J.Jpn. Surg. Soc.. 122(1): 43-47, 2021

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Feature topic

TREATMENT FOR NEUROENDOCRINE THYMIC TUMORS

Thymic neuroendocrine tumors (TNETs) are rare, with a rate of incidence among all thymic epithelial tumors of 2-5%. According to the 4th edition of the WHO classification published in 2015, TNETs include small-cell carcinoma (SCC), large-cell neuroendocrine carcinoma (LCNEC), and atypical and typical carcinoids. The first-line treatment for a TNET is surgical resection. Generally, complete thymectomy including the tumor and locoregional lymphadenectomy should be performed for patients with TNETs. As prognosis mainly depends on the completeness of surgical resection, aggressive multidisciplinary treatment such as induction chemotherapy/chemoradiotherapy followed by extended surgery is indicated. For unresectable or recurrent disease, drug therapy is administered. Platinum-based chemotherapy is used for SCC or LCNEC, and the mammalian target of rapamycin (mTOR) inhibitor everolimus is available for carcinoids. In addition, 2.5-5% of patients with multiple endocrine neoplasia-1 (MEN-1) develop thymic carcinoids. TNETs were reported to be one of the major causes of death in MEN-1 patients, and thus it is important for them to undergo surveillance screening and treatment for thymic carcinoids. This review article describes the diagnosis and treatment of TNETs including the latest findings and the role and results of surgical treatment.

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