Abstract
Simple SummaryPrimary 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.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.
Highlights
This article is an open access articlePrimary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin lymphoma, which accounts for approximately 4% of central nervous system tumors [1]
Primary central nervous system T-cell lymphoma accounts for approximately 2% of PCNSL cases [3]
Anaplastic large cell lymphoma (ALCL) is divided into anaplastic lymphoma kinase (ALK)positive and ALK-negative tumors according to the pattern of genetic abnormalities [5]
Summary
Primary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin lymphoma, which accounts for approximately 4% of central nervous system tumors [1]. Most PCNSL cases are B-cell lymphomas, and the most common histological type is diffuse large B-cell distributed under the terms and conditions of the Creative Commons. Primary central nervous system T-cell lymphoma accounts for approximately 2% of PCNSL cases [3]. Is a T-cell or null cell lymphoma characterized by the proliferation of large lymphoid cells that express a large amount of CD30 on the cell surface; this subtype was first reported by Stein et al in 1985 [4]. ALCL is divided into anaplastic lymphoma kinase (ALK)positive and ALK-negative tumors according to the pattern of genetic abnormalities [5].
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