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Primary cutaneous ALCL with phosphorylated/activated cytoplasmic ALK and novel phenotype: EMA/MUC1+, cutaneous lymphocyte antigen negative.

Abstract
Primary cutaneous anaplastic large-cell lymphoma (ALCL) ordinarily is distinguished from systemic ALCL by clinical presentation, absence of anaplastic lymphoma kinase (ALK) expression, and immunophenotype (CLA+, EMA/MUC1-). We present an exceptional case of an elderly man with primary cutaneous ALCL and no systemic disease for a 13-year period. Recurrent skin tumors in this patient were characterized by anaplastic, often multinucleated, cells infiltrating the lymphatics and associated with pseudoepitheliomatous hyperplasia. Cutaneous lymphocyte antigen was absent and EMA/MUC1, typical of systemic ALCL, was strongly expressed by the tumor cells. Remarkably, the tumor cells expressed a cytoplasmic-only variant of ALK protein, as reported in 3 previous cases of primary cutaneous ALCL. Fluorescence in situ hybridization revealed lack of rearrangements of the chromosome 2 ALK gene locus usually involved by translocation t(2;5) or other chromosomal rearrangements that generate nucleophosmin-ALK or the variant ALK fusions that occur in systemic ALCL. Nonetheless, the cytoplasmic ALK protein in the patient's tumor cells was shown to be phosphorylated/activated, suggesting a novel mechanism of ALK activation. Primary cutaneous ALCL of this novel subtype should be distinguished from systemic ALCL to ensure proper clinical management.
AuthorsMarshall E Kadin, Jack L Pinkus, Geraldine S Pinkus, Ivan H Duran, Christine E Fuller, Mihaela Onciu, Hiroyuki Kawaguchi, Stephan W Morris
JournalThe American journal of surgical pathology (Am J Surg Pathol) Vol. 32 Issue 9 Pg. 1421-6 (Sep 2008) ISSN: 1532-0979 [Electronic] United States
PMID18670345 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
Chemical References
  • Antigens, Differentiation, T-Lymphocyte
  • Antigens, Neoplasm
  • CTAGE1 protein, human
  • Membrane Glycoproteins
  • Mucin-1
  • ALK protein, human
  • Anaplastic Lymphoma Kinase
  • Protein-Tyrosine Kinases
  • Receptor Protein-Tyrosine Kinases
Topics
  • Age of Onset
  • Aged
  • Anaplastic Lymphoma Kinase
  • Antigens, Differentiation, T-Lymphocyte
  • Antigens, Neoplasm (biosynthesis)
  • Enzyme Activation (physiology)
  • Humans
  • In Situ Hybridization, Fluorescence
  • Lymphoma, Large-Cell, Anaplastic (genetics, metabolism, pathology)
  • Male
  • Membrane Glycoproteins (biosynthesis)
  • Middle Aged
  • Mucin-1 (biosynthesis)
  • Neoplasm Recurrence, Local (metabolism, pathology)
  • Phenotype
  • Phosphorylation
  • Protein-Tyrosine Kinases (genetics, metabolism)
  • Receptor Protein-Tyrosine Kinases
  • Reverse Transcriptase Polymerase Chain Reaction
  • Skin Neoplasms (genetics, metabolism, pathology)

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