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Neuroendocrine Tumors (NETs) of the Mediastinum and Thymus

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Neuroendocrine Tumors: Review of Pathology, Molecular and Therapeutic Advances

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Abstract

Neuroendocrine tumors (NETs) of the mediastinum and thymus are rare and are usually discovered incidentally. Rosai and Higa were the first to acknowledge the existence of carcinoid tumors in the thymus and to separate them from more common tumors arising in this location, such as thymoma. The source of the primary NETs of the thymus has been proposed that they are derived from Kulchitsky cells. In this chapter we discussed the World Health Organization (WHO) classification of the NETs. We also spotlighted on some chromosomal imbalances that have been found by investigators in the most recent molecular biology literatures of the thymic NETs, as gain of chromosome (Xp, 7p, 7q, 11q, 12q, 20q, and 19p) and loss (6q, 6p, 4q, 3p, 10q, 11q, and 13q). Losses of heterozygosity (LOH) at chromosome 1p and loss of chromosomes 3, 9p21, and Y have been also reported. At the end of this chapter, we discussed the up-to-date prognosis, management, response evaluation, and follow-up of the patient.

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Abbreviations

AC Atypical carcinoid

LCNEC Large cell neuroendocrine cell carcinoma

NET Neuroendocrine tumor

SCNEC Small cell neuroendocrine cell carcinoma

TC Typical carcinoid

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Authors and Affiliations

  1. Department of Pathology, H. Lee Moffitt Cancer Center and Research Institute, University of South Florida, Tampa, FL, USA

    Sameer Al Diffalha MD

  2. H. Lee Moffitt Cancer Center and Research Institute, University of South Florida, Tampa, FL, USA

    Farah Khalil MD

  3. Department of Pathology and Cell Biology, University of South Florida, Tampa, FL, USA

    Farah Khalil MD

Authors
  1. Sameer Al Diffalha MD
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  2. Farah Khalil MD
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Correspondence to Sameer Al Diffalha MD .

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Editors and Affiliations

  1. Diagnostic & Experimental Pathology Lilly Res Laboratories, Eli Lilly & Co., Indianapolis, Indiana, USA

    Aejaz Nasir

  2. H. Lee Moffitt Cancer Ctr & Res, University of South Florida, Tampa, Florida, USA

    Domenico Coppola

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Al Diffalha, S., Khalil, F. (2016). Neuroendocrine Tumors (NETs) of the Mediastinum and Thymus. In: Nasir, A., Coppola, D. (eds) Neuroendocrine Tumors: Review of Pathology, Molecular and Therapeutic Advances. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-3426-3_8

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