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

Abstract

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 infi ltration. MEN-1 gene mutation was detected by employing the direct nucleotide sequencing method. Postoperative 2-fl uoro-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.

Thymic neuroendocrine tumour in multiple endocrine neoplasia type 1: female patients are not rare exceptions

“…While the reasons for this difference are unclear, ethnicity could be one explanation. Several additional case reports exist on Japanese female MEN1 patients with Th‐NET who have not been registered in our database . Similarly, there have been some case reports from Asian and Western countries on female MEN1 patients with Th‐NET, indicating that there may be more female Th‐NET patients than previously considered.…”

Recent results of basic and clinical research in MEN1: opportunities to improve early detection and treatment

“…Thymus carcinoid MEN1-related thymic carcinoid is often insidious with a poor prognosis. In a French MEN1 group, thymic tumors were exclusively found in males [30], whereas in Japan, 37% of MEN1 thymus carcinoid was found in females [31,94].…”

Thymic Neuroendocrine Neoplasms: Biological Behaviour and Therapy

“…However, a Japanese series showed that 36% of MEN-1 patients with thymic carcinoids were female: of the 10 patients only one was a smoker. In addition, there are several case reports from Asia on female MEN-1 patients with thymic carcinoids [32,33] . However, in a series from the MD Anderson hospital in Houston in 2016, there was a similar male/female ratio of 2: 1 in both ethnic groups, suggesting that ethnic differences might not be significant [34] .…”