
neck.
GRAPH 6: SITE DISTRIBUTION OF BENIGN SOFT TISSUE TUMORS

30%

40%
upper extremity
lower extremity
trunk
head and neck

18%

10%
TABLE 13: ANATOMICAL SITES OF MALIGNANT SOFT TISSUE TUMORS

Extremities

Upper Lower Head and Neck

SL.No Tumor type Prox Dist Prox Trunk

Fibroblastic 0

Gastrointestinal stromal 0

Total 0

Malignant soft tissue tumors showed a marked predilection for upper extremity and
trunk.

GRAPH 7: SITE DISTRIBUTION OF MALIGNANT SOFT TISSUE TUMORS

Upper extremity
50% 50%
Trunk
 CLINICAL PRESENTATION

Majority of soft tissue tumors presented with a painless mass, which had been present
for 2 months to 3 years.

Malignant tumors presented with duration from 8 months to 2 years.

GROSS PATHOLOGY

TABLE 14: DISTRIBUTION OF BENIGN STT ACCORDING TO SIZE

Total benign tissue tumors 98 98%

Benign soft tissue tumors measure <5cm 93 93%

Benign soft tissue tumors measure >5cm 5 5%

TABLE 15: DISTRIBUTION OF MALIGNANT STT ACCORDING TO SIZE

Total Malignant tissue tumors 2 %

Malignant soft tissue tumors measure <5cm 1 50%

Malignant soft tissue tumors measure >5cm 1 50%

On gross, majority of the benign tumors (93%) was well circumscribed measuring
less than 5 cm. 5 cases of lipoma were greater than 5cm. in contrast 50 of the malignant soft
tissue tumors measured more than 5 cm.

Most benign soft tissue tumours are soft to firm in consistency while malignant are
firm to hard.
 GRAPH 8: GROSS APPEARANCE OF ALL STT

7%
18%

15%

Grey white
Grey yellow
Grey brown
Yellow

60%

GRAPH 9: GROSS FETURES OF MALIGNANT STT

Microscopically, all soft tissue tumors were histologically typed flowing recent WHO 2013
classification of soft tissue tumor.

The various histological subtypes of different tumor groups encountered are

discussed under individual tumors.
 INCIDNECE, AGE SEX, COMMON SITE AND MORPHOLOGICAL

SUBTYPES OF VARIOUS BENIGN SOFT TISSUE TUMOR GROUPS

TABLE 16: TUMORS INCIDENCE, AGE, SEX AND SITE DISTRIBUTION OF

BENIGN FIBROBLASTIC TUMORS

No. of cases (%) of

benign tumors

Age in Years Common site

(Range)
SL.No Histological type Sex M/F

1 Fibroma 6 21-38 4/2 Upper extremity

2 Desmoids type fibromatosis

1 76 0/1 Trunk

3 Angiofibroma 1 27 1/0 Head & Neck

5 Dermatofibrosarcoma pertuberans
1(1%) 32 0/1 Upper extremity

Total 9 (9%) 21-78 5/4 Upper extremity

The commonest benign fibrous tumor was fibroma (6 cases) which occurred more
commonly in the young age group with predilection upper extremity

There were only one case each of angioribroma, fibromatosis and

Dermatofibrosarcoma pertuberans.
 TABLE 17: INCIDENCE, AGE, SEX AND SITE DISTRIBUTION OF BENIGN
FIBROHISTIOCYTIC TUMORS

No. of cases (%) of

benign tumors

Age in Years Common site

(Range)
SL.No Histological type Sex M/F

Benign fibrous histiocytoma 2 (2%) 11-40 Upper Extremity

TOTAL 2 (2%) 11-40 Upper extremity

There were 2 cases of benign Fibrous histiocytomas involving both upper and lower
extremity with equal male and female ratio.
 TABLE 18: INCIDENCE, AGE, SEX, AND SITE DISTRIBUTION OF BENIGN
ADIPOSE TISSUE TUMORS

No. of cases (%) of

benign tumors

Age in Years Common site

(Range)
SL.No Histological type Sex M/F

Lipoma 62 (63%) 3-68 35/27 Upper extremity

Fibro lipoma 2 (1%) Upper extremity Trunk

Angio Lipoma 1 (1%) Lower extremity

Lipomatosis 1 (1%) 62/M Trunk

Total 66 (66%) 3-68 39/27 Upper extremity

The commonest adipocytic tumors were lipomas followed by Fibrolipomas which

showed slight female predilection and common site is upper extremity.

