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Published in: BMC Endocrine Disorders 1/2021

Open Access 01-12-2021 | Computed Tomography | Case report

A rare case of multiple endocrine neoplasia type 1 initially presenting as an asymptomatic, huge mediastinal mass: case report

Authors: Ji Eun Jun, You-Cheol Hwang, Kyu Jeong Ahn, Ho Yeon Chung, In-Kyung Jeong

Published in: BMC Endocrine Disorders | Issue 1/2021

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Abstract

Background

Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited syndrome that concurrently involves various endocrine glands. We report a rare case of MEN1 in a 43-year-old man whose first manifestation was an asymptomatic mediastinal mass.

Case presentation

A 13-cm-sized mediastinal mass was diagnosed as an atypical thymic carcinoid by computed tomography and percutaneous needle biopsy. In addition, hypercalcemia from a left inferior parathyroid hyperplasia, and a non-functioning gastric neuroendocrine tumor seen on esophagogastroduodenoscopy were found. Therefore, the patient was clinically diagnosed with MEN1 syndrome, and underwent surgical resection of thymic carcinoid tumor after pre-operative concurrent chemoradiation therapy to decrease tumor size and volume. Parathyroid lesion and gastric neuroendocrine tumor were also removed. Finally, a MEN1 gene mutation was observed in the patient and his 7-year-old son.

Conclusion

Despite its rare occurrence, thymic carcinoid tumor should be considered as a MEN1-associated tumor and necessitates screening of other endocrine glands. Thymic carcinoid tumor carries a poor prognosis in patients with MEN1, and thus it needs to be carefully monitored even after radical excision.
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Metadata
Title
A rare case of multiple endocrine neoplasia type 1 initially presenting as an asymptomatic, huge mediastinal mass: case report
Authors
Ji Eun Jun
You-Cheol Hwang
Kyu Jeong Ahn
Ho Yeon Chung
In-Kyung Jeong
Publication date
01-12-2021
Publisher
BioMed Central
Published in
BMC Endocrine Disorders / Issue 1/2021
Electronic ISSN: 1472-6823
DOI
https://doi.org/10.1186/s12902-021-00695-9

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