PMID- 19350420 OWN - NLM STAT- MEDLINE DCOM- 20091001 LR - 20211020 IS - 1355-008X (Print) IS - 1355-008X (Linking) VI - 36 IP - 1 DP - 2009 Aug TI - Co-existence of glucagonoma with recurrent insulinoma in a patient with multiple endocrine neoplasia-type 1 (MEN-1). PG - 20-4 LID - 10.1007/s12020-009-9183-1 [doi] AB - Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by tumors of the parathyroid glands, the anterior pituitary, and the endocrine pancreas. Our patient was a 58-year-old man who manifested typical features of MEN-1 including primary hyperparathyroidism, lung carcinoid, and lipomas and insulinoma. He was admitted to our hospital because of recurrent hypoglycemia and a growth of pancreatic tumors. The first operation for insulinoma was performed when he was 20 years old. We found a germline mutation of the MEN1 gene (E45G, exon 2) in this patient. According to these examinations and his clinical course, the patient was diagnosed as having a recurrence of insulinoma. He subsequently underwent surgery for the pancreatic tumors. The majority of these tumor cells were immunohistochemically positive for insulin and negative for glucagon. A few nodules showed immunohistochemical staining positivity for glucagon but they were negative for insulin. Although it is uncommon for patients with MEN1 to exhibit insulinoma and glucagonoma, this case suggests the need for careful analysis of pancreatic tumors in patients with MEN1. FAU - Nishiuchi, Takamasa AU - Nishiuchi T AD - Department of Internal Medicine, Faculty of Medicine, Kagawa University, 1750-1, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan. FAU - Imachi, Hitomi AU - Imachi H FAU - Murao, Koji AU - Murao K FAU - Fujiwara, Mako AU - Fujiwara M FAU - Muraoka, Tomie AU - Muraoka T FAU - Kikuchi, Fumi AU - Kikuchi F FAU - Nishiuchi, Yukiko AU - Nishiuchi Y FAU - Kushida, Yoshio AU - Kushida Y FAU - Haba, Reiji AU - Haba R FAU - Ishida, Toshihiko AU - Ishida T LA - eng PT - Case Reports PT - Journal Article DEP - 20090407 PL - United States TA - Endocrine JT - Endocrine JID - 9434444 RN - 0 (MEN1 protein, human) RN - 0 (Proto-Oncogene Proteins) SB - IM MH - Glucagonoma/genetics/*pathology MH - Humans MH - Insulinoma/genetics/*pathology MH - Male MH - Middle Aged MH - Multiple Endocrine Neoplasia Type 1/genetics/*pathology MH - Neoplasm Recurrence, Local/genetics/pathology MH - Pancreatic Neoplasms/genetics/*pathology MH - Proto-Oncogene Proteins/genetics EDAT- 2009/04/08 09:00 MHDA- 2009/10/02 06:00 CRDT- 2009/04/08 09:00 PHST- 2008/12/20 00:00 [received] PHST- 2009/03/26 00:00 [accepted] PHST- 2009/02/17 00:00 [revised] PHST- 2009/04/08 09:00 [entrez] PHST- 2009/04/08 09:00 [pubmed] PHST- 2009/10/02 06:00 [medline] AID - 10.1007/s12020-009-9183-1 [doi] PST - ppublish SO - Endocrine. 2009 Aug;36(1):20-4. doi: 10.1007/s12020-009-9183-1. Epub 2009 Apr 7.