Benign adipocytic tumors were most common tumors of all STT’S 66 Cases 66%
 TABLE 19: INCIDENCE, AGE, SEX AND SITE DISTRIBUTION OF BENIGN
VASCULAR TUMORS

No. of cases (%) of

benign tumors

Age in Years Common site

(Range)
SL.No Histological type Sex M/F

Hemangioma 8(8%) 6-40 Head and neck

Lymphangioma 3 (3%) 6-70 Upper extremity

Total 18 (12.4%) 6-60 Head and Neck

Benign vascular tumors are the second most common benign tumor group (11%).
Hemangiomas occurred in first three decades, they showed a striking predilection for the
head and neck region unlike other benign soft tissue tumor lymphangioma, were more
common in first two decades benign vascular tumors were more common in females their
males(4/7)
 TABLE 20: INCIDENCE, AGE, SEX AND SITE DISTRIBUTION OF BENIGN
PERIPHERAL NERVE SHEATH TUMORS TUMORS

1 Neurofibroma 8 (8%) 18-75 3/5 Upper Extremity

2 Neurofibromatosis 1 (1%) 14-75 3/6 Head and Neck

Total 9 (9%) 14-75 3/6 Upper extremity

Peripheral nerve sheath tumors constitutes 9% of all benign soft tissue tumors with a
wide range of age distribution with M:F of 0.5. and the most common site was upper
extremity. Neurofibroma was commonest benign tumor comprises of 8% of all benign soft
tissue tumors followed by neurofibromatosis 1%.
INCIDENCE, AGE, SEX, COMMON SITE AND HISTOOGICAL SUBTYPES OF
VARIOUS MALIGNANT SOFT TISSUE GROUPS

TABLE 21: INCIDENCE, AGE, SEX AND SITE DISTRIBUTION OF MALIGNANT

FIBROBLASTIC TUMORS

No. of cases (%)Age

of in Years
Sex M/F Common site
malignant tumors(Range)
Histological type

Adult Fibrosarcoma 1 (50%) Lower Extremity

FIBROSARCOMA: Only one cases is encountered in the present study.

TABLE 22: INCIDENCE, AGE, SEX AND SITE DISTRIBUTION OF MALIGNANT
GASTROINTESTINAL STROMAL TUMORS

No. of cases (%)Age

of in Years
Sex M/F Common site
malignant tumors(Range)
Histological type

GIST 1 (50%) 45-58 Trunk

Figure 1 : photograph shows well encapsulated greasy yellow colored hear shaped giant
lipoma.

Figure 2: Angiofibroma : Composed of dilated vessel with hyalinised walls and fascicles
of spindle cells. (H&E, 100X).

Figure 3: A, Lipoma: Lobules of mature adipocytes with small eccentric nuclei (H&E,
100X): B, Angiolipoma: Composed of mature adipocytes and capillaries. (H&E, 100X).

Figure 4: Giant cell tumor of tendon sheath: A, Low power showing lobulated tumor,
(H&E, 100X); B, Admixture of mononuclear histiocytoid cells, foamy cells, lymphocytes
and scattered osteoclastic giant cells. (H&E, 400X),
Figure 5: Cavernous Haemangioma: Low power view showing dilated blood vessels
lined by flattened endothelial cells (H&E, 40X);

Figure 6: Capillary Haemangioma : Numerous round to oval capillaries lined by

plumed endothelial cells (H&E, 40X).

Figure 7: Fibroma: Nodular mass of dense and hyalinized fibrous tissue arranged in
fascicles (H&E, 40X).

Figure 8: Desmoid Type Fibromatosis: Profliferation of bland spindled cells arranged in

ill defined fascicles (H&E, 40X).
Figure 9: Neurofibroma: Dispersed spindle shaped cells with ovoid and comma shaped
nuclei and scant cytoplasm separated by collagen fibers and myxoid (H&E, 100X).

Figure 10 : Schwanomma:P Spindle cells arranged in biphasic pattern with compact

areas exhibiting palisading pattern (Antoni A) and loosely arranged cells (Antoni B).
(H&E, 100X).

Figure11: Benign fibrous histiocytoma: Monomorphic spindle cells with plump to

elongated vesicular nuclei arranged in storiform pattern (H&E, 100X).

Figure 12: Dermatofibrosarcoma protuberans: Uniform spindled tumor cells with

plump to way nuclei arranged in storiform and whorled pattern (H&E, 100X).
Figure 13:A Fibrosarcoma: Gross appearance of fibrosarcoma showing a well defined
mass with haemorrhage and necrosis. Figure B: Fibrosarcoma:Spindle shaped
fibroblasts in a collagen background arranged in herringbone pattern , (H&E, 40X).

Figure 14: Malignant peripheral nerve sheath tumor :A: Tumor composed of cellular
areas alternating with less cellular areas arranged in fascicular growth pattern. (H&E,
100X).. Figure B: immunohistochemistry showing focal positivity for S-100 protein.
(100X). Figure C: immunohistochemistry showing positive for nestin. (100X)
Figure 15: A : Gross picture of Gstrointestinal stromal tumor demonstrates a well-
circumscribed homogeneous mass protruding into the mucosa of the small intestine

Figure B: Gastroinestinal stromal tumors showing spindle shaped cells with areas of
haemorrhage and necrosis. (H&E, 40X)

Figure 16: A: Photomicrograph of GIST Shows spindle, elongated and oval cells with
vesicular nuclei and variable amount of cytoplasm with 4-5 mitotic

Figure. (H&E, 40X). Figure B: Immunohistochemistry shows CD 117 partial positivity

(100X). Figure C: Immunohistochemistry shows DOG 1 consistent and even positivity
(100X)
Figure 17 A: Undifferintiated pleomorphic sarcoma :: Photograph shows
psuedoencapsulated, nodular with necrotic and myxoid areas.

