Thymic Neuroendocrine Neoplasia: Therapy with Everolimus

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Introduction: Due to the low incidence of 0.02/100 000 no established medical therapy for thymic neuroendocrine neoplasia (t-NEN) exists.

Aim(s): This is the first report on the effect of everolimus on t-NEN

Materials and methods: 4 patients (37; 38; 54; 55 yrs, 2 male, 2 female, all nonsmokers) with t-NEN (Ki-67 10;20;20;5 % respectively) received everolimus 10 mg/d after failure of at least 1 previous medical therapy (2,3,6,1 resp.). Everolimus was applied after a median of 32.4 months after first diagnosis (range 5 - 56 months). In 3 patients thoracic pain led to the diagnosis, whereas in 1 patient the t-NEN was an incidental finding. The tumor size at first diagnosis was 22;13;11; 9 cm resp. Thymectomy was performed in 3 cases. All patients developed metastasis, in 3 cases after thymectomy. No hormonal hypersecretion was observed, 1 patient had multiple endocrine neoplasia type 1

Conference: 13th Annual ENETSConcerence (2016)

Presenting Author:

Authors: Lang M, Anamaterou C,

Keywords: thymus neuroendocrine neoplasia, thymic carcinoid, everolimus, targeted therapy, mTOR inhibitor, MEN1,