title: Epithelioid sarcoma or malignant rhabdoid tumor of soft tissue? Epithelioid immunophenotype and rhabdoid karyotype.
abstract: Two children and one young adult with extremity sarcomas demonstrating an aggressive clinical behavior are described. Histologically, all three tumors displayed features compatible with a deep-seated epithelioid sarcoma or with a malignant rhabdoid tumor of soft tissue. Immunohistologically, both vimentin and epithelial antigens were demonstrated; however, no desmin was detectable. In all three cases, the DNA profile was diploid. In one case, a trisomy of chromosome 2 was found in the tumor cells, a phenomenon also observed in embryonal rhabdomyosarcomas. It is concluded that, although the immunohistologic findings support the epithelioid character of the tumors, this chromosomal finding suggests a relationship with rhabdomyosarcomas and justifies the designation of rhabdoid.
authors.affiliation: Department of Pathology, University of Groningen, The Netherlands.
authors.forename: W M
authors.initials: WM
authors.lastname: Molenaar
authors.forename: B
authors.initials: B
authors.lastname: DeJong
authors.forename: A
authors.initials: A
authors.lastname: Dam-Meiring
authors.forename: A
authors.initials: A
authors.lastname: Postma
authors.forename: J
authors.initials: J
authors.lastname: DeVries
authors.forename: H J
authors.initials: HJ
authors.lastname: Hoekstra
mesh_terms: D000328:Adult; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D007621:Karyotyping; D008297:Male; D010641:Phenotype; D011003:Ploidies; D012208:Rhabdomyosarcoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D012509:Sarcoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*