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Sarcomas -- Evaluation. Often is referred as “lipoma” or a “trauma” Size: <5 , > 5, > 10 cm Location: Extremity --> lung deep to fascia more often high grade Head and neck --> lung GI or retroperitoneal --> liver. Sarcoma -- Nodal Metastases. Nodal metastases are rare
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Sarcomas -- Evaluation • Often is referred as “lipoma” or a “trauma” • Size: • <5 , > 5, > 10 cm • Location: • Extremity --> lung • deep to fascia more often high grade • Head and neck --> lung • GI or retroperitoneal --> liver
Sarcoma -- Nodal Metastases • Nodal metastases are rare • Clinical assessment only • Stage IV when present • No special efforts for node basin resection
Sarcomas -- Pre-op imaging • CT scan or MRI • Plan for post-op radiation bed (if needed) • Allow 3-D visualization of tumor for resection • Extremity or head and neck: • CXR (if low grade sarcoma on biopsy) • CT chest (if high grade sarcoma on biopsy)
Sarcomas -- Biopsy • Tru-Cut Core biopsy • CT guided if intra- or retro-peritoneal • Open biopsy • Incisional only • Longitudinal • Biopsy incision must be excised at final excision • FNA inadequate
Sarcomas -- Staging • Stage I • Grade 1 (G1) -- Well differentiated • Stage IA -- < 5cm • Stage IB -- > 5 cm • Stage II • Grade 2 (G2) -- Moderately well differentiated • Stage IIA -- < 5cm • Stage IIB -- > 5 cm
Sarcomas -- Staging • Stage III • Poorly differentiated • Stage IIIA -- < 5cm • Stage IIIB -- > 5 cm • Stage IV • Nodal (IVA) or distant (IVB) metastases • Any grade and any size
Sarcomas -- Neoadjuvant chemo • More effective in children • Ifosfamide and adriamycin (or epirubicin) • Chemo mainstay in some sarcomas • Ewing’s sarcoma, embryonal rhabdomyosarcoma, most osteogenic sarcomas
Sarcomas -- Neoadjuvant chemo • Controversial in many sarcomas • High grade sarcomas -- some sensitive • MFH, liposarcoma, synovial cell sarcoma • High grade sarcomas -- some resistant • GIST, leiomyosarcoma, epithelioid, hemangiopericytoma, extraskeletal myxoid chrondrosarcomas
Sarcomas -- Neoadjuvant chemo • Allows chemosensitivity assessment pre-op • Allows pathologic chemo-necrosis assessment • Micro-metastasis treatment earlier • Surgical bulk reduction • Important in tumors > 10 cm
Sarcomas -- Radiation • Intermediate or high grade sarcomas • Extremity • Head and Neck • No difference in pre-op or post-op • Wide field, 5000 - 5500 Gy • Spare area of skin to prevent circumferential stricture
Sarcomas -- Radiation • Brachytherapy -- iridium • Catheters should be placed at time of resection
Sarcomas -- Surgery • 2 to 3 cm margins -- do not violate tumor • Some recommend 3 - 5 cm -- microsatellites • One uninvolved fascial plane • Include skin, SQ tissue, adjacent soft tissue, and incision site, sometimes periosteum, bone ctx. • Preserve neurovascular bundle if low grade • Excise adventitia of artery, vein, nerve if necessary • Frozen section of margins as needed
Sarcomas -- Surgery • With post-op radiation, full compartment resection not necessary • Function and limb sparing achievable with post-op radiation • Post-op coverage • Flaps • Eliminate dead space, compression, drains
Retroperitoneal Sarcoma -- Surgery • 45 degree positioning in OR • Incision -- 11th rib extended to paramedian • Rotate colon, duodenum (Kocher), or other viscera • Sacrifice kidney, involved organs as needed
Retroperitoneal sarcoma -- radiation • Vena Cava -- radiation helpful ?
Intra-abdominal Sarcomas -- Surgery • Total gastrectomy not necessary • Adequate margins needed, however
Sarcomas -- Metastasis treatment • Metastases seen in 25% of sarcomas • Lungs are site of metastases in 75%
Sarcomas -- Metastasis treatment • Lung -- improved outcome with resection • Resect isolated/localized met (s) only • Multiple met resection in experimental settings only • Liver -- few survivors • Do not resect • Chemoembolization, cryoablation, RF ablation
Sarcomas -- Recurrence treatment • Recurrence in 1/3 of sarcoma patients • Re-resect with appropriate margins • 20 - 30% of extremities require amputation
Sarcomas -- Survival • Small low-grade tumors -- > 90% disease free survival • Large, high grade tumors -- 20 - 30% overall survival
Sarcomas -- Follow-up • Extremity and head and neck • CXR and extremity MRI/CT every 6 months for 5 years • Retroperitoneal and intra-abdominal • CT every 6 months for 3 years then yearly
Sarcomas -- Screening • Neurofibromatosis (vonRecklinghausen) • 3-10% lifetime risk • Familial adenomatous polyposis • Li-Fraumeni (p53) -- mother at risk for breast CA • Retinoblastoma, HIV (Kaposi’s) • Prior radiation (radium, thorium) • Arsenic, dioxin (Agent Orange)