A distinctive cutaneous malignant neoplasm expressing the Langerhans cell phenotype. Synchronous occurrence with B-chronic lymphocytic leukemia.
The authors describe a 63-year-old woman who developed a histologically distinctive malignant cutaneous neoplasm composed of large pleomorphic cells with abundant cytoplasm and multilobate, often clefted nuclei that occasionally contained small nucleoli. This neoplastic cell population metastasized to a regional lymph node already involved by a B-cell derived chronic lymphocytic leukemia expressing surface IgMk, BA-1, and OKT1. The large metastatic tumor cells lacked surface immunoglobulin, B-lymphocyte associated antigen BA-1, T-lymphocyte associated antigens OKT1 and OKT3, and the monocyte/macrophage markers lysozyme and alpha 1-antichymotrypsin. These tumor cells expressed HLA-DR antigens, adenosine triphosphatase (ATPase), OKT6, and contained S-100 protein, i.e., they expressed the phenotype peculiar to epidermal Langerhans cells. The typical clinical and histologic features of Histiocytosis X were absent. Thus, this case appears to represent a distinctive cutaneous neoplasm composed entirely of malignant cells of dendritic cell origin which, by immunophenotypic and histochemical analysis, appear to be related to epidermal Langerhans cells.
D000911:Antibodies, Monoclonal; D001402:B-Lymphocytes / Q000473:pathology*; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D007158:Immunologic Techniques; D007801:Langerhans Cells / Q000473:pathology; D007945:Leukemia, Lymphoid / Q000473:pathology*; D008207:Lymphatic Metastasis; D008854:Microscopy, Electron; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology*
D000911:Antibodies, Monoclonal