1. Paraneoplastic Syndromes and Thymic Malignancies: An Examination of the International Thymic Malignancy Interest Group Retrospective Database
- Author
-
Padda, Sukhmani K, Yao, Xiaopan, Antonicelli, Alberto, Riess, Jonathan W, Shang, Yue, Shrager, Joseph B, Korst, Robert, Detterbeck, Frank, Huang, James, Burt, Bryan M, Wakelee, Heather A, and Badve, Sunil S
- Subjects
Biomedical and Clinical Sciences ,Oncology and Carcinogenesis ,Rare Diseases ,Cancer ,Adolescent ,Adult ,Aged ,Aged ,80 and over ,Child ,Child ,Preschool ,Databases ,Factual ,Female ,Humans ,Male ,Middle Aged ,Paraneoplastic Syndromes ,Retrospective Studies ,Thymus Neoplasms ,Young Adult ,Thymic epithelial tumor ,Thymoma ,Thymic carcinoma ,Paraneoplastic ,Myasthenia gravis ,Cardiorespiratory Medicine and Haematology ,Clinical Sciences ,Oncology & Carcinogenesis ,Clinical sciences ,Oncology and carcinogenesis - Abstract
IntroductionThymic epithelial tumors (TETs) are associated with paraneoplastic/autoimmune (PN/AI) syndromes. Myasthenia gravis is the most common PN/AI syndrome associated with TETs.MethodsThe International Thymic Malignancy Interest Group retrospective database was examined to determine (1) baseline and treatment characteristics associated with PN/AI syndromes and (2) the prognostic role of PN/AI syndromes for patients with TETs. The competing risks model was used to estimate cumulative incidence of recurrence (CIR) and the Kaplan-Meier method was used to calculate overall survival (OS). A Cox proportional hazards model was used for multivariate analysis.ResultsA total of 6670 patients with known PN/AI syndrome status from 1951 to 2012 were identified. PN/AI syndromes were associated with younger age, female sex, thymoma histologic type, earlier stage, and an increased rate of total thymectomy and complete resection status. There was a statistically significant lower CIR in the group with a PN/AI syndrome than in the group without a PN/AI syndrome (10-year CIR 17.3% versus 21.2%, respectively [p = 0.0003]). The OS was improved in the group with a PN/AI syndrome compared to the group without a PN/AI syndrome (median OS 21.6 years versus 17.0 years, respectively [hazard ratio = 0.63, 95% confidence interval: 0.54-0.74, p < 0.0001]). However, in the multivariate model for recurrence-free survival and OS, PN/AI syndrome was not an independent prognostic factor.DiscussionPreviously, there have been mixed data regarding the prognostic role of PN/AI syndromes for patients with TETs. Here, using the largest data set in the world for TETs, PN/AI syndromes were associated with favorable features (i.e., earlier stage and complete resection status) but were not an independent prognostic factor for patients with TETs.
- Published
- 2018