MANAGEMENT OF SOFT TISSUE SARCOMA 5.

4B
DATE: Feb. 12, 2020
LECTURER: NO LECTURE SURGERY

OUTLINE III. PATHOLOGY

I. Objectives  Sarcomas- heterogenous group of tumors arising from

II. Introduction mesenchymal cells.
A. Epidemiology  > 100 different histopathologies.
B. Tumor Distribution  Diagnosis made on morphologic pattern with aid of
III. Pathology immunohistochemical staining.
IV. Grading of Tumor  Many have distinct chromosomal translocations.
V. Clinical Presentation  Many have implications for tumor location, behavior
VI. Pattern of Spread and treatment.
VII. Evaluation
VIII. Medical Team Involved in Treatment A. MOST COMMON TYPES OF SOFT TISSUE SARCOMA
IX. Soft tissue Sarcoma Treatment Issues  Undifferentiated/unclassified sarcoma (pleomorphic/round
X. Take Home Points cell/spindle cell).
 Liposarcoma
 Leiomyosarcoma
TH NOTE: The content of the trans is purely based from the PPT sent
 Synovial sarcoma
by Dr. Ragaza for additional readings. No formal lecture was given
 Malignant peripheral nerve sheath tumor (MPNST)
regarding this.
 Rhabdomyosarcoma
I. OBJECTIVES  Primitive neuroectodermal tumor (PNET) /extra skeletal
1. Understand the biologic behavior of different histologic Ewings.
subtypes of sarcomas including response to radiation.  Angiosarcoma
2. Understand the rationale for the use of preoperative  Epithelioid sarcoma
radiation in extremity and retroperitoneal soft tissue  Clear cell sarcoma
sarcomas.  Alveolar soft part sarcoma
3. Understand the set up and clinical tumor volumes for
 Solitary fibrous tumors
treatment of extremity soft tissue sarcomas.
4. Understand the set up and clinical tumor volumes for
treatment of retroperitoneal soft tissue sarcomas. B. UNDIFFERENTIATED/ UNCLASSIFIED SOFT TISSUE
5. Appreciate the approach to patients with unplanned SARCOMA
surgical excision of soft tissue sarcomas.  This category is for sarcomas that lack specific differentiation.
II. INTRODUCTION  Includes some ‘MFT’- malignant fibrous histiocytoma.
A. EPIDEMIOLOGY
C. MYXOFIBROSACRCOMA
 2015 estimated new cases of soft tissue cancers– 11,930.
 Previously called myxoid MFH:
o 0.7% of all cancers.
o Infiltrates centimeters beyond the visible/palpable mass
 2015 estimated deaths from soft tissue cancers-4,870.
and when deep can invade usual barriers.
o 0.8% of all cancer deaths o Higher rate of positive margins.
o Commonly in the extremities.
B. TUMOR DISTRIBUTION
o Greater risk of local recurrence (up to 30%).
 (According to) PCS American College of Surgeons:
o Thigh/buttock/groin 46% D. LIPOSARCOMA
o Upper extremity 13%  One of most common sarcomas: 13%
o Torso 18%  24% of all extremity 45% of RP sarcomas.
o Retroperitoneum 13%  4 distinct WHO subtypes:
o Head and neck 9% o Well-differentiated/dedifferentiated liposarcoma
o Myxoid/round cell liposarcoma
o Pleomorphic liposarcoma
o Mixed

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EDITOR Madjid, N. Palay, Rafael
 E. WELL- DIFFERENTIATED LIPOSARCOMA (WLDS) F. LEIOMYOSARCOMA
 Also known as atypical lipomatous tumors (ALT).  Can occur anywhere in the body, common in the
 Has a ‘pushing’ growth pattern. retroperitoneum.
 Occurs in the:  Seen arising from vasculature – consider at surgery.
o Extremity muscles (most common)
o Retroperitoneal (RP) G. MALIGNANT PERIPHERAL NERVE SHEATH TUMORS
o Variety of other sites
 Originate from peripheral nerves.
 Behavior is different in limb vs. RP:
 50% occur in patients with NF type I.
o Recur less frequent and late
o Not develop metastases  Most common in the extremities, trunk, head and neck.
o Dedifferentiation is uncommon (0-6%).
H. EPITHELOID SARCOMAS
o Managed by marginal excision alone.
 Young adults
 Upper extremity predilection – especially distal UE.

J. SYNOVIAL SARCOMAS

 Originally thought to arise from the synovium of joints but

actual origin is unknown.
 2 types: monophasic and biphasic
 Young adults
 Histology that is more responsive to chemotherapy.

K. ANGIOSARCOMAS

 Uncommon
 Arise in skin/subcutaneous tissue – most typically of the
Figure 1 . Atypical lipomatous tumor vs. well- differentiated breast or H&N.
liposarcoma  One of most common sarcomas seen after RT.

