Malignant rhabdoid tumor arising from soft parts of the right thigh with unusual neurologic manifestation: report of a case.
A case of malignant rhabdoid tumor (MRT) arising from the soft tissue of the right thigh in a 49-year-old Chinese female with peripheral neuropathy is reported. The tumor, exhibiting the salient features of MRT, was composed of sheets and nests of polygonal cells with prominent nucleoli and characteristic paranuclear inclusion-like hyaline globules under light microscopy which corresponded to aggregates of intermediate filaments under electron microscopy. The results of immunohistochemical studies of the tumor cells were also characteristic: cytokeratin (+), vimentin (+), epithelial membrane antigen (EMA) (+), desmin (-), myoglobin (-), leukocyte common antigen (LCA) (-), kappa (-), lambda (-), IgG (-) and IgA (-). Serologic study revealed an M-component of IgA. The clinical evolution of the patient was highly aggressive and inevitably lethal. An adult malignant rhabdoid tumor is unusual, and its association with peripheral neuropathy and the coexistence of an M-component of IgA in this case appears to be unique. In this report, the differential diagnosis of histopathologic features, the association of peripheral neuropathy and the coexistence of an M-component of IgA are discussed.
Lee
T L
TL
Department of Pathology, Taipei Municipal Ho-Ping Hospital, Taiwan, R.O.C.
Lee
S H
SH
Lin
R Y
RY
Chuang
S M
SM
Leu
S H
SH
D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000209:etiology*; D012208:Rhabdomyosarcoma / Q000150:complications* / Q000276:immunology / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000150:complications* / Q000276:immunology / Q000473:pathology; D013848:Thigh
D007070:Immunoglobulin A