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.
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Authors | Marshall E Kadin, Jack L Pinkus, Geraldine S Pinkus, Ivan H Duran, Christine E Fuller, Mihaela Onciu, Hiroyuki Kawaguchi, Stephan W Morris |
Journal | The American journal of surgical pathology
(Am J Surg Pathol)
Vol. 32
Issue 9
Pg. 1421-6
(Sep 2008)
ISSN: 1532-0979 [Electronic] United States |
PMID | 18670345
(Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
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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
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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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