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Retroperitoneal Sarcoma (RS). Hashmi. approximately 15% of all sarcomas approximately 33% to 55% of all retroperitoneal tumors malignant tumors arising from mesenchymal cells located in adipose, muscle, connective tissue, or soft tissue contrast to carcinomas

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slide2
approximately 15% of all sarcomas
  • approximately 33% to 55% of all retroperitoneal tumors
  • malignant tumors arising from mesenchymal cells
    • located in adipose, muscle, connective tissue, or soft tissue
    • contrast to carcinomas
      • epithelial origin (breast, colon, pancreas, etc)
  • metastasize via hematogenous route
    • typically to the liver or lung
  • most common variants of RS
    • liposarcoma (40%)
    • leiomyosarcoma (30%)
    • malignant fibrous histiocytoma (<10%)
slide3
most common clinical presentation is an abdominal mass
    • non-specific, vague abdominal discomfort or pain, weight loss, and early satiety, intestinal obstruction or bleeding, lower extremity swelling or pain
  • computed tomography (CT) is the initial test of choice, then MRI
    • tumor location and size
    • relationship to surrounding anatomic structures
    • identification of metastatic lesions
  • differential diagnosis
    • germ cell tumor, teratoma, cyst, hematoma and abscess, functioning and non-functioning adrenal tumor, renal tumor, pancreatic tumor, advanced gastrointestinal carcinoma, soft tissue sarcoma, lymphoma, and other neoplasms
slide4
surgery is the standard treatment
  • surgical failures
    • large tumor size on presentation
    • inability to achieve wide surgical margins
    • limitations of existing adjuvant radiation and chemotherapy regimens
  • failure can be seen even 5 to 10 years in nearly 90% of patients
  • long-term recurrence rate is thought to be over 70%
  • prognosis is worse for RS than for other trunk or extremity sarcomas
  • >30% incidence of vascular involvement
  • >60% of cases of these lesions require multi-visceral resections
    • colon, kidney, small bowel, pancreas, bladder
slide5
Low grade sarcomas
    • treated surgically
    • adjuvant radiation therapy or chemotherapy
  • High grade sarcomas
    • neoadjuvant chemotherapy
    • these tumors are more likely to undergo metastasis
    • tumors are treated more aggressively
slide6
neo-adjuvant chemotherapy may be used in an attempt to ‘shrink’ the tumor
  • postoperative radiotherapy may be of benefit
  • adjuvant chemotherapy + surgical therapy may produce a modest improvement in the recurrence-free survival rate
  • recurrences can be treated with resection in the absence of metastasis
  • adjuvant treatment following re-resection should be considered for all patients who did not previously undergo chemotherapy
  • pulmonary metastasis have median survival of 6 to 12 months
    • pulmonary metastasis resection may associated with prolonged survival
  • hepatic metastasis survival rates have been lesser
  • unresectable metastatic disease
    • chemotherapy may provide some clinical efficacy
slide7
surveillance is based on low vs high grade tumor
  • low-grade disease
    • physical examination
    • ct scan of chest, abdomen, and pelvis
    • every 3 to 6 months for 2 to 3 years, then annually
  • high-grade disease
    • physical examination
    • ct scan of chest, abdomen, and pelvis
    • every 3 to 4 months for 3 years, then every 6 months for 2 years, then annually
gastrointestinal stromal tumors gist
gastrointestinal stromal tumors (GIST)
  • 1-3% of all gastrointestinal malignancies
  • GIST is a form of connective tissue cancer/sarcoma - a non-epithelial tumor
    • 70% occur in the stomach
    • 20% in the small intestine
    • <10% in the esophagus
  • characteristics of GISTs
    • spindle cells in 70-80%, epitheloid aspect in 20-30%
  • immunohistochemistry
    • antibodies that stain the molecule CD117 (also known as c-kit)
  • GISTs are thought to arise from interstitial cells of Cajal (ICC)
    • normally part of the autonomic nervous system of the intestine
    • pacemaker function in controlling motility
  • Most small GISTs (<5 and especially <2 cm) with a low rate of mitosis (<5 dividing cells per 50 HPF) are benign
    • surgery
    • do not require adjuvant therapy
  • Larger GISTs (>5 cm), and especially when the cell division rate is high (>6 mitoses/50 HPF) may be more malignant
    • neoadjuvant + surgery +/- adjuvant
  • c-kit tyrosine kinase inhibitor  imatinib (Glivec/Gleevec)