Clinical Features and Prognostic Factors for Primary Anaplastic Large Cell Lymphoma of the Central Nervous System: A Systematic Review
by
, ,
Yudai Hirano
1, 
Satoru Miyawaki
1,*,
Shota Tanaka
1,
Kazuki Taoka
2,
Hiroki Hongo
1,
Yu Teranishi
1,
Hirokazu Takami
1,
Shunsaku Takayanagi
1,
Mineo Kurokawa
2 and
Nobuhito Saito
1 1
Department of Neurosurgery, The University of Tokyo, Bunkyo-ku, Tokyo 113-8655, Japan
2
Department of Hematology and Oncology, The University of Tokyo, Bunkyo-ku, Tokyo 113-8655, Japan
*
Author to whom correspondence should be addressed.
Cancers 2021, 13(17), 4358; https://doi.org/10.3390/cancers13174358
Submission received: 1 August 2021
/
Revised: 21 August 2021
/
Accepted: 23 August 2021
/
Published: 28 August 2021
(This article belongs to the Special Issue Molecular Pathogenesis and Management of Anaplastic Large Cell Lymphoma)
Simple Summary
Primary anaplastic large cell lymphoma (ALCL) of the central nervous system (CNS) is a subtype of primary central nervous system lymphoma (PCNSL). ALCL is divided into anaplastic lymphoma kinase (ALK)-positive ALCL and ALK-negative ALCL, according to ALK expression. ALK-positive cancers tend to develop at a younger age and tend to have a better prognosis. Almost all past articles on primary ALCL of the CNS have been case reports and there have been no randomized trials or cohort studies on this subject. We thus performed a systematic review of primary ALCL of the CNS. According to the author’s survey, 36 case reports have been published in English-language journals. In this paper, we have summarized the clinical features and prognostic factors for primary ALCL of the CNS based on previous studies.
Abstract
Primary anaplastic large cell lymphoma (ALCL) of the central nervous system (CNS) is a subtype of primary CNS lymphoma (PCNSL). There are very few comprehensive reports on this extremely rare tumor. Therefore, it is necessary to investigate the clinical features and prognostic factors for primary ALCL of the CNS. We performed a systematic review of the published literature. Past cases were comprehensively searched using PubMed, Cochrane Library, and Web of Science. Clinical information, such as age, sex, anaplastic lymphoma kinase (ALK) status, lesion sites, treatment methods, and survivorship were extracted. Thirty-nine cases with information on ALK status and treatment course were identified. The average observation period was 13 months, and the overall 2-year survival rate was 58%. Univariate analyses showed a statistically significantly better prognosis among patients < 40 years of age (p = 0.039, HR 0.32 (0.11–0.95)) and in relation to ALK positivity (p = 0.010, HR 0.24 (0.08–0.71) and methotrexate treatment (p = 0.003, HR 0.17 (0.05–0.56)). Because of the sparsity of cases, it is necessary to accumulate cases in order to perform more detailed analyses.
