Tumor genetics and survival of thymic neuroendocrine neoplasms: A multi-institutional clinicopathologic study

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 th...

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Veröffentlicht in:Genes chromosomes & cancer 2014-09, Vol.53 (9), p.738-749
Hauptverfasser: Ströbel, Philipp, Zettl, Andreas, Shilo, Konstantin, Chuang, Wen-Yu, Nicholson, Andrew G., Matsuno, Yoshihiro, Gal, Anthony, Laeng, Rolf Hubert, Engel, Peter, Capella, Carlo, Marino, Mirella, Chan, John Kwok-Cheung, Rosenwald, Andreas, Travis, William, Franks, Teri J., Ellenberger, David, Schaefer, Inga-Marie, Marx, Alexander
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container_end_page 749
container_issue 9
container_start_page 738
container_title Genes chromosomes & cancer
container_volume 53
creator Ströbel, Philipp
Zettl, Andreas
Shilo, Konstantin
Chuang, Wen-Yu
Nicholson, Andrew G.
Matsuno, Yoshihiro
Gal, Anthony
Laeng, Rolf Hubert
Engel, Peter
Capella, Carlo
Marino, Mirella
Chan, John Kwok-Cheung
Rosenwald, Andreas
Travis, William
Franks, Teri J.
Ellenberger, David
Schaefer, Inga-Marie
Marx, Alexander
description 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.
doi_str_mv 10.1002/gcc.22183
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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. 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subjects Adolescent
Adult
Aged
Aged, 80 and over
Child
Female
Humans
Male
Middle Aged
Neuroendocrine Tumors - genetics
Neuroendocrine Tumors - mortality
Neuroendocrine Tumors - pathology
Risk Factors
Survival Rate
Thymus Neoplasms - genetics
Thymus Neoplasms - mortality
Thymus Neoplasms - pathology
Young Adult
title Tumor genetics and survival of thymic neuroendocrine neoplasms: A multi-institutional clinicopathologic study
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