A clinicopathologically distinctive primary splenic histiocytic neoplasm. Demonstration of its histiocyte derivation by immunophenotypic and molecular genetic analysis.
We describe a primary splenic neoplasm composed of cytomorphologically malignant-appearing erythrophagocytic histiocytoid cells reminiscent of those seen in malignant histiocytosis. However, this neoplasm displayed certain distinctive clinicopathologic features--including localization to the spleen, where it grew as separate discrete nodules--that distinguish it from all previously reported cases of malignant histiocytosis. The cells expressed a monocyte/histiocyte immunophenotype and lacked clonal immunoglobulin and T-cell receptor beta-chain gene rearrangements. Our results suggest that this neoplasm represents a clinicopathologically distinctive and possibly unique tumor derived from the tissue macrophage lineage.
Franchino
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Department of Pathology, Columbia University College of Physicians and Surgeons, New York, New York 10032.
Reich
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Distenfeld
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Ubriaco
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Knowles
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DM
D000328:Adult; D006644:Histiocytes / Q000473:pathology*; D006801:Humans; D007158:Immunologic Techniques; D008297:Male; D008854:Microscopy, Electron; D008967:Molecular Biology; D010641:Phenotype; D013160:Splenic Neoplasms / Q000235:genetics / Q000473:pathology* / Q000648:ultrastructure