F. PLEOPMORPHIC LIPOSARCOMA
 Chemoresponsive sarcoma- taxanes
 High rate of metastases; resemble undifferentiated
IV. GRADING OF TUMOR
unclassified sarcomas.
 usually a 3-tier system
G. MYXOID/ ROUND CELL LIPOSARCOMA
 Prognostic in some sarcomas.
 Mean age in mid-40’s.
 Some tumor types not typically graded:
 Extremity in vast majority.
o MPNST- all felt to be high grade
 Responsive to chemotherapy (trabectedin).
o Epithelioid
› Round cell LPS: component must be > 5%; high rate of
o Clear cell sarcoma
metastases.
o Angiosarcoma
› Myxoid LPS: distant metastases often too soft tissue sites,
o Extra-skeletal myxoid chondrosarcoma
more than lung.
o Synovial sarcoma
 Dramatic response to radiation.
 McGill- 50 patients are evaluated response to V. CLINICAL PRESENTATION
radiation therapy. Median decrease in tumor  Extremity
volume: o Enlarging painless mass
o <1% for high grade sarcomas o Pain
o 13.8 % non- myxoid low grade o Functional limitations
sarcoma o Symptoms associated with compression of local
o 82.1% myxoid liposarcoma structures.
 Retroperitoneal
o Abdominal mass – often incidentally found.
o Pain
o Gastrointestinal: early satiety, obstruction,
bleeding.

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5.4B MANAGEMENT OF SOFT TISSUE SARCOMA
 o Lymphedema, neurologic or musculoskeletal sx.  Ideally by surgeon who will do definitive
 Rare surgery.
o Fevers/leukocytosis  Longitudinal in extremity.
o Paraneoplastic hypoglycemia (leiomyosarcoma).  Adequate hemostasis
o Symptoms from distant metastases.
VIII. MEDICAL TEAM INVOLVED IN MANAGEMENT
 Treatment requires input from team:
VI. PATTERN OF SPREAD o Radiologist (biopsy/organs/structures involved)
 Extremity o Pathologist (grade/tumor type)
o Along longitudinal tissue planes – within the o Surgeon- (orthopedic/general/plastics/vascular)
compartment. o Medical oncologist
o If involves nerves/vessels, can track along o Radiation oncologist
o Compresses/distorts adjacent soft tissue
o Tumor can be well beyond the mass. IX. SOFT TISSUE SARCOMAS TREATMENT ISSUES
o Hematogenous- predominantly to the lung. A. EXTREMITY
 At diagnosis 10%
 Exceptions: myxoid liposarcoma SURGICAL RESECTABILITY
o Lymphatic -rare, except certain pathologies.  Vascular involvement
 Epithelioid (18%)  Joint involvement
 Rhabdomyosarcoma (12%)  Tissue planes -adjacency to bone
 Clear cell sarcoma (11%)  Functional loss
 Angiosarcoma (13%)  Need for plastic surgery/hand surgeon.
 Other reports: synovial cell, extra-skeletal RADIATION ISSUES
Ewings.
 Retroperitoneal  Can the patient lie still?
o Pushing/displacing adjacent organs.  Extremity: can a stripe of normal tissue be spared?
o Enveloping structures.  What surgical issues will impact the radiation?
o Hematogenous metastases – often late, if at all  What will be removed?
 Liver  Should radiation be preoperative or postoperative?
 Lung TREATMENT ISSUES
 1994-1997 Toronto Randomized Trial
VII. EVALUATION
 Extremity/trunk imaging
o Plain radiography
o MRI of primary site
o (CXR)
o CT chest Figure 2 . (Pre and Post- op result of radiation therapy of upper
 Add Abdomen/pelvis, if tumor in extremity)
groin/myxoid –round cell liposarcoma. › End point: acute wound complications.
o Cell LPS › Defined: second operation or extended non- surgical
o PET wound care.
 Retroperitoneal
o CT abdomen/pelvis
o CXR
o CT chest
o MRI
o PET
 Biopsy
Figure 3 . Toronto Randomized Trial Long Term
o FNA
Evaluation
o Core needle biopsy – multiple samples
o Simulation: Customized immobilization
 Site of biopsy is important for
 Proximal extremity
representative sample.
› ST motion
 CT/ultrasound guidance.
› skin folds
o Incisional biopsy – if needed (extremity)
› genitalia

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 › distance of arm to head  Radially 1.5 cm but limited at fascia/bone
boundaries (unless involved).
 CTV should include peritumoral edema

 Distal extremity
 Fixation to prevent rotation and move other  Target volume definitions for postperative RT.
limb out.  CTV: shrinking field technique
› Initial volume
o Surgical bed reconstructed from preop
imaging.
 Fusion of preop MRI with postop
planning CT.
o Further evaluation based on postoperative
changes, operative and pathology report,
surgical clips.
o Expand volume 1.5 cm radially/4 cm
longitudinally.
› Boost volume
o Same as initial volume except in the
longitudinal.
o Use GTV reconstructed with 2 cm margins
Figure 4 . Photo of fixation of distal extremity.
 Other issue: scar/drain site
 Historically included – Low risk situations,
 Mid extremity
drain site could be omitted.
 Let tissue be dependent if possible.
 Local control related to fields
 LR patients had significantly:
o higher grade
o margin positive
o recurrent disease
o more postoperative boost patients
o slightly older
o more shoulder relapses
o Evaluation of recurrence site

