A 39 year old woman with history of GERD presented to the ED for unretractable abdominal pain with nausea and vomiting despite chronic treatment with omeprazole. She also noted dyspnea on exertion, fatigue, and paler skin with a 80 pound weight loss over the past year. She described her vomitus as dark coffee grounds but denied hematochezia. On exam, she was afebrile, HR 110, BP 122/59, RR 24, and Sat 100%. She appeared pale and abdomen was diffusely tender to palpation, worse in the mid-epigastric region. Labs significant for hemoglobin of 2.1 g/dL, hematocrit of 9.5%, alkaline phosphatase 179 U/L, corrected calcium of 12.1 mg/dL and PTH of 299 (11-67 pg/ml). Initial CT demonstrated irregular gastric wall thickening, a 1.6 cm heterogeneous pancreatic tail mass, 7.8 cm portacaval mass, bilateral adrenal adenomas, and multiple thyroid nodules concerning for malignancy. Patient was admitted and transfused with 5 units of pRBC. Additional labs demonstrated a gastrin level >10000 (0-100 pg/ml) and chromogranin level of 92,600 (0-95 ng/ml).
Endoscopy showed a stomach lined with nodular, friable mucosa and pedunculated protruding tumors on all walls from the GEJ to the incisura. Brain MRI showed no evidence of pituitary involvement and urine catecholamines were unremarkable. Endoscopic ultrasound with FNA biopsy of portacaval/parahilar lymph node showed staining consistent with neuroendocrine tumor cells. Sestamibi scan showed parathyroid adenomas versus thyroid adenomas or carcinomas. Findings were consistent with the diagnosis of MEN1. Patient was started on high dose esomeprazole and octreotide. Subtotal parathyroidectomy was performed and subsequently developed hungry bone syndrome with persistent hypocalcemia requiring calcium citrate, calcitriol, ergocalciferol, teriparatide, and IV calcium gluconate.
Discussion: Multiple endocrine neoplasia (MEN1) syndrome presents as a rare, heritable disorder with predispositions of the parathyroid glands (95%), anterior pituitary (15-20%) and pancreatic islet cells (20-70%) with additional adenomas of the adrenal glands. Zollinger-Ellison syndrome is the most common functional pancreatic endocrine tumor in MEN1 (20-60%) with gastrin levels typically less than 1000. Medical treatment is preferred over surgery and directed at controlling gastric acid secretion with high dose proton pump inhibitors and somatostatin analogs. Parathyroid masses are surgically removed by either subtotal or total parathyroidectomy. With proper management, patients have a 15-year survival of 93%.
Conclusions: We report a severe a case of MEN1 that presented as a chronic gastrointestinal bleed. MEN1 should be considered in the presentation of a gastric tumor with suspected metastasis in the setting of hypercalcemia.
To cite this abstract:
Stormes, B; Koch, K; Byrd, R; Perkins, W . MORE THAN YOU’RE LOOKING FOR: A SEVERE PRESENTATION OF MEN1. Abstract published at Hospital Medicine 2017, May 1-4, 2017; Las Vegas, Nev.
Journal of Hospital Medicine.
2017; 12 (suppl 2).
https://www.shmabstracts.com/abstract/more-than-youre-looking-for-a-severe-presentation-of-men1/. Accessed October 17, 2019.
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