• Yudai Hirano, Satoru Miyawaki, Michiaki Satou, Kazuki Taoka, Kazuhiro Toyama, Masako Ikemura, Ryo Tanaka, Shunsaku Takayanagi, Shota Tanaka, Hirofumi Nakatomi, Mineo Kurokawa, and Nobuhito Saito.
• Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan.
• World Neurosurg. 2020 Jun 1; 138: 169-173.

BackgroundPrimary central nervous system (CNS) anaplastic large cell lymphoma (ALCL) is an uncommon type of brain tumor, usually treated with a regimen that includes high-dose methotrexate (MTX). Only a few cases of primary CNS anaplastic lymphoma kinase (ALK)-positive ALCL have been reported so far, with no reported cases of a small cell variant.Case DescriptionA 26-year-old man presenting with headache and visual field impairment was found to have a supratentorial mass mimicking meningioma. Craniotomy was performed for tumor resection, and postoperative histologic examination revealed atypical cells that were nonenlarged lymphocytes with irregularly shaped and enlarged nuclei; these cells were cluster of differentiation 30 and ALK-positive, leading to the diagnosis of a small cell variant of ALK-positive ALCL. In this case, the tumor exhibited an aggressive behavior with MTX resistance with metastases in the pelvis but responded well to cytarabine and etoposide (CYVE).ConclusionsIn general, CNS ALK-positive ALCL responds well to MTX, but small cell variants show aggressive behavior and may be resistant to MTX. For small cell variants of ALCL that are resistant to MTX therapy, as in this case, CYVE therapy may be an effective treatment.Copyright © 2020 Elsevier Inc. All rights reserved.

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