Figure B : UPS Photomicrograph exhibiting spindle shaped in sheets at paces separated

by bands of collagen and many tumor giant cells. (H&E, 100X).

Figure 18: UPS: photomicrograph showing pleomorphic spindle shaped to round cells
with vesicular nuclei and prominent nucleoli and moderate amount of eosinophiic
cytoplasm, numerous osteoclast type giant cells with bizarre nuclei and abnormal
mitotic figure. (H&E, 200X)

Figure 19: Immunohistochemisty showing diffusely positive for (A) CD34, (B) Vimentin,
(C) CD99, (H&E, 100X)
 DISCUSSION

During the study period of 3 years, 100 soft tissue tumors were received in the
department of pathology, Deccan College of Medical Science and Hospital formed 9.5% of
all tumors of which 65.9% were benign soft tissue tumors among all benign tumors and 0.9%
(<1%) were malignant soft tissue tumors among all malignant tumors.

A total of 100 soft tissue tumors were studied in the present study. Benign soft tissue
tumors were 98 in number and malignant tumors were 2 constituting 98% and 2%
respectively.

TABLE 23: COMPARATIVE ANALYSIS OF INCIDENCE OF BENIGH AND

MALIGNNT SOFT TISSUE TUMORS

Authors No. of CasesBenign (B) Malignant (G) B:M Ratio

Myre Jensen (1981) 1403 1331 94.6 72 18:5:1

Pramila Jain (2014) 370 335 90.6 35 9.4:1

Amit Bharu (2015) 150 145 96.7 5 29:1

Present Study 100 98 2 49:1

The percentage of malignant tumors was comparable with the study of Myhre
Jensen (1981), which can be explained by the inherent bias in a referral population. In
Pramilajain study also benign tumour is more than 90%. The relative frequency of benign to
malignant soft tissue tumors is difficult to estimate accurately since many of the benign
tumors cause a few problems and thus the patients do not report to the clinician.

The general consensus is that the benign soft tissue tumors outnumber malignant
counterparts by a considerable margin.
INCIDENCE OF VARIOUS BENIGN TUMORS

TABLE 24: COMPARATIVE ANALYSIS OF RELATIVE INCIDENCE OF

VARIOUS BENIGN SOFT TISSUE TUMORS

Tumor type Pramila jain (2014)

Myhre Jensen (1981)
Kransdorf (1995)
Amit Bharv (2015)
Present study

Fibroblastic 2.9 10.5 20.6 9 9

Fibrohistiocytic 1.9 15.8 12.8 2 2

Adipocytic 47.3 48.1 16.1 66 66

Peripheral nerve sheath 18.6 5.1 10 9 9

tumors

Smooth muscle 0.5 3.8 1.7 1 1

Vascular 18.9 11.7 7.6 11 11

The commonest benign tumor type was the adipocytic tumor forming 66% of
benign soft tissue tumors, which is more as compared with the studies of Pramilajain and
Myhre Jensen where they constituted 47.3% and 48.1% of benign tumors respectively.

The second most common benign tumor group was the vascular tumors, which
constituted 11%, which is comparable to the studies of Pramila Jain and Myhre Jensen where
they formed 18.9% and 11.7% respectively.
INCIDENCE OF VARIOUS MALIGNANT TUMORS

TABLE 25: COMPARATIVE ANALYSIS OF INCIDENCE (%) OF VARIOUS

MALIGNANT SOFT TISSUE TUMORS

Tumor type M. Jensen Kransdorf Amit Bharv Present Study

No. of cases 72 12370 5 2

Fibrosarcoma 18 5.3 20 50

UPS 40.3 24.1 20 -

MPNST - 6 20 -

GIST - - 40 50

There were two malignant cases reported, Fibrosarcoma Constituted 50%,

gastrointestinal stromal tumors constituted 50% percentage of GIST is slightly compatible
with Amit bharv study. GIST had been recently included in WHO 2013 classification so
previous studies did not include this in their studies. In contrast all other tumor was
comparable with the other studies.

SEX INCIDENCE OF SOFT TISSUE TUMORS

There were 45 males and 55 females with male to female ratio of 1:1.1 in contrast
to the studies of Kransdorf as outlined in the table below.

