title: Primary anaplastic large cell lymphoma of the central nervous system: prognostic effect of ALK-1 expression.
abstract: Anaplastic large cell lymphoma (ALCL) rarely occurs in the central nervous system. Although defined by its composition of large, pleomorphic, CD30-positive lymphocytes, ALCL is heterogeneous. Most are T cell but some are null cell. Most but not all have a characteristic 2:5 translocation producing the fusion protein ALK-1, which is reliably detected by immunohistochemistry. In systemic ALCL, ALK-1 expression correlates with young patient age and a favorable prognosis. Herein we report four new cases of primary central nervous system ALCL from the Mayo Clinic and incorporate additional data from five previously published cases. ALK-1 expression was determined in all nine tumors. Patient age was 4-66 years (mean 29 years) with a bimodal distribution: 6 < or = 22 years, 3 > or = 50 years. Six were female. Tumors were mostly supratentorial, five were multifocal, and seven had involvement of dura or leptomeninges. Seven tumors were T cell, two were null cell, and five of nine were ALK-1 immunopositive. Total mortality was six of nine. Three patients, 4-18 years of age (mean 13 years), were alive at 4.8-6.1 years postdiagnosis; these tumors were all ALK positive. Five patients, 13-66 years of age (mean 43 years), died of tumor 4 days to 11 weeks postdiagnosis; four of five of these tumors were ALK negative. One 10-year-old child with an ALK-positive tumor died of sepsis, but in remission. Central nervous system ALCL is aggressive. Our study suggests that a better outcome may be associated with young age and ALK-1 positivity, prognostic parameters similar to systemic ALCL.
authors.affiliation: Department of Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: David H
authors.initials: DH
authors.lastname: George
authors.forename: Bernd W
authors.initials: BW
authors.lastname: Scheithauer
authors.forename: Fügen V
authors.initials: FV
authors.lastname: Aker
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Kurtin
authors.forename: Peter C
authors.initials: PC
authors.lastname: Burger
authors.forename: José
authors.initials: J
authors.lastname: Cameselle-Teijeiro
authors.forename: Roger E
authors.initials: RE
authors.lastname: McLendon
authors.forename: Joseph E
authors.initials: JE
authors.lastname: Parisi
authors.forename: Werner
authors.initials: W
authors.lastname: Paulus
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Roggendorf
authors.forename: Cirilo
authors.initials: C
authors.lastname: Sotelo
mesh_terms: D030201:Activin Receptors, Type I / Q000096:biosynthesis*; D030301:Activin Receptors, Type II; D000293:Adolescent; D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000378:metabolism* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D017728:Lymphoma, Large-Cell, Anaplastic / Q000378:metabolism* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis
chemical_list: C425364:ACVRL1 protein, human; D030201:Activin Receptors, Type I; D030301:Activin Receptors, Type II