Document Detail

Primary leptomeningeal ALK+ lymphoma in a 13-year-old child.
MedLine Citation:
PMID:  19131793     Owner:  NLM     Status:  MEDLINE    
A distinct pathologic entity characterized by expression of the anaplastic lymphoma kinase (ALK) protein (hence described as ALK lymphoma) has emerged within the heterogeneous group of CD30 anaplastic large-cell lymphomas. Central nervous system (CNS) involvement is extremely rare in anaplastic large-cell lymphoma. In children, only isolated cases have been reported, mainly as secondary CNS involvement. We report on a 13-year-old boy presenting with headaches and diplopia. Cerebrospinal fluid was infiltrated with atypical large granular lymphocytes. Magnetic resonance imaging of the brain revealed leptomeningeal enhancement. A frontal lobe biopsy showed a pleomorphic neoplasm diffusely infiltrating the meninges composed of large cells with bizarre nuclei similar to those evidenced in cerebrospinal fluid. Immunohistochemical stains showed diffuse strong positivity for CD8, CD30, anaplastic lymphoma kinase protein: p80 and negative monocyte-macrophage and B cell markers. TCR gamma was clonally rearranged. This finding was confirmed by reverse transcription-polymerase chain reaction analysis of the NPM/ALK fusion protein. Epstein-Barr virus was not detected. No evidence of extra-CNS disease was found by imaging study, cytologic examination, or molecular studies. The patient underwent complete remission with polychemotherapy followed by a CNS irradiation. At +10 months from onset, he suffered a full relapse. After a short-term remission with vinblastine, he underwent nonmyeloablative allogeneic bone marrow transplantation, but unfortunately died from multiple organ failure. This case is the first reported occurrence of a primary meningeal ALK lymphoma in a child.
Etienne Merlin; Stéphane Chabrier; Virginie Verkarre; Elisabeth Cramer; Eric Delabesse; Jean-Louis Stéphan
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  30     ISSN:  1536-3678     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  2008 Dec 
Date Detail:
Created Date:  2009-01-09     Completed Date:  2009-02-03     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  963-7     Citation Subset:  IM    
CHU Clermont-Ferrand, Centre Régional de Cancérologie et Thérapie Cellulaire Pédiatrique, Hôtel-Dieu, France.
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MeSH Terms
DNA, Neoplasm / analysis
Fatal Outcome
Gene Rearrangement
Genes, T-Cell Receptor gamma / genetics
Lymphoma, Large-Cell, Anaplastic / complications,  drug therapy,  metabolism,  pathology*
Meningeal Neoplasms / drug therapy,  metabolism,  pathology*
Multiple Organ Failure
Protein-Tyrosine Kinases / cerebrospinal fluid,  genetics,  metabolism*
Reg. No./Substance:
0/DNA, Neoplasm; EC 2.7.1.-/p80(NPM-ALK) protein; EC Kinases; EC lymphoma kinase

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