Soft Tissue Sarcoma

Soft Tissue SarcomaSoft Tissue Sarcoma

Craig Blum, M.D.Craig Blum, M.D.

Soft Tissue SarcomaSoft Tissue Sarcoma

Arise from Arise from embyronic embyronic mesodermmesoderm

Ecoderm- skinEcoderm- skin Endoderm- guts Endoderm- guts Mesoderm- Mesoderm-

skeleton and skeleton and muscles, muscles, connective tissueconnective tissue

Histologic TypesHistologic Types OsteosarcomaOsteosarcoma ChondrosarcomaChondrosarcoma GISTGIST Ewing SarcomaEwing Sarcoma

FibrosarcomaFibrosarcoma LiposarcomaLiposarcoma RhabdomyosarcomaRhabdomyosarcoma Epitheloid SarcomaEpitheloid Sarcoma Desmoplastic small Desmoplastic small

round cell tumorround cell tumor

IncidenceIncidence

Rare tumorsRare tumors 2007:2007:

• 9200 US9200 US• 3560 deaths3560 deaths

colon 112,000colon 112,000 lung 214,000lung 214,000

EXT

RP

TRUNK

VISCERA

OTHER

CauseCause

Unclear:Unclear:• Genetic factors:Genetic factors:

chromosomal chromosomal abnormalitiesabnormalities

• Radiation: Radiation: long latencylong latency

• Chemical exposure:Chemical exposure:industrial industrial

agentsagents• Lymphedema:Lymphedema:

**absite**absite lymphangiosarcomaslymphangiosarcomas

ClassificationClassification

Best predictor of a sarcoma’s biologic Best predictor of a sarcoma’s biologic aggressiveness / metastatic potential aggressiveness / metastatic potential is GRADEis GRADE• Cellularity, nuclear atypia, degree of Cellularity, nuclear atypia, degree of

necrosis, mitotic activity. necrosis, mitotic activity. • Subjectivity (40%)Subjectivity (40%)

ClinicalClinical

Asymptomatic Asymptomatic massmass• 38% large than 38% large than

10cm at 10cm at presentationpresentation

Imaging: Imaging: • MRI versus CTMRI versus CT• PETPET

PearlPearl

Few sarcomas Few sarcomas metastasize to metastasize to lymph nodeslymph nodes

Extremity Extremity Lung Lung Abdominal Abdominal Liver Liver

then lung then lung

BiopsyBiopsy

<5 cm: excisional <5 cm: excisional biopsy with clear biopsy with clear marginsmargins

>5cm: incisional or >5cm: incisional or core needle bx core needle bx (CNB)(CNB)

Don’t use FNA bc Don’t use FNA bc sample too smallsample too small

TreatmentTreatment

Surgical Tx is Surgical Tx is foundation of foundation of treatment of STStreatment of STS

LSS standard of careLSS standard of care Amputation reserved Amputation reserved

for cancers that for cancers that involve major vessels, involve major vessels, bones, nerves such bones, nerves such that function would be that function would be compromised. compromised.

Surgical ManagementSurgical Management

Goal margin 1 -2 cm when possible. Goal margin 1 -2 cm when possible. Regional lymphadenectomy is NOT Regional lymphadenectomy is NOT

usually indicated bc only 2.6% usually indicated bc only 2.6% sarcomas metastasize to LNsarcomas metastasize to LN• RhabdomyosarcomaRhabdomyosarcoma• Epitheloid sarcoma (CT of hands)Epitheloid sarcoma (CT of hands)• Clear cell sarcoma (tendons)Clear cell sarcoma (tendons)• Synovial sarcomaSynovial sarcoma• Vascular sarcomaVascular sarcoma

RadiationRadiation

Take home:Take home:• [prospective, randomized trails] [prospective, randomized trails]

demonstrated adjuvant radiotherapy demonstrated adjuvant radiotherapy helped prevent local recurrence in pts helped prevent local recurrence in pts with high grade STS.with high grade STS.

• However, did not demonstrate improved However, did not demonstrate improved overall survival. overall survival.

• [retrospective studies] <5cm (small) [retrospective studies] <5cm (small) STS may be resected with negative STS may be resected with negative margins alone. margins alone.

ChemoChemo

Take home: Take home: • ““At present, given the uncertainty At present, given the uncertainty

regarding its efficacy, postoperative regarding its efficacy, postoperative adjuvant chemotherapy for treatment of adjuvant chemotherapy for treatment of STS is probably best employed in the STS is probably best employed in the context of appropriate clinical trials.”context of appropriate clinical trials.”

• Preop “theoretical advantages” but Preop “theoretical advantages” but again, insufficient evidence for any again, insufficient evidence for any survival benefit.survival benefit.

Targeted TherapyTargeted Therapy

Targeted tyrosine Targeted tyrosine kinase receptor c-kinase receptor c-kit for treating GISTkit for treating GIST

Hyperthermic Isolated Limb Hyperthermic Isolated Limb PerfusionPerfusion

Pts where LSS is Pts where LSS is not possible not possible initiallyinitially

May make it May make it possible to resect possible to resect with a negative with a negative margin.margin.

Not well Not well established in USestablished in US

Metastatic DiseaseMetastatic Disease

Pulmonary mets Pulmonary mets are present in 20% are present in 20% of pts with trunk of of pts with trunk of extremity extremity sarcomas. sarcomas. • Medically fit, no Medically fit, no

extrathoracic dx, extrathoracic dx, mets resectable mets resectable pulmonary pulmonary metastasectomy.metastasectomy.

MSKCC studyMSKCC study

RhabdomyosarcomaRhabdomyosarcoma

most common soft most common soft tissue sarcoma in tissue sarcoma in infants and infants and childrenchildren• head and neck; head and neck;

around the eyes -- around the eyes -- 35-40% 35-40%

• GU tract -- 20% GU tract -- 20% • extremities -- 15-extremities -- 15-

20% 20% • trunk -- 10-15% trunk -- 10-15%