Soft Tissue Sarcomas

September 25, 2016

Introduction
Rare:

only 8300 new cases annually in U.S.

3900 die annually from STS
Mesodermal origin

Location and Type

Etiology

h/o Radiation therapy increases grade of tumors and

risk for metastasis
Chemical exposure

Thorotrast, vinyl chloride, arsenic for hepatic angiosarcoma

Genetic syndromes
Neurofibromatosis nerve sheath tumors
Familial gastrointestinal stromal tumor syndrome KIT
mutation

Skin hyperpigmentation, uticaria, cutaneous mast cell dx

Classification

Soft tissue and bone

viscera (gastrointestinal, genitourinary, and gynecologic organs)

nonvisceral soft tissues (muscle, tendon, adipose, pleura, and connective
tissue)

By differentiation (usually with IHC staining)

adipocytic tumors
fibroblastic/myofibroblastic tumors
fibrohistiocytic tumors
smooth muscle tumors
pericytic (perivascular) tumors
primitive neuroectodermal tumors (PNETs)
skeletal muscle tumors
vascular tumors
osseous tumors
tumors of uncertain differentiation

Biopsy

Most present as painless mass leading to delayed

diagnosis as lipoma or hematoma
Core needle biopsy guided by palpation or by image
guidance if not palpable

Few cases of tumor seeding with closed biopsy so some

recommend tattooing site for later excision with specimen

Excisional biopsy for superficial small lesions if

needle biopsy non-diagnostic
Incision biopsy
Longitudinal incision without tissue flaps with meticulous
hemostasis to prevent tumor seeding in hematomas
Send biopsy fresh and orientated

Tumor seeding after biopsy

Imaging
MRI
For

extremity masses
Gives good delineation between muscle, tumor and
blood vessels

CT

for abdominal and retroperitoneal

PET
May

help determine high vs. low grade

May be helpful in recurrences

Staging

AJCC/UICC Staging System for Soft Tissue Sarcomas

T1: <5cm

T2: >5cm

T1a: superficial to muscular fascia

T1b: Deep to muscular fascia
T2a: superficial to muscular fascia
T2b: Deep to muscular fascia

N1: Regional nodal involvement

Grading

G1:
G2:
G3:
G4:

Well-differentiated
Moderately differentiated
Poorly differentiated
Undifferentiated

Staging
Stage IA

G1,2

T1a,b

N0

M0

Stage IB

G2,2

T2a,b

N0

M0

Stage IIA

G3,4

T1a,b

N0

M0

Stage IIB

G3,4

T2a

N0

M0

Stage III

G3,4

T2b

N0

M0

Stage IV

Any G

Any T

N1

M1

Staging system predicts survival and risk of metastasis, but not local recurrence
**Does not take into account extremity vs. visceral

Survival by stage

Relative risk for recurrence and

survival

Age >50 years

Local recurrence at presentation
Microscopically positive margin
Size 5.010.0 cm
Size > 10.0 cm
High-grade
Deep location
Local recurrence

1.6
2.0
1.8
1.9
1.5
4.3
2.5
1.5

Surgery
Limb-sparing

vs amputation

Comparison

study with post-op radiation in limb sparing

showed no difference in survival

Amputation

still may be indicated for

neurovascular or bone involvement

Resection
Arbitrary

2 cm margin if no plan for post-op

radiotherapy
Negative margins may be adequate for postop radiation therapy
Presence

of positive margins increases local recurrence

by 10-15%

No

need for lymph node dissection as only 23% have nodal metastasis

Adjuvant radiotherapy
Small,

low grade tumors resected with 2 cm

margins may not require radiation
Improves local control but not survival
Whether improved local control leads to
improved survival is controversial

Local recurrence with post-op

brachytherapy

Pre-op or post-op radiation?

Some

avoid pre-op use because of

increased wound complications (although
this is debatable)
RCT

looking at wound complication rate pre-op vs postop radiation showed 35% vs 17%

Risk confined to lower extremity

Conclusions:

pre-op may be better for upper extremity

and head & neck because of equal wound complication
risk and benefit of lower radiation doses to more vital
tissues

Pre-op vs post-op radiotherapy

Chemotherapy
Can

improve local control, but not survival

Doxorubicin and ibosfamide have response
rates of 20%
Use only in advanced disease
Combination with radiation or neoadjuvant
therapy are controversial
Hypothermic isolated limb perfusion may be
used for palliation

Treatment of Recurrence
20-30%

of STS patients will recur

More common in retroperitoneal and head &
neck high grade tumors because hard to get
clear margins
38%

for retroperitoneal
42% for head and neck
5-25% for extremity

After

re-resection recurrence is 32% for

extremity and much higher for visceral

Metastatic disease
Lung

most common site of mets, but visceral

often go to liver
Median survival from development of
metastatic disease is 8-12 months
Resection of pulmonary mets can give 5 year
survival of 32% if all mets can be removed
>3

mets is poor prognosticator

Case #1
64

y/o male with increasing abdominal girth

Retroperitoneal Sarcomas

15% of all sarcomas

Liposarcoma 42% and leiomyosarcoma 26%
CT scan can show cystic/solid/necrotic components and relation
to surroundings
CXR to r/o mets, chest CT if CXR abnormal
Biopsy not necessary unless suspect a lymphoma or germ cell
tumor or plan preop chemo or radiation
En bloc resection is standard treatment

bowel prep
assess bilateral kidney function
50-80% need organ resection
78% of primary lesions can be completely resected

Liposarcoma

Survival after resection of primary

retroperitoneal sarcoma

Prognosis for retroperitoneal sarcomas

5

year survival after complete resection of

54-65%
Drops

to 10-36% if incompletely resected

Recurrence

occurs in 46-59% of completely

resected tumors

Radiation or chemotherapy for

retroperitoneal sarcomas
Radiation

GI and neurotoxicities limit delivery of sufficient

doses
May improve local control
Recommended for use only in clinical trials given
lack of data either way

Chemotherapy

Use for recurrent, unresectable or metastatic

disease

Case #2
49 y/o female with GERD undergoing EGD

GIST

Separate subtype of sarcoma defined by expression

of c-Kit (CD117)
Surgery: complete resection without local or regional
lymphadenectomy
Very resistant to traditional chemotherapy
Gleevec (imantinib mesylate)
c-Kit is constitutively active tyrosine kinase receptor
Drug is tyrosine kinase inhibitor used in CML
Initial studies showed 54% response rates
Two RCTs currently looking at adjuvant treatment

GIST

GIST

Extremity sarcomas

Synovial sarcoma
MFH

Breast sarcomas
1%

of all breast neoplasms

Wide excision with negative margins
No clear role for adjuvant radiotherapy

Sarcoma after mastectomy

Vascular sarcomas
Angiosarcoma,

hemangiosarcoma,
lymphangiosarcoma, hemangiopericytoma
Key points:
Hepatic

angiosarcoma thorotrast, vinyl chloride,

arsenic
Stewart Treves lymphangiosarcoma in chronic
lymphedema

High

risk for bleeding during excision

No clear role for chemo or radiation