Primary anaplastic large cell lymphoma of the central nervous system: prognostic effect of ALK-1 expression. Export

@article{citeulike:2783566, 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.}, address = {Department of Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.}, author = {George, D. H. and Scheithauer, B. W. and Aker, F. V. and Kurtin, P. J. and Burger, P. C. and Cameselle-Teijeiro, J. and McLendon, R. E. and Parisi, J. E. and Paulus, W. and Roggendorf, W. and Sotelo, C.}, citeulike-article-id = {2783566}, citeulike-linkout-0 = {http://view.ncbi.nlm.nih.gov/pubmed/12657933}, citeulike-linkout-1 = {http://www.hubmed.org/display.cgi?uids=12657933}, issn = {0147-5185}, journal = {The American journal of surgical pathology}, keywords = {anaplastic-large-cell-lymphoma, lymphoma, neuro-oncologie}, month = {April}, number = {4}, pages = {487--493}, posted-at = {2008-05-11 02:13:48}, priority = {2}, title = {Primary anaplastic large cell lymphoma of the central nervous system: prognostic effect of ALK-1 expression.}, url = {http://view.ncbi.nlm.nih.gov/pubmed/12657933}, volume = {27}, year = {2003} }

TY - JOUR ID - citeulike:2783566 L3 - citeulike-article-id:2783566 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 < 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. IS - 4 JF - The American journal of surgical pathology SN - 0147-5185 AD - Department of Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. EP - 493 TI - Primary anaplastic large cell lymphoma of the central nervous system: prognostic effect of ALK-1 expression. VL - 27 SP - 487 KW - anaplastic-large-cell-lymphoma KW - lymphoma KW - neuro-oncologie AU - George, DH AU - Scheithauer, BW AU - Aker, FV AU - Kurtin, PJ AU - Burger, PC AU - Cameselle-Teijeiro, J AU - McLendon, RE AU - Parisi, JE AU - Paulus, W AU - Roggendorf, W AU - Sotelo, C PY - 2003/04// UR - http://view.ncbi.nlm.nih.gov/pubmed/12657933 ER -