Figure 5 . Fixation of mid lower extremity.  Unplanned excision: common phenomena

 1970’s-1980’s – 10 cm margins (5 cm for low grade). o Typically smaller
 o Typically subcutaneous (mistaken for lipomas)
1990’s – NCIC study used 5 cm margins.
o Often low grade
 Histologic data showed MRI signal 0-7.1 cm, mean 2.5.
o Residual disease 24-74%
 Tumor cells seen in 10/15 cases most within 1 cm but
 How does inadvertent excision affect outcome/
up to 4 cm from mass.
treatment?
 Presence of tumor cells not correlate with
o Re-excision is the norm. To obtain negative
dema/tumor size.
margins.
 Target volume definitions for preoperative RT
o Inappropriate skin incision common.
o GTV: tumor seen on gadolinium-enhanced T1
o Controlled studies – mostly show similar local
MRI.
control andsurvival to those with primary
 Ideally done in treatment position.
management.
 Fused with planning CT images.
o Rate of use of radiation is higher.
o CTV: GTV +
o Higher rate of more aggressive surgery
 Longitudinally 3-4 cm (but limit it across
(amputation)-leading to poorer functional
joints/out of compartment).
outcome.

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  IMRT in extremity STS RADIATION ISSUES
 Advantage  Volume
o Decrease dose to normal structures: bone,  Patient GI stability
soft tissue  Ability to spare normal tissues(meet constraints).
o More conformity  Know kidney plans and function.
 Disadvantage  Preoperative vs. postoperative.
o Cost
o Higher dose to full circumference of limb. To TREAT OR NOT TO TREAT?
o Potential dose to other parts of the body  Postoperative Radiation Therapy
(other limb/head).  Rarely can achieve adequate dose.
 Use: upper thigh – most to gain  More gastrointestinal toxic.
 Use of Intensity Modulated Radiation Therapy (IMRT)  Preoperative Radiation Therapy
with Image Guided Radiation Therapy (GRT)  Tumor readily identifiable.
 Decrease high wound complication rate.  Tumor displaces bowel.
 Minimized dose to ‘skinflaps’ as determined  Potential tumor reduction.
inconjunction with thesurgeon.  Pseudocapsule formation/margin improvement.
 PTV: 50 Gy/25  Typically a lower dose is felt to be needed.
 Flaps: <20 Gy  EORTC 62092: evaluating preoperative RT

SIMULATION

 Upper and lower body immobilization

 Oral contrast – esp for upper abdominal tumors
 IV contrast – to see psoas muscle invasion
 4D simulation for upper abdominal tumors if organ motion >
1 cm consider gaiting.
GROSS TOTAL VOLUME (GTV)
 Register with MRI for muscle extent
 Create ITV to account for tumor motion.

CLINICAL TOTAL VOLUME

 GTV with 2-2.5 cm margin cephalo-caudal.
 GTV with 1.5-2 cm margin radially.
 Exclude: bone, kidney, liver
 Include rim of adjacent bowel/air cavity (5 mm)
 Include any disease extending to the inguinal canal
Figure 6 . Use of IMRT with IGRT
 OUTCOME DOSE ESCALATION AREAS OF HIGH RISK
o Local control is excellent (>90% at 3-5  IORT/IOHDR – challenging due to size/toxicity.
years)  Brachytherapy - toxicity high
o Impact of chemotherapy is less clear  Concurrent boost with IMRT
(compared to radiation therapy) o Defined margin risk area/meeting constraints to bowel.
o Belgium study – only treated the posterior border of
B. RETROPERITONEAL tumor
o U Ab study – SIB to 57.5 Gy
SURGICAL RESECTABLITY ISSUES o Treatment was tolerated well
 Resectable to R0 o Numbers too small and too short of FUP to know
 Vascular involvement impact.
 Organ involvement  OUTCOME: Significantly worse than other sites.
 Functional loss o Local disease free survival.
 Need for vascular or Other surgical specialty o Disease specific survival.

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Figure 7 . Outcome of IMRT use for retroperitoneal tumor.

X. TAKE HOME POINTS

1. Soft tissue sarcomas are not one but multiple different tumors,
each with special characteristics that need to help mold the
treatment and influence the patient outcome.
2. Most extremity soft tissue sarcomas rarely involve lymph nodes
and primarily metastasize to the lungs. Know the ‘exception’
sarcomas.
3. Appreciate and be able to interact with other team members in
the management of soft tissue sarcomas.
4. Preoperative radiation in extremity lesions reduces volume and
dose of radiation with equal local control and less long term
toxicity/better functional outcome.
5. Understand the approach to patients with unplanned surgical
excision of soft tissue sarcomas.
6. Be able to describe the appropriate clinical tumor volumes in
preoperative extremity and retroperitoneal soft tissue sarcomas.

X. REFERRENCES

The Management of Soft Tissue Sarcomas PPT by Ivy A. Petersen,

MD (Mayo Clinic)

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