TABLE 26: COMPARATIVE ANALYSIS OF SEX RATIO OF ALL SOFT TISSUE

TUMORS

Authors Males Females M:F Ratio

Kransdorf (1995) 16727 13,611 1.2:1

Pramilajain (2014) 163 1.3:1

Amit Bharv (2015) 1:1.1

Present study 1:1.2

 TABLE 27: COMPARATIVE ANALYSIS OF THE SEX INCIDENCE OF BENIGN
AND MALIGNANT SOFT TISSUE TUMORS

Authors Males BenignMales Malignant

Female Benign
Female Malignant
M:F ratio Benign
M:F ratio Malignant

Myhew Jensen (1981) 630 48 731 24 0.9:1 2:1

Pramila Jain (2014) 184 23 151 12 1.2:1 1.9:1

Amit Bharv 69 4 76 1 0.9:1 4:1

Present study 45 0 53 2 - -

In the case of benign tumor group there where 45 males and 53 females with a male to
female ratio of 0.9:1, which is comparable to the study of Myhre Jensen where the male to
female ratio was 0.9:1 as outlined above.

In case of malignant tumors there were 4 males and 1 females with a male to female
ratio of 4:1, which is more as comparable to the studies of M. Jensen and P.Jainas detailed in
the table above.

AGE INCIDENCE OF BENIGN AND MALIGNANT SOFT TISSUE TUMOR

TABLE 28: COMPARATIVE ANALYSIS OF AGE INCIDENCE OF BENIGN AND

MALIGNANT SOFT TISSUE TUMORS

Authors Average age (Benigh) Average age (Malignant)

M. Jenson (1981) 44.5 years 40.5 years

Basher (2010) 27.6 years 39.1 years

Amit bharv (2014) 35.7 years 53.4 years

Present study 39.2 57.3

 In the present study the age ranged from 4 to 76 years. The average age in the case of
benign tumors was 39.2 years and 57.3 years in the case of malignant tumors, which is
comparable to the studies of M. Jensen, Amit Bharu however Bashar studies show lower
average age for both benign and malignant. The range in the benign tumor group was 4 to 76
years with a peak incidence in the fourth decade. The age range in the case of malignant
tumors varied from with a peak incidence in the fifth decade.
SITE DISTRIBUTION OF BENIGN AND MALIGNANT SOFT TISSUE SOFT
TISSUE TUMORS

TAB9LE 29: COMPARATIVE ANALYSIS OF ANATIOMICAL SITE

DISTRIBUTION OF BENIGN SOFT TISSUE TUMORS

Authors Upper extremity Lower extremity Trunk Head and Neck

Venkatraman. J (2014) 28(32.2%%) 24(27.6%) 21(24.1%) 14(16.1%)

Kransdorf (1995) 5946(31.8%) 5373 (28.8%) 3757 (20.1%) 2569(13.8%)

Amit Bharu(2015) 55 (37.9%) 17(11.7%) 26(17.9%) 47(32.4%)

Present Study 38 (38) 11 (11) 19 (19) 30(30)

In the present study the commonest site was upper extremity followed by head and
neck region which is comparable to the Kransdorf and Venkatraman studies.

TABLE 30: COMPARATIVE ANALYSIS OF ANATIOMICAL SITE

DISTRIBUTION OF MALIGNANT SOFT TISSUE TUMORS

Authors Upper extremity Lower extremity Trunk Head and Neck

Kransdorf (1995) 2201 (18%) 4592 (37.1%) 2702 (26.1%) 857 (7%)

Shaham Beg (2012) 1(4.8%) 9(42.8%) 9(42.8%) 2(9.5%)

Amit bharu(2015)
2(40%) 1(20%) 2(40%) 0

Present study
0 1(50%) 1(50%) 0
 In the present study the malignant soft tissue tumors observed to have a strong
predilection for lower extremities and trunk and abdomen constituting 50% each. However
in kansdorf and Shaham beg study lower extremity was the commonest site for malignant
tumor and GIST is not included in older classification as soft tissue tumor so this variation in
trunk and abdomen in acceptable.

CLINICAL PRESENTATION

Majority of the soft tissue tumors presented with a painless mass of varying duration
ranging from a few months to many years. 3 cases of malignant tumors presented with
recurrence and only 1 case showed involvement of underlying bone.

PATHOLOGY

On gross, Majority (95..2%) of benign soft tissue tumors were well encapsulated and
presented with a size less than 5 cm while 60% of malignant soft tissue tumors measured
more than 5 cm, which had been noted by Myhre Jensen where the comparative figures were
95% and 75%, respectively

Microscopically, the different histological variants encountered are discussed under

individual tumor groups.
ANALYSIS OF INCIDENCE, AGE, SEX ANDCOMMON SITE DISTRIBUTION OF
INDIVIDUAL BENIGN TUMOR GROUPS

TABLE 31: COMPARATIVE ANALYSIS OF AGE, SEX, AND SITE DISTRIBUTION

OF BENIGN FIBROBLASTIC TUMORS

Authors
No. of cases (% of benign
Age (Range) Male (%) Female(%) Common (%)
tumors)

