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Soft Tissue Sarcomas

Soft Tissue Sarcomas

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Soft Tissue Sarcomas

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  1. Soft Tissue Sarcomas April 1, 2014

  2. Introduction • Rare: only 8300 new cases annually in U.S. • 3900 die annually from STS • Mesodermal origin

  3. Location and Type

  4. 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

  5. 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

  6. 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

  7. Tumor seeding after biopsy

  8. 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

  9. Staging • AJCC/UICC Staging System for Soft Tissue Sarcomas • T1: <5cm • T1a: superficial to muscular fascia • T1b: Deep to muscular fascia • T2: >5cm • T2a: superficial to muscular fascia • T2b: Deep to muscular fascia • N1: Regional nodal involvement • Grading • G1: Well-differentiated • G2: Moderately differentiated • G3: Poorly differentiated • G4: Undifferentiated

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

  11. Survival by stage

  12. Relative risk for recurrence and survival • Age >50 years 1.6 • Local recurrence at presentation 2.0 • Microscopically positive margin 1.8 • Size 5.0–10.0 cm 1.9 • Size > 10.0 cm 1.5 • High-grade 4.3 • Deep location 2.5 • Local recurrence 1.5

  13. 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

  14. Resection • Arbitrary 2 cm margin if no plan for post-op radiotherapy • Negative margins may be adequate for post-op radiation therapy • Presence of positive margins increases local recurrence by 10-15% • No need for lymph node dissection as only 2-3% have nodal metastasis

  15. 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

  16. Local recurrence with post-op brachytherapy

  17. 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 post-op 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

  18. Pre-op vs post-op radiotherapy

  19. 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

  20. 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

  21. 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

  22. Case #1 • 64 y/o male with increasing abdominal girth

  23. 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

  24. Liposarcoma

  25. Survival after resection of primary retroperitoneal sarcoma

  26. 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

  27. 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

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

  29. 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

  30. GIST

  31. GIST

  32. Extremity sarcomas Synovial sarcoma MFH

  33. Breast sarcomas • 1% of all breast neoplasms • Wide excision with negative margins • No clear role for adjuvant radiotherapy

  34. Sarcoma after mastectomy

  35. Vascular sarcomas • Angiosarcoma, hemangiosarcoma, lymphangiosarcoma, hemangiopericytoma • Key points: • Hepatic angiosarcoma – thorotrast, vinyl chloride, arsenic • Stewart Treve’s – lymphangiosarcoma in chronic lymphedema • High risk for bleeding during excision • No clear role for chemo or radiation