Tumor genetics and survival of thymic neuroendocrine neoplasms: A multi‐institutional clinicopathologic study |
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| Authors: | Philipp Ströbel Andreas Zettl Konstantin Shilo Wen‐Yu Chuang Andrew G. Nicholson Yoshihiro Matsuno Anthony Gal Rolf Hubert Laeng Peter Engel Carlo Capella Mirella Marino John Kwok-Cheung Chan Andreas Rosenwald William Travis Teri J. Franks David Ellenberger Inga‐Marie Schaefer Alexander Marx |
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| Affiliation: | 1. Institute of Pathology, University Medical Center G?ttingen, G?ttingen, Germany;2. Institute of Pathologie Viollier, Basle, Switzerland;3. Department of Pathology, Ohio State University, Columbus, OH;4. Department of Pathology, Chang Gung Memorial Hospital and Chang Gung University, Linkou, Taiwan;5. Department of Histopathology, Royal Brompton and Harefield NHS Foundation Trust and National Heart and Lung Institute, Imperial College, London, UK;6. Department of Surgical Pathology, Hokkaido University Hospital, Sapporo, Japan;7. Pathology & Laboratory Medicine, Emory University School of Medicine, Atlanta, GA;8. Pathologie L?nggasse, Bern, Switzerland;9. Department of Pathology, Roskilde Hospital, Roskilde, Denmark;10. Department of Human Morphology, Varese, Italy;11. Department of Pathology, Regina Elena National Cancer Institute, Rome, Italy;12. Department of Pathology, Queen Elizabeth Hospital, Hong Kong, China;13. Institute of Pathology, University of Würzburg, Germany;14. Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY;15. Pulmonary & Mediastinal Pathology, The Joint Pathology Center, Silver Spring, MD;16. Department of Medical Statistics, University Medical Center G?ttingen, G?ttingen, Germany;17. Department of Pathology, Brigham and Women's Hospital, Boston, MA;18. Institute of Pathology, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany |
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| Abstract: | Thymic neuroendocrine tumors (TNET) are rare primary epithelial neoplasms of the thymus. This study aimed to determine clinically relevant parameters for their classification and for therapeutic decisions. We performed a comprehensive histological, clinical, and genetic study of 73 TNET cases (13 thymic typical carcinoids [TTC], 40 thymic atypical carcinoids [TAC], and 20 high‐grade neuroendocrine carcinomas [HGNEC] of the thymus), contributed by multiple institutions. The mean number of chromosomal imbalances per tumor was 0.8 in TTC (31% aberrant cases) versus 1.1 in TAC (44% aberrant cases) versus 4.7 in HGNEC (75% aberrant cases). Gains of 8q24 (MYC gene locus) were the most frequent alteration and one of the overlapping features between carcinoids and HGNEC. The 5‐year survival rates for TTC, TAC, and HGNEC were 100, 60, and 30%. The 10‐year survival rates for TTC and TAC were 50 and 30% (P = 0.002). Predictive mitotic cut‐off values for TTC versus TAC were 2.5 per 10 high‐power fields (HPF; indicating a higher death rate, P = 0.062) and 15 per 10 HPF (indicating higher risk of recurrence, P = 0.036) for separating HGNEC from TAC. We conclude that the current histopathologic classifications of TNET reflect tumor biology and provide important information for therapeutic management. © 2014 Wiley Periodicals, Inc. |
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