PMID- 9196616 OWN - NLM STAT- MEDLINE DCOM- 19970708 LR - 20190813 IS - 0300-0664 (Print) IS - 0300-0664 (Linking) VI - 46 IP - 4 DP - 1997 Apr TI - Cystic glucagonoma with loss of heterozygosity on chromosome 11 in multiple endocrine neoplasia type 1. PG - 511-6 AB - A 52-year-old man with a past history of a pituitary adenoma and hyperparathyroidism due to a parathyroid adenoma was admitted because of a solitary tumour of the pancreas revealed by ultrasonography. His family history was unremarkable. Plasma glucagon levels were slightly elevated (280 ng/l, normal range, 40-180 ng/l) with decreased plasma amino acid levels. Plasma glucagon levels disclosed an exaggerated response during an arginine infusion test and paradoxical elevation during a 75 g oral glucose tolerance test. Endoscopic ultrasonography revealed a monolocular cystic mass of about 3 cm in diameter in the body of the pancreas. A pancreatic tumour was diagnosed before surgery as a cystic glucagonoma. Intra-operative ultrasonography showed one cystic mass in the body of pancreas and two other solid tumours, about 1 cm and 0.5 cm in diameter, in the tail of the pancreas. Histologically, all three tumours showed neoplastic epithelial cells with round nuclei forming cords and/or a ribbon-like arrangement. They showed positive staining for Grimelius' silver stain and immunopositive cells for glucagon. Genetic analysis of the main cystic tumour revealed loss of heterozygosity (LOH) on chromosome 11. After the operation, the responses of plasma glucagon to arginine infusion and oral glucose became normal. Here we describe the usefulness of these provocation tests for early diagnosis and post-operative follow-up in a rare cystic glucagonoma associated with multiple endocrine neoplasia type 1 (MEN 1) which had LOH on chromosome 11. FAU - Sarui, H AU - Sarui H AD - Third Department of Internal Medicine, Gifu University School of Medicine, Japan. FAU - Yoshimoto, K AU - Yoshimoto K FAU - Okumura, S AU - Okumura S FAU - Kamura, M AU - Kamura M FAU - Takuno, H AU - Takuno H FAU - Ishizuka, T AU - Ishizuka T FAU - Takao, H AU - Takao H FAU - Shimokawa, K AU - Shimokawa K FAU - Itakura, M AU - Itakura M FAU - Saji, S AU - Saji S FAU - Yasuda, K AU - Yasuda K LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PL - England TA - Clin Endocrinol (Oxf) JT - Clinical endocrinology JID - 0346653 RN - 9007-92-5 (Glucagon) RN - 94ZLA3W45F (Arginine) SB - IM MH - Arginine MH - *Chromosomes, Human, Pair 11 MH - Glucagon/blood MH - Glucagonoma/diagnosis/diagnostic imaging/*genetics MH - Glucose Tolerance Test MH - *Homozygote MH - Humans MH - Male MH - Middle Aged MH - Multiple Endocrine Neoplasia Type 1/diagnostic imaging/*genetics MH - Pancreatic Neoplasms/diagnosis/diagnostic imaging/*genetics MH - Ultrasonography EDAT- 1997/04/01 00:00 MHDA- 1997/04/01 00:01 CRDT- 1997/04/01 00:00 PHST- 1997/04/01 00:00 [pubmed] PHST- 1997/04/01 00:01 [medline] PHST- 1997/04/01 00:00 [entrez] AID - 10.1046/j.1365-2265.1997.1380965.x [doi] PST - ppublish SO - Clin Endocrinol (Oxf). 1997 Apr;46(4):511-6. doi: 10.1046/j.1365-2265.1997.1380965.x.