Successful management of a multiple endocrine neoplasia type 1‐associated thymic neuroendocrine neoplasms with acute chest pain as initial symptom: A rare case report

Abstract

Key Clinical MessageAcute chest pain can be the first manifestation of multiple endocrine neoplasia type 1(MEN1)‐associated thymic neuroendocrine neoplasms (NEN). Comprehensive treatment may be an effective strategy for MEN1‐associated NEN.AbstractMultiple endocrine neoplasia type 1(MEN1)‐associated thymic neuroendocrine neoplasms (NEN) is caused by the mutation of tumor suppressor MEN1 gene. Patients with MEN1‐associated NEN initially presenting with acute chest pain are very rare. In the manuscript, we reported a case of a 45‐year‐old man who developed MEN1‐associated NEN with acute chest pain as initial symptom. Thoracoscopic thymotomy was performed and thymic NEN was successfully removed. Genetic test showed a germline mutation of MEN1 gene in this patient. Immunohistochemical staining exhibited Syn(+), CgA(+), INSM1(+), CD56(+) and Ki67‐positive cells (2%) in MEN1‐associated NEN. Further evaluation unveiled MEN1‐associated benign tumors including digestive NEN and pituitary gland adenoma. The 99mTc‐HYNIC‐TOC scintigraphy showed that focally increased radioactivity in the mid‐upper abdomen. This patient was administered with 50Gy/25F of radiation dose to treat the postoperative lesions. Subsequently, sandostatin LAR (30 mg per week) was used as systemic therapy. He had no recurrence or metastasis for 6‐month follow‐up. Thus, acute chest pain can be the first manifestation of MEN1‐associated NEN, and comprehensive treatment including surgery, radiation and systemic treatment may be an effective strategy for MEN1‐associated NEN.