Clinical Features, Treatments, and Outcomes of Patients with Thymic Carcinoids and Multiple Endocrine Neoplasia Type 1 Syndrome at MD Anderson Cancer Center.

Abstract

Thymic carcinoids are rare neuroendocrine tumors that occur in 1-5% of patients with multiple endocrine neoplasia type 1 (MEN1) and are a major cause of morbidity and mortality. The few published reports associate these tumors with male sex and smoking. Our objective was to describe cases of these tumors treated at our institution. We performed a retrospective chart review of all patients diagnosed with MEN1 at our tertiary referral center from 1980 to 2014. Patients with a histopathologic, fine-needle aspiration, or clinical diagnosis of a thymic carcinoid were included. Two hundred ninety-one patients fulfilled the criteria for a diagnosis of MEN1. Clinicopathologic characteristics, MEN1 genetic testing results, treatments, and survival rates were analyzed. Nine patients had a thymic carcinoid, six men (67%) and three women (33%). Six patients were non-smokers (67%). Two patients had synchronous (22%) and eight patients (89%) had metachronous distant metastasis. The 10-year overall survival rate was 45% (lower 95% upper 95% CI 20-100%). The 10-year disease-free survival rate was 42% (lower 95% upper 95% CI 15-100%). Five patients had MEN1 genetic testing, and the genotypes of affected individuals were p.W341X, c.275_286delGCTTCACCGCCC, p.R98X, c.1350+(1_11)del11, and partial duplication of exons 9 and 10. A higher percentage of MEN1-related thymic carcinoids can occur in women and in non-smokers than previously reported. Both novel and known mutations were present in our cohort. Eighty nine percent of patients developed a metachronous metastasis from the thymic carcinoid. Patients with MEN1 and thymic carcinoids should be followed closely.