Atypical carcinoid of thymus associated with multiple endocrine neoplasia syndrome type 1

Thymic carcinoid associated with multiple endocrine neoplasia syndrome type 1 (MEN-1) is a rare tumor. We report a case of MEN-1-related thymic carcinoid. The patient reported herein had already been diagnosed with MEN-1 and was found to have a mediastinal mass. She underwent thymectomy with partial resection of the left innominate vein and lung. Histological examination revealed atypical carcinoid with infiltration. MEN-1 gene mutation was detected by employing the direct nucleotide sequencing method. Postoperative 2-fluoro-2-deoxyglucose positron emission tomography showed probable multiple metastases in the vertebrae and myocardium. However, she has been alive and asymptomatic for 2 years postoperatively. MEN-1-related thymic carcinoid is often insidious with a poor prognosis. We suggest chest computed tomography scan or magnetic resonance imaging for MEN-1 patients and serological or genetic screening for patients with thymic carcinoid to screen for MEN-1.