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Primary Cerebral ALK-1-Positive Anaplastic Large Cell Lymphoma in a Child

Case Report and Literature Review Karikari I.O.a · Thomas K.K.a · Lagoo A.b · Cummings T.J.b · George T.M.c
aPediatric Neurosurgery Service and bDepartment of Pathology, Duke University Medical Center, Durham, N.C., cChildren’s Hospital of Austin, Austin, Tex., USA Pediatr Neurosurg 2007;43:516–521 (DOI:10.1159/000108799)


A 4-year-old African American male was referred to the Pediatric Neurosurgery Service for evaluation of new onset seizures and worsening mental status. An MRI of the brain revealed a pineal region mass with diffuse leptomeningeal enhancement and compression of the basilar cisterns. A biopsy of the brain revealed histologic and immunophenotypic findings characteristic of ALK-1+ anaplastic large cell lymphoma (ALCL). ALCL rarely occurs in the central nervous system and poses a significant diagnostic challenge often leading to a delay in the initiation of appropriate treatment. We describe a case of a rapidly deteriorating clinical course in a child with central nervous system ALCL and review the current literature on ALCL occurring in the central nervous system.


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