- Browse by Subject
Browsing by Subject "thymic carcinoma"
Now showing 1 - 3 of 3
Results Per Page
Sort Options
Item The Integrated Genomic Landscape of Thymic Epithelial Tumors(Elsevier, 2018-02) Radovich, Milan; Pickering, Curtis R.; Felau, Ina; Ha, Gavin; Zhang, Hailei; Jo, Heejoon; Hoadley, Katherine A.; Anur, Pavana; Zhang, Jiexin; McLellan, Mike; Bowlby, Reanne; Matthew, Thomas; Danilova, Ludmila; Hegde, Apurva M.; Kim, Jaegil; Leiserson, Mark D. M.; Sethi, Geetika; Lu, Charles; Ryan, Michael; Su, Xiaoping; Cherniack, Andrew D.; Robertson, Gordon; Akbani, Rehan; Spellman, Paul; Weinstein, John N.; Hayes, D. Neil; Raphael, Ben; Lichtenberg, Tara; Leraas, Kristen; Zenklusen, Jean Claude; Pathology and Laboratory Medicine, School of MedicineThymic epithelial tumors (TETs) are one of the rarest adult malignancies. Among TETs, thymoma is the most predominant, characterized by a unique association with autoimmune diseases, followed by thymic carcinoma, which is less common but more clinically aggressive. Using multi-platform omics analyses on 117 TETs, we define four subtypes of these tumors defined by genomic hallmarks and an association with survival and World Health Organization histological subtype. We further demonstrate a marked prevalence of a thymoma-specific mutated oncogene, GTF2I, and explore its biological effects on multi-platform analysis. We further observe enrichment of mutations in HRAS, NRAS, and TP53. Last, we identify a molecular link between thymoma and the autoimmune disease myasthenia gravis, characterized by tumoral overexpression of muscle autoantigens, and increased aneuploidy.Item NUT Midline Carcinoma Masquerading As a Thymic Carcinoma(ASCO, 2016-05) Gökmen-Polar, Yesim; Kesler, Kenneth; Loehrer, Patrick J., Sr.; Badve, Sunil; Department of Pathology & Laboratory Medicine, IU School of MedicineThymic carcinomas are rare tumors that arise from the epithelium of the thymus gland and characterized by cytologic atypia, invasiveness, and high risk of relapse and death.1–3 The current WHO schema recognizes at least 11 histologic subtypes.4–7 Undifferentiated thymic carcinoma is one of the subtypes that can be indistinguishable from other poorly differentiated carcinomas such as NUT midline carcinoma (NMC).8 Despite the aggressive nature of both diseases, a correct diagnosis is important because of the recent development of targeted therapies for NMCs. Herein we describe two cases of a particularly aggressive form of disease and discuss the differential diagnosis of these lesions.Item Paraneoplastic Syndromes and Thymic Malignancies: An Examination of the International Thymic Malignancy Interest Group Retrospective Database(Elsevier, 2017) 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.; Badve, Sunil S.; Pathology and Laboratory Medicine, School of MedicineIntroduction 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 (ITMIG) retrospective database was examined to determine (i) baseline and treatment characteristics associated with PN/AI syndromes and (ii) 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 6670 patients with known PN/AI syndrome status were identified from 1951-2012. PN/AI syndromes were associated with younger age, female sex, type B1 thymoma, earlier stage, and an increased rate of total thymectomy and complete resection status. There was a statistically significant lower CIR in the PN/AI (+) group compared to the PN/AI (-) group (10-year 17.3% vs. 21.2%, respectively, p=0.0003). The OS was improved in the PN/AI (+) group compared to the PN/AI (-) group (HR 0.63, 95% CI 0.54-0.74, P<0.0001, median OS 21.6 years versus 17.0 years, respectively). However, in the multivariate model for recurrence-free survival and OS, PN/AI syndrome was not an independent prognostic factor. Discussion Previously, there has been mixed data regarding the prognostic role of PN/AI syndromes for patients with TETs. Here, using the largest dataset in the world for TETs, PN/AI syndromes were associated with favorable features (i.e. earlier stage, complete resection status) but were not an independent prognostic factor for TETs.