Primary neuroendocrine tumours of the thymus: a clinicopathologic and prognostic study in 19 patients §

Background: We sought to evaluate factors influencing long-term survival in 19 patients with primary neuroendocrine tumours of the thymus. Methods: From January 1990 to December 2004, 19 patients (14 males, 5 females; mean age 48.6 years) were surgically treated for a primary neuroendocrinetumourofthethymus.Results:AllpatientsunderwentradicalR0thymomectomyandwerefollowedupfora totalof1459months (median: 69 months; range: 8—180). Nine patients had associated paraneoplastic syndrome. No operative mortality occurred. Two patients underwentre-dosurgerybecauseoflocalrecurrence,respectively, 25and35monthsaftersurgery.Fivepatientsdiedofdisease,respectively, 51, 70, 95, 131 and 153 months after surgery. One patient died of myocardial infarction with no evidence of disease. Thirteen patients are alive, of which 10 are free from disease and three with disease. The overall 5-year and 10-year actuarial survival rates were 91.6% and 69.8%, respectively (median survival: 153 months). The10-year survival was evaluated according to histology (typicalcarcinoid 100%; atypicalcarcinoid:66.6%; large cell neuroendocrine tumours: 0%), Masaoka staging (stage I: 100%; stage II: 50%; stage III: 66.6%; stage IV: 0%), presence of paraneoplastic syndrome (no: 87.5%; yes: 0%) and postoperative radiotherapy (yes: 40%; no: 83.3%). Conclusions: The prognosis of primary neuroendocrine tumours of the thymus is related to the grading of the neoplasm, the presenceof a paraneoplastic syndrome and to the Masaoka staging but not to the postoperative radiotherapy. # 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.