Primary anaplastic large cell lymphoma of the central nervous system

Prognostic effect of ALK-1 expression

David H. George, Bernd W. Scheithauer, Fügen V. Aker, Paul J. Kurtin, Peter C. Burger, José Cameselle-Teijeiro, Roger E. McLendon, Joseph E. Parisi, Werner Paulus, Wolfgang Roggendorf, Cirilo Sotelo

Research output: Contribution to journalArticle

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 ≤ 22 years, 3 ≥ 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.

Original languageEnglish (US)
Pages (from-to)487-493
Number of pages7
JournalAmerican Journal of Surgical Pathology
Volume27
Issue number4
DOIs
StatePublished - Apr 1 2003

Fingerprint

Anaplastic Large-Cell Lymphoma
Central Nervous System
Neoplasms
Null Lymphocytes
T-Lymphocytes
Sepsis
Immunohistochemistry
Lymphocytes
Mortality

Keywords

  • ALK-1
  • Brain
  • Lymphoma
  • Prognosis

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine

Cite this

George, D. H., Scheithauer, B. W., Aker, F. V., Kurtin, P. J., Burger, P. C., Cameselle-Teijeiro, J., ... Sotelo, C. (2003). Primary anaplastic large cell lymphoma of the central nervous system: Prognostic effect of ALK-1 expression. American Journal of Surgical Pathology, 27(4), 487-493. https://doi.org/10.1097/00000478-200304000-00008

Primary anaplastic large cell lymphoma of the central nervous system : Prognostic effect of ALK-1 expression. / George, David H.; Scheithauer, Bernd W.; Aker, Fügen V.; Kurtin, Paul J.; Burger, Peter C.; Cameselle-Teijeiro, José; McLendon, Roger E.; Parisi, Joseph E.; Paulus, Werner; Roggendorf, Wolfgang; Sotelo, Cirilo.

In: American Journal of Surgical Pathology, Vol. 27, No. 4, 01.04.2003, p. 487-493.

Research output: Contribution to journalArticle

George, DH, Scheithauer, BW, Aker, FV, Kurtin, PJ, Burger, PC, Cameselle-Teijeiro, J, McLendon, RE, Parisi, JE, Paulus, W, Roggendorf, W & Sotelo, C 2003, 'Primary anaplastic large cell lymphoma of the central nervous system: Prognostic effect of ALK-1 expression', American Journal of Surgical Pathology, vol. 27, no. 4, pp. 487-493. https://doi.org/10.1097/00000478-200304000-00008
George, David H. ; Scheithauer, Bernd W. ; Aker, Fügen V. ; Kurtin, Paul J. ; Burger, Peter C. ; Cameselle-Teijeiro, José ; McLendon, Roger E. ; Parisi, Joseph E. ; Paulus, Werner ; Roggendorf, Wolfgang ; Sotelo, Cirilo. / Primary anaplastic large cell lymphoma of the central nervous system : Prognostic effect of ALK-1 expression. In: American Journal of Surgical Pathology. 2003 ; Vol. 27, No. 4. pp. 487-493.
@article{13d6b72fb1be42e3a25bf135f643ea45,
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 ≤ 22 years, 3 ≥ 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.",
keywords = "ALK-1, Brain, Lymphoma, Prognosis",
author = "George, {David H.} and Scheithauer, {Bernd W.} and Aker, {F{\"u}gen V.} and Kurtin, {Paul J.} and Burger, {Peter C.} and Jos{\'e} Cameselle-Teijeiro and McLendon, {Roger E.} and Parisi, {Joseph E.} and Werner Paulus and Wolfgang Roggendorf and Cirilo Sotelo",
year = "2003",
month = "4",
day = "1",
doi = "10.1097/00000478-200304000-00008",
language = "English (US)",
volume = "27",
pages = "487--493",
journal = "American Journal of Surgical Pathology",
issn = "0147-5185",
publisher = "Lippincott Williams and Wilkins",
number = "4",

}

TY - JOUR

T1 - Primary anaplastic large cell lymphoma of the central nervous system

T2 - Prognostic effect of ALK-1 expression

AU - George, David H.

AU - Scheithauer, Bernd W.

AU - Aker, Fügen V.

AU - Kurtin, Paul J.

AU - Burger, Peter C.

AU - Cameselle-Teijeiro, José

AU - McLendon, Roger E.

AU - Parisi, Joseph E.

AU - Paulus, Werner

AU - Roggendorf, Wolfgang

AU - Sotelo, Cirilo

PY - 2003/4/1

Y1 - 2003/4/1

N2 - 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 ≤ 22 years, 3 ≥ 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.

AB - 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 ≤ 22 years, 3 ≥ 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.

KW - ALK-1

KW - Brain

KW - Lymphoma

KW - Prognosis

UR - http://www.scopus.com/inward/record.url?scp=0037379312&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0037379312&partnerID=8YFLogxK

U2 - 10.1097/00000478-200304000-00008

DO - 10.1097/00000478-200304000-00008

M3 - Article

VL - 27

SP - 487

EP - 493

JO - American Journal of Surgical Pathology

JF - American Journal of Surgical Pathology

SN - 0147-5185

IS - 4

ER -