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. 2018 Mar;13(3):436-446.
doi: 10.1016/j.jtho.2017.11.118. Epub 2017 Nov 27.

Paraneoplastic Syndromes and Thymic Malignancies: An Examination of the International Thymic Malignancy Interest Group Retrospective Database

Sukhmani K Padda  1 , Xiaopan Yao  2 , Alberto Antonicelli  2 , Jonathan W Riess  3 , Yue Shang  4 , Joseph B Shrager  5 , Robert Korst  6 , Frank Detterbeck  2 , James Huang  7 , Bryan M Burt  8 , Heather A Wakelee  9 , Sunil S Badve  10
Affiliations

Paraneoplastic Syndromes and Thymic Malignancies: An Examination of the International Thymic Malignancy Interest Group Retrospective Database

Sukhmani K Padda et al. J Thorac Oncol. 2018 Mar.

Abstract

Introduction: Thymic epithelial tumors (TETs) are associated with paraneoplastic/autoimmune (PN/AI) syndromes. Myasthenia gravis is the most common PN/AI syndrome associated with TETs.

Methods: The 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.

Results: A 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.

Discussion: Previously, 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.

Keywords: Myasthenia gravis; Paraneoplastic; Thymic carcinoma; Thymic epithelial tumor; Thymoma.

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Conflict of interest statement

Conflict of Interest Statement: No relevant COI for all authors for this submitted work.

Dr. Wakelee has the following disclosures but not relevant to this submitted work: Consultant/Honoraria: Peregrine, ACEA, Pfizer, Helsinn, Genentech (uncompensated) Grant/Research: Clovis, Exelixis, AstraZeneca/Medimmune, Genentech/Roche, BMS, Gilead, Novartis, Xcovery, Pfizer, Celgene, Pharmacyclics, Lilly

Dr. Padda has the following disclosures but not relevant to this submitted work: Consultant/Honoraria: G1 therapeutics, Janssen, AstraZeneca Research: EpicentRx, Forty Seven Inc.

Figures

Figure 1
Figure 1
Flow Diagram. *Numbers do not add up to 373 because there is overlap among patients with missing information for each variable. (PN/AI=paraneoplastic/autoimmune syndrome)
Figure 2
Figure 2
Cumulative Incidence of Recurrence in All Patients, PN/AI (+) vs. PN/AI (−) (PN/AI=paraneoplastic/autoimmune syndrome)
Figure 3
Figure 3
Overall Survival in All Patients, PN/AI (+) vs. PN/AI (−) (PN/AI=paraneoplastic/autoimmune syndrome)
Figure 4
Figure 4
Overall Survival, PN/AI (+) vs. PN/AI (−), in (A) Thymoma Patients and (B) Thymic Carcinoma Patients (PN/AI=paraneoplastic/autoimmune syndrome)
See this image and copyright information in PMC

Comment in

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