Primary Central Nervous System Anaplastic Large Cell Lymphoma, ALK Positive.
Am J Clin Pathol
; 158(2): 300-310, 2022 08 04.
Article
em En
| MEDLINE
| ID: mdl-35460414
ABSTRACT
OBJECTIVES:
Primary central nervous system anaplastic large cell lymphoma, anaplastic lymphoma kinase positive (primary CNS ALCL, ALK+) is a rare CNS lymphoma whose description is limited to case reports. These tumors have a variable clinical course, and prognosis is primarily determined by age. We present the largest case series to date of primary CNS ALCL, ALK+, with observational data.METHODS:
A retrospective search of multiple academic centers was performed to identify cases of primary CNS ALCL, ALK+. We also performed a review of published cases of primary CNS ALCL, ALK+. Clinical history, radiography, pathology, and genetic testing data were obtained to determine the prognostic implications in the context of clinical course.RESULTS:
We identified three cases of primary CNS ALCL, ALK+ from our databases. A literature review identified 30 published reports of 31 individual cases. Clinical features for the combined 34 cases included a median age of 18.5 years, with a male to female ratio of 4.71, and the most common symptom was headache. Genetic studies demonstrated an ALK rearrangement by fluorescence in situ hybridization, and a gene fusion assay confirmed an NPM1-ALK gene fusion in one case.CONCLUSIONS:
We present the largest case series to date of a rare primary CNS lymphoma with additional diagnostic and clinical information.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Linfoma Anaplásico de Células Grandes
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
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Prognostic_studies
Limite:
Adolescent
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Female
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Humans
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Male
Idioma:
En
Ano de publicação:
2022
Tipo de documento:
Article