M. Jenson 140(10.5%) 20-69 years 57.1 42.9 Trunk

Kransdorf 1786(9.5%) 13-75 years 51.2 48.8 Trunk

Amit Bharv 11(7.6%) 13-78 Years 54.5 45.4 Head and neck

Present study 9 (9%) 11-78 5 4 Head and neck

The benign fibrous tumors constituted 9% of all benign tumors. The commonest site
was the face (head and neck) followed by trunk comparable to the studies of other authors.
TABLE 32: COMPARATIVE ANALYSIS OF AGE, SEX AND SITE DISTRIBUTION
OF BENIGN FIBROHISTOCYTIC TUMORS

Authors
No. of cases (% of benign
Age (Range) Male (%) Female(%) Common (%)
tumors)

M. Jenson 211 (16%) 20-69 years 33.1 66.0 Lower extremity

Kransdorf 2456 (13.2%) <1-57 54 46 Lower extremity

Amit Bharv 4(2.7%) 19-60 50 50 Lower extremity

Present study 2 (2%) 11-40 50 50 Upper and lower extremity

There were 2 cases each of benign fibrous histiocytomas, and tenosynovial giant cell
tumor, commonest site being lower extremity with equal sex predeliction. The incidence of
benign fibrohistiocytic tumors was less when compared to the studies of other authors
however age range and commonest site was comparable to the above studies.

TABLE 33: COMPARATIVE ANALYSIS OF AGE, SEX AND SITE DISTRIBUTION

OF BENIGN ADIPOSE TUMORS

Authors No. of cases (% of benign

Age (Range) Male (%)Female(%)Common (%)
tumors)

Pramila jain (2014)

175 (47.29%) 6-80 years 57.1 42.9 Trunk

Myhre Jensen (1981)

640(48.1%) 20-9 years 47.3 52.7 Trunk

2999(16.1%) 26-8 years 71.8 28.2 Trunk

Kransdorf (1995)

99(68.29%) 3-68 years 48.5 51.5 Upper extremity

Amit Bharv

66(66%) 1-70 45.4 54.6 Upper extramity

Present study
 From the above table it is obvious that lipomas are the commonest of soft tissue
tumors with a peak occurrence in the third and fourth decade, which is comparable to the
studies of other authors. There was 65 cases of lipomas followed by cases of lipomatosis
with a peak incidence in the third and fourth decade and shows slight female sex predeliction
which is quite comparable with the M. Jenson studies. The most common site for lipoma in
present study was upper extremity followed by head and neck, in contrast to other study
where most common site was trunk.

TABLE 34 : COMPARITIVE ANALYSIS OF AGE, SEX, AND SITE DISTRIBUTION

OF BENIGN VASCULAR TUMORS

Authors No. of cases (% of benign

Age (Range) Male (%)Female(%)Common Site
tumors)

157 (11.8%) 0-59 41.4 58.6 Head and neck

Myhre Jensen (1981)

1418 (7.6%) <1-65 44.1 55.9 Head and neck

Kransdorf (1995)

18(12.4%) 6-60 Head And Neck

Amit bharu(2004)

11(11%) 2-60 45.4 54.5 Head and neck

Present study

There were 11 cases of benign vascular tumors 11% . there was a striking predilection
for the Head and neck region, which favourably compares with the studies of other authors.
Vasculartumor shows Female preponderance.. In present study while in other study there was
a slight male preponderance.
TABLE 35 : COMPARITIVE ANALYSIS OF AGE, SEX, AND SITE DISTRIBUTION
OF BENIGN PERIPHERAL NERVE SHEATH TUMORS

Authors No. of cases (% of benign

Age (Range) Male (%)Female(%)Common Site
tumors)

68 (5.1%) 20-69 47.3 52.7 Upper extremity

Myhre Jensen (1981)

1859 (10%) 16-72 55.6 44.4 Lower extremity

Kransdorf (1995)

12 (8.3%) 14-75 33.3 66.7 Upper extremity

Amit Bharu

9(9%) - 33.3 66.7 Upper extremity

Present study

Benign peripheral nerve sheath tumors constitute 9% of all benign soft tissue tumors
which is comparable to the above studies with female predeliction and common site was
upper extremity which is more comparable to the M.Jenson study while kansdrorf study
shows male predeliction ad commonest site was lower extremity however more female
predeliction was seen in present study.

OTHER TUMORS

There was one case of Renal Leiomyoma Occurring in Young female

ANALYSIS AND DISCUSSION OF INCIDENCE, AGE, SEX AND COMMON SITE
DISTRIBUTION OF MALIGNANT TUMOR GROUPS

TABEL 36: COMPARASTIVE ANALYSIS OF AGE, SEX AND SITE

DISTRIBUTION OF MALIGNANT FIBROBLASTIC TUMORS

Authors No. of cases (% of benign

Age (Range) Male (%)Female(%)Common Site
tumors)

Kransdorf (1995) 650 (5.4%) 1-72 51.3 48.7 Lower extremity

LAZIM (2008) 8 (22.8%) 16-55 87.5 12.5 Upper extremity

Present study 1(50%) 48 100% Lower extremity

The malignant fibroblastic tumors included 1 case of adult fibrosarcoma occurring in female
aged 48 years over the right thigh, sex predilection, age incidence cannot be commented on
present study on account of very less data however in kransdorf study there was slight male
predilection and commonest site was lower extremity which is comparable to the present
study while in Lazim study upper extremity was the commonest site.

