Korean J Helicobacter Up Gastrointest Res.  2019 Mar;19(1):65-70. 10.7704/kjhugr.2019.19.1.65.

A Case of Asymptomatic Multiple Endocrine Neoplasia Type I with Thymic Carcinoid

• Park SK
• Lee MW
• Han IS
• Park YJ
• Han SY
• Park JW
• Lee BE
• Kim GH
• Kim SS

• KMID: 2440658
• DOI: http://doi.org/10.7704/kjhugr.2019.19.1.65

Abstract

Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant hereditary disorder caused by germline mutation of the MEN1 gene. It is characterized by tumors of the anterior pituitary gland, parathyroid glands, and endocrine pancreas. Thymic carcinoid tumor is uncommon and associated with a high mortality, but its natural history has not been investigated yet. We report a case of asymptomatic MEN 1 with a thymic carcinoid tumor. A 37-year-old man underwent a routine medical checkup and upper gastrointestinal endoscopy revealed a duodenal neuroendocrine tumor (NET). Further studies showed the coexistence of pancreatic tumor, parathyroid hyperplasia, pituitary adenoma, and thymoma. The patient underwent duodenal endoscopic mucosal resection, distal pancreatectomy, subtotal parathyroidectomy, and thymectomy. The pathological test revealed a duodenal NET, pancreatic NET, parathyroid hyperplasia, and thymic carcinoid tumor. He was treated for MEN 1. We report this asymptomatic case of MEN 1 with a literature review.

Keyword

Hyperparathyroidism; Multiple endocrine neoplasia type 1; Neuroendocrine tumors; Thymoma

MeSH Terms

Adult
Carcinoid Tumor*
Endoscopy, Gastrointestinal
Germ-Line Mutation
Humans
Hyperparathyroidism
Hyperplasia
Islets of Langerhans
Mortality
Multiple Endocrine Neoplasia Type 1*
Multiple Endocrine Neoplasia*
Natural History
Neuroendocrine Tumors
Pancreatectomy
Parathyroid Glands
Parathyroidectomy
Pituitary Gland, Anterior
Pituitary Neoplasms
Thymectomy
Thymoma