Primary central nervous system extranodal NK/T-cell lymphoma, nasal type: case report and review of the literature

Primary central nervous system (CNS) extranodal NK/T-cell lymphoma, nasal type (NKTCL), is an extremely rare tumor. To the best of our knowledge, only four cases have been described previously. Here, we report a case of primary CNS NKTCL in a 25-year-old immunocompetent Chinese male. The patient pre...

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Veröffentlicht in:Journal of neuro-oncology 2011-06, Vol.103 (2), p.387-391
Hauptverfasser: Guan, Hong, Huang, Yuhua, Wen, Wen, Xu, Meiquan, Zan, Qin, Zhang, Zhixiong
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Huang, Yuhua
Wen, Wen
Xu, Meiquan
Zan, Qin
Zhang, Zhixiong
description Primary central nervous system (CNS) extranodal NK/T-cell lymphoma, nasal type (NKTCL), is an extremely rare tumor. To the best of our knowledge, only four cases have been described previously. Here, we report a case of primary CNS NKTCL in a 25-year-old immunocompetent Chinese male. The patient presented with worsening dizziness, headaches, and vomiting for approximately 2 weeks. Magnetic resonance imaging demonstrated three masses with solid components entirely in the parenchyma of the right hemisphere, and no sinonasal/nasopharyngeal lesions were found. The patient underwent a partial resection of the right temporal mass. Histological examination revealed that intermediate-sized, pleomorphic lymphocytes were arranged in an angiocentric distribution with large geographic necroses. The tumor cells expressed CD3ε, CD56, TIA-1, granzyme B, and Epstein−Barr virus-encoded RNAs. A rearrangement study showed T-cell receptor γ-chain gene rearrangement with monoclonal appearance. Postoperative chemotherapy and radiotherapy were also given, but the lymphoma failed to respond to therapy and the patient died 18 months later. Our observation and the four others found in the literature indicate that primary CNS NKTCL occurs predominantly in adult males. This is the youngest patient with primary CNS NKTCL reported.
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Postoperative chemotherapy and radiotherapy were also given, but the lymphoma failed to respond to therapy and the patient died 18 months later. Our observation and the four others found in the literature indicate that primary CNS NKTCL occurs predominantly in adult males. 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subjects Adult
Antineoplastic Agents - therapeutic use
Brain Neoplasms - genetics
Brain Neoplasms - pathology
Brain Neoplasms - therapy
Case Report
Combined Modality Therapy
Fatal Outcome
Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor
Herpesvirus 4, Human - genetics
Humans
Lymphoma, Extranodal NK-T-Cell - genetics
Lymphoma, Extranodal NK-T-Cell - pathology
Lymphoma, Extranodal NK-T-Cell - therapy
Male
Medicine
Medicine & Public Health
Neurology
Oncology
Radiotherapy
RNA, Viral - isolation & purification
title Primary central nervous system extranodal NK/T-cell lymphoma, nasal type: case report and review of the literature
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