OTHER MALIGNANTTUMOURS

GASTROINTESTINAL STROMAL TUMORS

In the present study 1 case of gastrointestinal stromal tumors were encountered which
constitutes 50% of all malignant soft tissue tumors.

Gastrointestinal stromal tumors was the new entity in new WHO 2013 Classification
so no study was available for comparison.
 CONCLUSION

The diagnosis and management of soft tissue tumors require a team perspective. Even
though soft tissue sarcomas are rare and usually present just as painless mass, the clinician
must be able to diagnose it early for better management.

Soft tissue lesions are a large and heterogeneous group of neoplasms. Traditionally,
tumors have been classified according to histogenetic features. The large majority of soft
tissue tumors are benign, with a very high cure rate rafter surgical excision. Malignant
mesenchymal neoplasms amount to less than 1% of the overall human burden of malignant
tumors but they are life threatening and may pose significant diagnostic andvtherapeutic
challenge. Hence the present study was undertaken to assess the histopathological pattern of
soft tissue tumors using routine, special and immunohistochemical stains and to classify soft
tissue tumors according to new 2013 WHO classification.

In the present study, benign soft tissue tumors 98% greatly outnumbered malignant
soft tissue tumors 2%. The overall age range of the patients was 3 to 78 years and an
approximate male to female ratio of 1:1.1

Of the 100 cases analyzed, adipocytic tumors were the most common diagnosis
constituting 66% of all the soft tissue tumors. The next common overall diagnosis was
vascular tumors in 11% of cases followed by fibroblastic tumors in 10% of cases.

Peripheral nervesheath tumors were diagnosed 9% and fibrohistiocytic tumors

were 2% of cases. Gastrointestinal stromal tumors were 1% of all the soft tissue tumors
diagnosed. Smooth muscle tumors and tumors of uncertain differentiation each were the least
common diagnosis constituting 1% al all the soft tissue tumors.

Light microscopic morphology, supplemented whenever required by ancillary

techniques especially immunohistochemistry, remains the comerstone for the diagnosis of
soft tissue tumors.
 SUMMARY

The present study is a Histopathological study of 100 cases of soft tissue

tumors recorded at the department of pahtology, Deccan College of Medical Science Hospital
over a period of 3 years (September 2015 to September 2017). The following conclusions
were drawn.

1. The soft tissue tumors (100 cases) constituted 9.5% of the entire biopsy material
(1052 cases) recorded in the department of pathology.
2. Benign soft tissue tumors constituted (13.9%) of all benign tumors and malignant soft
tissue tumor accounted for 0.9% of all malignant tumors diagnosed during the study.
3. Benign soft tissue tumors formed 98% of all soft tissue tumors while malignant soft
tissue tumors constituted 2% of all soft tissue tumors with a benign to malignant ratio
of 49:1
4. Benign soft tissue tumor showed a peak age incidence in the fourth decade.
5. Malignant soft tissue tumors showed a peak age incidence in the fifth and sixth
decade.
6. Soft tissue tumors in general showed slightly female preponderance with a male to
female ratio of 1:1.1
7. The male to female ratio among benign soft tissue tumors was 1:1.1 and 4:1 among
malignant soft tissue tumors.
8. The benign soft tissue tumor showed predilection for upper extremities and head and
neck
9. Like the benign tumor, the malignant soft tissue tumors showed a marked site
predilection for the upper extremities.
10. Majority of the soft tissue tumors presented as a painless mass of duration ranging
from 2 months to 3 years. Malignant tumors presented with duration from 8 months to
2 years.
11. 95.2% of benign tumors measured less than 5 cm, while 60% of malignant tumors
measured more than 5 cms.
12. On detailed histomorphoogical examination, the signal most common histological
group was the adipose tumor, which accounted for 66% of all soft tissue tumors.
13. The commonest benign tumor was lipoma (66%) of all benign tumors of soft tissue
followed by vascular tumors 11%, fibroblastic (10%) to peripheral nerve sheath (9%)
14. The commonest malignant soft tissue tumor was tumor of gastrointestinal stromal
tumors (40%), undifferentiated pleomorphic sarcoma (20%), fibrosarcoma (20%) and
malignant peripheral nerve sheath tumor (20%) in the descending order of frequency.
15. The commonest benign soft tissue tumor in the first and second decade was lipoma
followed by haemangioma.
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 ANNEXURE – II

PROFORMA

1. Serial Number: 2. Hospital Number:

3. Name of the Patient:

4. Age: 5. Sex:

6. Occupation

7. Chief Complaints:

Duration:

Pain:

Trauma:

8. Past History:

9. Family History:

10. Clinical Findings:

11. Radiological Investigations:

12. Histopathological Findings:

A] Gross Procedure:

-incisional Biopsy

-Excisional Biopsy

-other (specify)

Tumor site:

-specify (if known)

-Not specified

Tumor Size (cm):

-Greatest dimensions

- Additional dimensions

Macroscopic extent of the tumour:

Superficial – Dermal / Subcutaneous

Deep Deep: Fascial/Subfascial?intramuscular?medistinal/intra

Abdominal/Retroperitoneal/Head & neck / Other (specify)

Cannot be determined.

