title: Poorly differentiated synovial sarcoma: an analysis of clinical, pathologic, and molecular genetic features.
abstract: Poorly differentiated synovial sarcoma is a variant of synovial sarcoma in which the tumor cells lack the bland spindle cell appearance of the usual type monophasic synovial sarcoma. Although poorly differentiated synovial sarcoma has been recognized as an entity for many years, no series addressing the clinicopathologic features of this variant have appeared. We describe the histologic, immunohistologic, and molecular findings of a series of 20 poorly differentiated synovial sarcomas. Three types of poorly differentiated synovial sarcoma can be recognized: a large cell epithelioid variant, a small cell variant, and a high-grade spindle cell variant. Epithelial membrane antigen reactivity was seen in 95% of cases, and reactivity for cytokeratin was seen in 42%. The S100 antigen was expressed in 63% of cases. Electron microscopic findings in poorly differentiated synovial sarcoma parallel those found in usual type synovial sarcoma. In 10 cases, material was available for molecular studies; 9 of 10 cases showed the presence of t(X;18) or the associated fusion gene product. These data indicate that poorly differentiated synovial sarcoma is a lesion that shares immunologic, ultrastructural, and molecular characteristics with the usual synovial sarcoma. Follow-up data were available in 16 patients with a mean follow-up of 39 months. Eight patients died with a mean survival time of 33 months. Poorly differentiated synovial sarcoma is a variant of synovial sarcoma that may be associated with a poor prognosis.
authors.affiliation: Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, USA.
authors.forename: M
authors.initials: M
authors.lastname: van de Rijn
authors.forename: F G
authors.initials: FG
authors.lastname: Barr
authors.forename: Q B
authors.initials: QB
authors.lastname: Xiong
authors.forename: M
authors.initials: M
authors.lastname: Hedges
authors.forename: J
authors.initials: J
authors.lastname: Shipley
authors.forename: C
authors.initials: C
authors.lastname: Fisher
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D002887:Chromosomes, Human, Pair 18 / Q000235:genetics; D015342:DNA Probes / Q000737:chemistry; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013584:Sarcoma, Synovial / Q000737:chemistry / Q000235:genetics* / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000737:chemistry / Q000235:genetics* / Q000473:pathology*; D014178:Translocation, Genetic; D014960:X Chromosome / Q000235:genetics
chemical_list: D014408:Biomarkers, Tumor; D015342:DNA Probes; D004273:DNA, Neoplasm