PMID- 19039643 OWN - NLM STAT- MEDLINE DCOM- 20090326 LR - 20211020 IS - 0941-1291 (Print) IS - 0941-1291 (Linking) VI - 38 IP - 12 DP - 2008 TI - Cervical metastases of glucagonoma in a patient with multiple endocrine neoplasia type 1: report of a case. PG - 1137-43 LID - 10.1007/s00595-008-3763-1 [doi] AB - Multiple endocrine neoplasia type 1 (MEN 1) is a syndrome characterized by tumors of the parathyroid glands, pancreatic islet cells, duodenum, and pituitary gland. We report a case of cervical metastases of glucagonoma with MEN 1. The patient was a 34-year-old woman admitted to our hospital with epigastric pain. Her medical history included two resections of prolactinoma and two upper GI hemorrhages secondary to duodenal ulcers. Computed tomography (CT) showed two hypervascular lesions in the tail of the pancreas and cervical ultrasound showed multiple hypoechogenic ovoid images in the neck. A cervical CT scan confirmed two 15-mm lymph nodes in the left cervical region and 111In-DOTATOC imaging showed focal abnormal somatostatin expression in the pancreatic tail and the cervical nodes. The patient had asymptomatic hypoglycemic episodes, with blood sugar levels as low as 30 mg/dl, which raised our suspicion of MEN 1 associated with pancreatic insulinoma. Thus, we performed a distal pancreatectomy with bilateral cervical dissection and parathyroid gland resection. Histopathological examination revealed 12 pancreatic tumors as well as metastases in four cervical lymph nodes. The resected parathyroid glands had normal structure, suggesting parathyroid hyperplasia. A follow-up CT scan, 18 months after surgery, showed new tumors in the head of the pancreas and in the duodenal wall. A pancreatoduodenectomy was performed and histopathological examination revealed nine nonfunctioning endocrine tumors in the pancreas, one tumor in the duodenal wall, and metastases in two peripancreatic lymph nodes. The patient recovered well and remains asymptomatic. FAU - Butte, Jean M AU - Butte JM AD - Department of Digestive Surgery, Pontificia Universidad Catolica de Chile, Marcoleta 367, Santiago, Chile. FAU - Montero, Pablo H AU - Montero PH FAU - Solar, Antonieta AU - Solar A FAU - Torres, Javiera AU - Torres J FAU - Olmos, Pablo R AU - Olmos PR FAU - Goni, Ignacio AU - Goni I FAU - Quintana, Juan C AU - Quintana JC FAU - Martinez, Jorge AU - Martinez J FAU - Llanos, Osvaldo AU - Llanos O LA - eng PT - Case Reports PT - Journal Article DEP - 20081128 PL - Japan TA - Surg Today JT - Surgery today JID - 9204360 SB - IM MH - Adult MH - Cholecystectomy MH - Duodenal Neoplasms/pathology/secondary MH - Female MH - Glucagonoma/metabolism/*pathology/surgery MH - Humans MH - Hyperplasia MH - Hypoglycemia/etiology MH - Immunohistochemistry MH - Lymphatic Metastasis MH - Multiple Endocrine Neoplasia Type 1/*pathology MH - Neck MH - Pancreatectomy MH - Pancreatic Neoplasms/metabolism/*pathology/surgery MH - Pancreaticoduodenectomy MH - Parathyroid Glands/pathology/surgery MH - Splenectomy EDAT- 2008/11/29 09:00 MHDA- 2009/03/27 09:00 CRDT- 2008/11/29 09:00 PHST- 2007/09/11 00:00 [received] PHST- 2008/01/09 00:00 [accepted] PHST- 2008/11/29 09:00 [pubmed] PHST- 2009/03/27 09:00 [medline] PHST- 2008/11/29 09:00 [entrez] AID - 10.1007/s00595-008-3763-1 [doi] PST - ppublish SO - Surg Today. 2008;38(12):1137-43. doi: 10.1007/s00595-008-3763-1. Epub 2008 Nov 28.