Soft Tissue Sarcomas
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Soft Tissue Sarcomas

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Soft Tissue Sarcomas Soft Tissue Sarcomas Presentation Transcript

  • Soft Tissue Sarcomas June 8, 2009
  • 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
          • 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
  • Staging **Does not take into account extremity vs. visceral Staging system predicts survival and risk of metastasis, but not local recurrence M1 N1 Any T Any G Stage IV M0 N0 T2b G3,4 Stage III M0 N0 T2a G3,4 Stage IIB M0 N0 T1a,b G3,4 Stage IIA M0 N0 T2a,b G2,2 Stage IB M0 N0 T1a,b G1,2 Stage IA
  • Survival by stage
  • 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
  • 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 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
  • 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 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
  • 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 MFH Synovial sarcoma
  • 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 Treve’s – lymphangiosarcoma in chronic lymphedema
    • High risk for bleeding during excision
    • No clear role for chemo or radiation