-presence or absence of necrosis

Other descriptive characteristics;

Colour, Firm/Soft, Gritty, Fatty, Gelatinous, Calcified, Hemorrhagic

Margins (if excisional biopsy): circumscribed/encapsulated or infiltrative.

B} Microscopy:

Histologic type (WHO Classification of soft tissue tumours)

-specify:

-Cannot be determined:

Mitotic Rate

Specify:_x 10 High Power Fields (HPF)

Necrosis

-present: Extent-_%

-cannot be determined.

Histological Grading (French Federation of Cancer Centers Sarcoma Group

{FNLCC}

-Grade 1

-Grade 2

-Grade 3

-Ungraded Sarcoma

-Cannot be determined.

Margins (for excisional biopsy only)

-cannot be assessed

-margins negative for sarcoma

-distance of sarcoma from closest margin was noted down.

:Specification of margins

-Margins positive for sarcoma

:Specification of Margins

Additional Pathologic Findings:

 ANNEXURE – III

STAINING PROCEDURE

Haematoxylin and Eosin

1. Deparaffinized sections were brought to water.

2. Sections were stained with Harris Haematoxyin for 4 minutes and rinsed in tap water.
3. Differentiation was done in acid alcohol and section were blued in tap water for 5
minutes.
4. Sections were paced in 1 % aqueous eosin for 15 seconds.
5. Differentiation was done by washing in running tap water for 30 seconds.
6. Sections were cleared in xylin and mounted in DPX.

Results: Nuclei : Blue

Cytoplasm: shades of pink

Periodic acid – Schiff reactions (PAS)

1. Deparaffinized sections were brought to water.

2. Sections were oxidized with periodic – Acid – Schiff solution for 5 minutes and
rinsed in distilled water.
3. Sections were placed in Schiff’s leuco – fuchsin for 15 minutes, followed by running
tap water for 10 minutes till pink colour develops
4. Counter staining was done with light green for few seconds and rinsed in tap water. D
5. Differentiation was done in acid alcohol and the sections were rinsed in tap water.
6. Dehydration was carried out by passing the sections through 95% alcohol and
absolute alcohol.
7. Sections were cleared in xyene and mounted in DPX.

Results :

Glycogen, Mucin, hyauronic acid, reticulin, fibrin thrombi, colloid droplets, hyaline and
atherosclerosis, hyaline deposits in glomeruli, colloid of pituitary stalks and thyroid , amyloid
infiltration and other elements show a positive reaction – rose to puplish red.

Nuclei – Blue

Background – Pale Green

VERHOEFF-VAN GIESON (VVG) STAIN

Reagents

1. 5% alcoholic hematoxylin
Hematoxylin-5 g
100% alcohol-100 ml
2. 10% aqueous ferric chloride
 Ferric Chloride -10g
Distilled water – 100 ml
3. Weigert’s iodine solution:
Potassium iodide – 2g
Iodine – 1g
Distilled water – 100 ml
4. Verhoeff’s Working Solution
5% alcoholic hematoxylin – 20 ml
10% Ferric chloride – 8 ml
Weigert’s iodine solution – 8ml
5. 2% aqueous ferric chloride (prepare fresh, not necessary):
10% ferric chloride -10 ml
Distilled water – 50 ml
6. 5% aqueous sodium thiosulfate
Sodium thiosulphate – 5 g
Distilled water – 100 ml
7. Van Gieson’s counterstain
1% aqueous acid fuchsin – 5 ml
Satuarted aqueous picric acid – 100 ml

Procedure of VVG staining

1. A positive control namely aorta, kidney or myometrium is stained witheach batch of

staining.
2. The sections were deparaffinised in xylene andhydrated through graded alcohols.
3. The sections are stained in Verheoff’s solution for 1 hour unti the tissue turns
completely black.
4. The sections are rinsed in tap water with 2-3 changes.
5. Differentiations is done using 2% ferric chloride for 1-2 minutes.
6. The sections are washed with several changes of tap water and checked
microscopically for black elastic fiber staining and gray background.
7. The sections are then treated with 5% sodium thiosulfate for 1 minute.
8. The solution is discarded and sections are washed in running tap water for 5 minutes.
9. The sections are counterstained in Van Gieson’s solutions for 3-5 minutes.
10. The sections are washed in running tap water, dehydrated through graded alcohol,
cleared in xylene and mounted.

