MEN I - TAddona - MultipleEndocrineNeoplasia...

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Multiple Endocrine Neoplasia  Multiple Endocrine Neoplasia  (MEN)  I (MEN)  I
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Case Presentation Case Presentation 62 y/o Female Pmhx - HTN & Cholecystectomy for biliary colic (1995) Presents to OSH for abd pain, N/V. Afeb, mild right sided abd tenderness. Elevated LFTs. ERCP performed for presumed retained stone – sphincterotomy w/ stent placement. Post ERCP cont abd pain & developed MI
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Transferred to MSH for cardiac cath/stent post – cath cont abd pain – GI workup begun 9/4 CT …
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Biochemical w/u for pheochromocytoma found to be neg (nl urine catecholamines & nl normetanephrine / metanephrine) Biochemical w/u for poss neuroendocrine pancreatic lesion – gastrin mild elev. (172/111); mild elev. chromogranin A (38/18); nl glucagon Pt also at this time reported FMHx of multiglandular hyperparathyroid in mother; labs drawn – hypercalcemic, elevated PTH; elevated prolactin Head CT negative – pt not candidate for MR (metal stent)
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9/9 SBS negative 9/11 & 9/12 Capsule endoscopy performed – capsule held up in antrum of stomach – EGD removal of capsule and placement of second capsule in duodenum Surgery consulted – N/V; Afeb normal VS; lower abdominal tenderness, nl lactate, mild levated WBC (12-13) 9/14 repeat CT showed
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metallic density in RLQ – capsule; cont wall thickening 9/17 Pt to OR for Small bowel resection/retrieval of capsule
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Pathology – Segment of sm bowel w/ ischemic necrosis & carcinoid tumor (1cm) extending into muscularis propria
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MULTIPLE ENDOCRINE MULTIPLE ENDOCRINE NEOPLASIA: MEN I SYNDROME NEOPLASIA: MEN I SYNDROME
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Contents Contents 1. Parathyroid disease 2. Anterior Pituitary Adenomas 3. Entero-pancreatic tumors:  Insulinomas  Gastrinomas  Glucagonomas  VIPomas  Somatostatinomas  Pancreatic Polypeptide-Producing tumors  Rare Islet Cell tumors  Surgical Therapy 4. Other tumors
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What is MEN–1 ? What is MEN–1 ? AD disorder on chromosome 11 Consensus recommends that MEN- 1 defined as 2/3 main MEN-1 tumor types Familial MEN-1 is at least one MEN-1case plus first degree relative w/ 1/3 tumors
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Primary Hyperparathyroidism Primary Hyperparathyroidism ~ 100% penetrance – earliest / most common  manifestation 1-2 % all hyperparathyroidism is MEN-1 Multiglandular involvement (unlike sporadic hyperPTH  w/ single gland adenoma) Diagnosing: Clinically - Stones, bones, groans, mental overtones Diagnosis – Biochemical =hypercalcemia w/ hi serum  PTH Imaging – sestamibi & bone densitometry
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MEN I - TAddona - MultipleEndocrineNeoplasia...

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