SOFT-TISSUE TUMORS AND

TUMOR-LIKE LESIONS
Dr. I Made Naris Pujawan, M.Biomed, Sp.PA
• Traditionally, soft-tissue tumors are defined as mesenchymal proliferations
that occur in the extraskeletal, nonepithelial tissues of the body, excluding
the viscera, coverings of the brain, and lymphoreticular system
• They are classified according to the tissue they recapitulate (muscle, fat,
fibrous tissue, vessels, and nerves)
• Some soft-tissue tumors have no normal tissue counterpart but have
constant clinicopathologic features warranting their designation as distinct
entities
• The true frequency of soft-tissue tumors is difficult to estimate, because
most benign lesions are not removed
• A conservative estimate is that benign tumors outnumber their malignant
counterparts (sarcomas) by a ratio of at least 100 : 1
• In contrast to carcinomas, sarcomas usually metastasize via hematogenous
routes, making the lung and skeleton common sites of dissemination
Fatty Tumors
• Benign tumors of fat, known as lipomas, are the most common soft-
tissue tumor of adulthood
• They are subclassified according to particular morphologic features as
conventional lipoma, fibrolipoma, angiolipoma, spindle cell lipoma,
myelolipoma, and pleomorphic lipoma
• The conventional lipoma, the most common subtype, is a well-
encapsulated mass of mature adipocytes that varies considerably in
size
• Infrequently, lipomas are large, intramuscular, and poorly
circumscribed
• Cured by simple excision
LIPOSARCOMA
• Liposarcomas are one of the most common sarcomas of adulthood and
appear in the 40s to 60s
• They usually arise in the deep soft tissues of the proximal extremities and
retroperitoneum, and are notorious for developing into large tumors
• Liposarcomas are histologically divided into well-differentiated,
myxoid/round cell, and pleomorphic variants
• The cells in well-differentiated liposarcomas are readily recognized as
lipocytes
• lipoblasts; they mimic fetal fat cells and contain round clear cytoplasmic
vacuoles of lipid that scallop the nucleus
• The myxoid/round cell variant of liposarcoma has a t(12;16)(q13;p11)
chromosomal abnormality in most cases
Fibrous Tumors and tumors like lesion
• Nodular Fasciitis
• It most often occurs in adults on the volar aspect of the forearm,
followed in order of frequency by the chest and back
• Affected individuals typically present with a several-week history of a
solitary, rapidly growing, and sometimes painful mass
• Preceding trauma is reported in only 10% to 15% of cases
• Nodular fasciitis arises in the deep dermis, subcutis, or muscle
• The lesion is richly cellular and contains plump, immature-appearing
fibroblasts and myofibroblasts arranged randomly or in short
intersecting fascicles
FIBROSARCOMA
• Fibrosarcomas occur anywhere in the body, but are most common in the
deep soft tissues of the extremities
• Typically these neoplasms are unencapsulated, infiltrative, soft, fish-flesh
masses often having areas of hemorrhage and necrosis
• Better differentiated lesions may appear deceptivelyencapsulated
• Histologic examination discloses all degrees of differentiation, from slowly
growing tumors that closely resemble cellular fibromatosis and sometimes
having spindled cells growing in a herringbone fashion to highly cellular
neoplasms dominated by architectural disarray, pleomorphism, frequent
mitoses, and areas of necrosis
• Fibrosarcomas are aggressive tumors, recurring in more than 50% of cases
and metastasizing in more than 25%
Fibrohistiocytic Tumors
• Fibrohistiocytic tumors contain cellular elements that resemble both
fibroblasts and histiocytes (macrophages)
• The phenotype of the neoplastic cells most closely resembles
fibroblasts, and the term fibrohistiocytic should be viewed as
descriptive in nature and not one that connotes the cell of origin
• BENIGN FIBROUS HISTIOCYTOMA (DERMATOFIBROMA)
• MALIGNANT FIBROUS HISTIOCYTOMA
Tumors of Skeletal Muscle
• Rhabdomyosarcoma, the most common soft-tissue sarcoma of childhood and
adolescence, usually appears before age 20
• It may arise in any anatomic location, but most occur in the head and neck or
genitourinary tract, where there is little if any skeletal muscle as a normal
constituent
• Rhabdomyosarcoma is histologically subclassified into embryonal, alveolar, and
pleomorphic variants
• The rhabdomyoblast—the diagnostic cell in all types—contains eccentric
eosinophilic granular cytoplasm rich in thick and thin filaments
• Rhabdomyoblasts may be round or elongate; the latter are known as tadpole or
strap cells, and may contain cross-striations visible by light microscopy
• Ultrastructurally, rhabdomyoblasts contain sarcomeres, and
immunohistochemically they stain with antibodies to the myogenic markers
desmin, MYOD1, and myogenin.
Tumors of Smooth Muscle
• Leiomyomas, the benign smooth muscle tumors, often arise in the uterus
• Leiomyomas may also arise from the arrector pili muscles found in the skin,
nipples, scrotum, and labia and less frequently develop in the deep soft
tissues and the wall of the gut
• Leiomyomas are usually not larger than 1 to 2 cm in greatest dimension
and are composed of fascicles of spindle cells that tend to intersect each
other at right angles
• The tumor cells have blunt-ended, elongated nuclei and show minimal
atypia and few mitotic figures
• Solitary lesions are easily cured; however, multiple tumors may be so
numerous that complete surgical removal is impractical
LEIOMYOSARCOMA
• Leiomyosarcomas account for 10% to 20% of soft-tissue sarcomas
• They occur in adults and afflict women more frequently than men
• Most develop in the skin and deep soft tissues of the extremities and
retroperitoneum
• Leiomyosarcomas present as painless firm masses. Retroperitoneal tumors may
be large and bulky and cause abdominal symptoms
• Histologically they consist of malignant spindle cells with cigar-shaped nuclei
arranged in interweaving fascicles
• Immunohistochemically, they stain with antibodies to smooth muscle actin and
desmin
• Treatment depends on the size, location, and grade. Superficial or cutaneous
leiomyosarcomas are usually small and have a good prognosis, whereas those of
the retroperitoneum are large, cannot be entirely excised, and cause death by
both local extension and metastatic spread
Synovial Sarcoma
• Synovial sarcoma is so named because it was once believed to recapitulate synovium, but the cell
of origin is still unclear
• In addition, although the term synovial sarcoma implies an origin from the joint linings, less than
10% are intra-articular
• Most occur in the 20s to 40s
• The majority develop in the deep soft tissue and about 60% to 70% involve the lower extremity,
especially around the knee and thigh
• Patients usually present with a deep-seated mass that has been noted for several years
• Uncommonly, these tumors occur in the head and neck or in viscera
• Synovial sarcomas are morphologically biphasic or monophasic.
• The histologic hallmark of biphasic synovial sarcoma is dual lines of differentiation (i.e., epithelial-like and
mesenchymal-like).
• The epithelial cells are cuboidal to columnar and form glands or grow in solid cords or aggregates
• The spindle cells are arranged in densely cellular fascicles that surround the epithelial cell
• Synovial sarcomas are treated aggressively with limb-sparing therapy and frequently
chemotherapy
 MATUR
SUKSMA