Results

Elastic fibers – Blue – black to black

Nuclei – Blue to black

Collagen – Red

Other tissue elements – Yellow

IMMUNOHISTOCHEMICAL (IHC) STAINING

Reagents Required

1. Wash buffer: 1 XPBS (0.137 M NaCL, 0.05 M NaH2Po4, pH 7.4)

2. Incubation buffer: 1% bovine serum albumin, 1% normal donkey serum, 0.3%
Triton X-100, and 0.01% Sodium azide in PBS
3. Primary antibodies
4. Cell and tissue staining kits: Kits include biotinylated secondary antibodies, serum
blocking reagent, peroxidase blocking reagent, avidin blocking reagent, biotin
blocking reagent, high sensitivity streptavidin – HRP conjugated (HSS-HRP), and
chromogen solution, Kits containing chromogenic substrates 3,3;
Diaminobenzidine (DAB) are used.
5. DAB enhancer
6. Hematoxylin counterstain
7. Aqueous mounting medium
8. Antigen retrieval reagents [Tris/EDTA pH 9.0 buffer]

Procedure of IHC staining

1. Deparaffinization and subsequent rehydration of sections are done as follows

-xylene: 2x3 minutes

-xylene 1:1 with 100% ethanol: 3 minutes
-100% ethanol: 2x3 minutes
-95% ethanol: 3 minutes
-70% ethanol: 3 minutes
-50% ethanol: 3 minutes
- sections rinsed in deionized water

2. Heat – Induced antigenic epitope retrieval is performed using a pressure cooker and
Tris/EDTA pH 9.0 buffer.

3. To quench endogenous peroxidase activity, the sections are incubated with 1-3 drops
peroxidase blocking reagent (3% H2O2 in water or methanol) for 5-15 minutes

4. The sections are rinsed and then gently washed in was buffer for 5 minutes.

5. To reduce non-specific hydrophobic interactions between the primary antibodies and the
tissue, the sections are incubated with 1-3 drops of serum blocking reagent for 15 minutes.
The reagent is drained and any excess reagent is wiped away.

6. to block binding to endogenous biotin, the sections are incubated with 1-3 drops of avidin
blocking reagent for 15 minutes. The reagent is drained and any excess reagent is wiped
away.
7.To block subsequent binding to the avidin, the sections are incubated with 1-3 drops of
biotin blocking reagent for 15 minutes. The reagent is drained and any excess regent is
wiped away.

8. The sections are incubated with primary antibodies in incubation buffer.

9. The sections are rinsed 3 times with wash buffer for 5 minutes each.

10. The sections are incubated with 1-3 drops of biotinylated secondary antibodies for 30-60
minutes (depending on the thickness of the section).

11. The sections are rinsed 3 times with wash buffer for 15 minutes each.

12. The sections are incubated with 1-3 drops of High Sensitivity Streptavidin – HRP
conjugate for 30 minutes for signal amplification.

13. The sections are rinsed 3 times with was buffer for 2 minutes each.

14. The sections are incubated with 1-5 drops of DAB/AEC Chromogen Solution for 3-20
minutes.

15. The sections are rinsed 3 times with was buffer for 10 minutes each.

16. The slides are rinsed in deionized water and drained.

17. The sections are counterstained with nuclear counterstain hematoxylin.

18. The sections are then dehydrated through graded alcohol, cleared in xylene and mounted.

Results

Positive immunostaining (cytoplasmic and/or nuclear) –Brown

 ANNEXURE – IV

KEY TO MASTER CHART

ABD Abdomen

AFM Angiofiboma

ALP Angiolipoma

AMFB angiomyofibroblastoma

AMLP Angiomyolipoma

BK back

CPH Capillary Haemangioma

CVH Cavernous Haemangioma

DF Dermatofibroma

DTF Desmoid Type Fibromatosis

DFSP Dermato Fibrosarcomaprotuberans

F Fibroma

FC Face

FS Fibrosarcoma

FLP Fibrolipoma

Gr. Br Grey brown

Gr.wh Grey white

Gr. Yw Grey Yellow

GIST Gastrointestinal stromal tumours

GCTTS Giant cell Tumour of tendon sheath

HD Head

HM Haemangioma

ISC Infected Sebaceous Cyst

JM Jejunum

LP Lipoma
LM Lymphangioma

LA Left Arm

LTH Left Thigh

LL Left Leg

LF Left Foot

LPM Lipomatosis

LMY Leiomyoma

MPNST Malignant Peripheral Nerve Sheath Tumor

NF Neurofibroma

NFM Neurofibromatosis

NK Neck

UPS Undifferentiated Pleomrphic Sarcoma

RA Right Arm

RFA Right Forearm

RTH Right Thigh

RL Right Leg

RF Right Foot

RH Right Hand

SN Shwannoma

SS Synovial Sarcoma

STM Soft Tissue Mass

STT Soft Tissue Tumor

TST Testes

Yw Yellow