Abstract

Thymic epithelial tumors (TET) include thymomas (T) and thymic carcinomas (TC). TET are rare malignant tumors usually associated with paraneoplastic syndromes (PNPS). The objective of the study is to describe our experience and to analyze the prognostic factors. Retrospective analysis of the clinical and pathological characteristics of 42 patients (pts) diagnosed with TET in our institution from 1990 to 2016. We analyzed the outcomes in terms of progression-free survival (PFS) and overall survival (OS) and their association with clinical factors: age, perfomance status, presence of PNPS, TNM staging, WHO classification, complete resection and treatment. Kaplan Meier method and Cox regression were used. Mean age: 55 years (27-86). 19 (45.2%): women. First treatment: surgery in 34 pts (81%) and platinum based chemotherapy in 6 pts (14%). 2 pts (5%) were untreated. 14 pts (41%) received postoperative radiotherapy. WHO classification: 6 pts (14.3%) type A, 12 (28.6%) AB, 3 (7.1%) B1, 7 (16.7%) B2, 7 (16.7%) B3 and 7 (16.7%) C. TNM staging: 20 pts (48.8%) stage I,4 (9.8%) stage II, 6 (14.6%) stage III and 9 (22%) stage IV. 25 ptss (59.5%) had PNPS at diagnosis: 21 (50%) myasthenia gravis, 2 (4.75%) aplastic anemia and 2 (4.75%) others. Table 1 shows OS and PFS according to WHO classification:Tabled 1WHO CWHO: A, AB, B1, B2, B3p valueHR (univariate)p valuePFS (months)(95%CI)21.2 (5.8-36.7)89.3(69.1-109.6)0.033.120.04OS (months)(95%CI)66.5 (0-157.5)296 (98.6-493.3)<0.00120.20.007 Open table in a new tab The presence of PNPS were associated with better OS than those without PNPS (236m, 95%CI 147-448m vs 66.5m, 95% CI 1.3-131.7m, p=0.006, HR 0.76, p=0.026 ) and also with early diagnosis (stage I for pts with PNPS 56% vs 42% without PNPS). In the multivariate analysis, only C type remained statistically significant (HR:13.5, p=0.024). No differences were found when using TNM classification or complete resection. Patients with C type TET had worst prognosis. The presence of a PNPS is associated with better OS possibly due to and early diagnosis.

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