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Multidisciplinary GI Conference 10.25.07

Case Presentation. 62 yo wf with two major acute GI bleedsPMH history notable for:Hodgkin's lymphoma treated with mantle radiationBreast cancer, s/p mastectomyS/p AoV replacement, MV repair, CABG 1 year agoS/p appendectomy and tubal ligationSmoker. Case Presentation. Upper endoscopy and colonoscopy negative.

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Multidisciplinary GI Conference 10.25.07

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    1. Multidisciplinary GI Conference 10.25.07

    2. Case Presentation 62 yo wf with two major acute GI bleeds PMH history notable for: Hodgkin’s lymphoma treated with mantle radiation Breast cancer, s/p mastectomy S/p AoV replacement, MV repair, CABG 1 year ago S/p appendectomy and tubal ligation Smoker

    3. Case Presentation Upper endoscopy and colonoscopy negative

    5. Capsule

    11. Path Summary in the Case Two foci of carcinoid tumor (0.8 cm and 0.5 cm) Both tumors involve submucosa No angiolymphatic invasion 4 mesenteric lymph nodes negative

    15. “Carcinoid” It’s like a carcinoma, ….but it’s NOT a carcinoma

    16. Carcinoid Pathologic Definition: well-differentiated neuroendocrine tumor of the dispersed endocrine system (i.e., outside of endocrine organs)

    17. Neuroendocrine Tumors Neuroendocrine tumors come in two varieties: a) tumors of the endocrine organs (pancreas, adrenal) b) tumors of the dispersed endocrine cells ( GI tract, bronchopulmonary, C cells of the thyroid) “Carcinoid” refers to neuroendocrine tumors of the dispersed endocrine system ONLY

    18. Classification of NE Tumors Well-differentiated NE tumors = Carcinoids Benign behavior or uncertain malignant potential Well-differentiated NE carcinomas (atypical carcinoids) Low-grade malignancy Poorly differentiated NE carcinomas (usually small cell) High-grade malignancy

    19. GI Carcinoids Well-differentiated NE tumors = Carcinoids Benign behavior or uncertain malignant potential Poorly differentiated NE carcinomas (small cell) High-grade endocrine neoplasm

    20. Classification of NE Tumors GI TRACT Well-differentiated NE tumors = Carcinoids Benign behavior or uncertain malignant potential Poorly differentiated NE carcinomas (small cell) High-grade endocrine neoplasm

    21. GI Carcinoids Easily recognized, well-defined shared morphologic features Tumor’s cell type corresponds to distribution in the GI tract of its normal counterpart Morphology, hormone production, and behavior can be predicted by tumor’s location Many are associated with known risk factors

    22. GI Carcinoids Morphology Located in mucosa and submucosa Arise at the base of the crypts Nested growth pattern (insular, acinar, trabecular) Small uniform cells Round regular nuclei Finely stippled “salt & pepper” chromatin Lack of prominent nucleoli and mitotic activity Stain with neuroendocrine markers

    23. GI Carcinoids IHC Shared Neuroendocrine Markers Chromogranin Synaptophysin Specific Neuroendocrine Hormones Gastrin (G cells) Somatostatin (D cells) Serotonin (EC cells) Glucagon (L cells)

    24. GI Carcinoids Prediction of Behavior Size is the best indicator Smaller than 1 cm unlikely to spread Larger than 2 cm usually aggressive Other Brisk mitotic rate Necrosis Deep invasion Site (small bowel)

    25. GI Neuroendocrine Cells Distribution Gastric body: ECL (enterochromaffin-like) cells Histamine Antrum and Duodenum: G cells Gastrin Duodenum: D cells Somatostatin Bowel: EC (enterochromaffin) cells Serotonin Stomach and bowel: L cells Enteroglucagon

    26. GI Carcinoid as: distinct individuals Foregut carcinoids Gastric, duodenal Midgut carcinoids Ileal/Jejunal Appendiceal Hindgut carcinoids Rectal

    27. Behavior of GI Carcinoids by Site Foregut carcinoids Gastric (10%), duodenal (<5%). Generally low malignant potential Midgut carcinoids Ileal/Jejunal (25%). 60% malignant Appendiceal (40%). 1% malignant Hindgut carcinoids Rectal (20%), 15% malignant

    28. Foregut Carcinoids: Gastric Type I High Gastrin State Type II High Gastrin State Type III

    29. What are the causes of high gastrin levels? Type I Atrophic Gastritis, Autoimmune Gastritis Type II ZE, MEN (type 1) ECL cells develop into carcinoids after chronic stimulation by high gastrin levels Clinical course is usually indolent (if <1 cm)

    31. Foregut Carcinoids, cont.: Type III. Gastrin-independent Sporadic Account for 20% of gastric carcinoids Most aggressive Metastatic in 60% at resection May produce 5-HT and cause carcinoid syndrome

    32. Appendiceal Carcinoids Typically benign course 1/300 appendices contains a carcinoid tumor Often found incidentally Goblet cell carcinoid variety makes serotonin and is more aggressive

    33. Small Bowel Carcinoids Most aggressive May present with obstruction or abdominal pain due to intussusception, mechanical effect of the tumor, or mesenteric ischemia due to local fibrosis or angiopathy Carcinoid syndrome present in up to 10% patients Multiple tumors in up to 30% of patients (worse prognosis)

    34. Colon Carcinoids Usually right colon, particularly cecum Carcinoid syndrome is rare

    35. Rectal Carcinoids Size correlates with metastases: <1 cm - rare; > 2 cm (>70%) Carcinoid syndrome is rare Local excision of small carcinoids Extensive excision of larger (similar to adenocarcinoma) Controversial for > 1 cm, < 2 cm

    36. Carcinoid Syndrome

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