title: Clinicopathologic and radiologic features of extraskeletal myxoid chondrosarcoma: a retrospective study of 40 Chinese cases with literature review.
abstract: The aim of this study is to describe the clinicopathologic and radiologic features of 40 cases of extraskeletal myxoid chondrosarcoma (EMC) from China. There were 25 males and 15 females (sex ratio, 1.7:1). Apart from an adolescent, all patients were adults with a median age of 49years. Twenty-four tumors (60%) occurred in the lower limb and limb girdles, especially the thigh, followed by the upper limb and limb girdles (20%) and trunk (10%). Other less commonly involved locations included the head and neck, sacrococcygeal region, and perineum. Tumors ranged in size from 1.5 to 19cm (mean, 7cm). By radiology, they appeared as hypoattenuated or isoattenuated masses on computed tomography with hyperintense signal on T2-weighted magnetic resonance imaging. Intralesional hypointense septa were present in most cases. Of the 40 tumors, 30 belonged to the classic subtype, whereas 9 cases were cellular, and 1 case had a rhabdoid phenotype. Tumor cells showed variable expression of synaptophysin (36%), S-100 protein (29%), epithelial membrane antigen (11%), and neuron-specific enolase (7%). Ki-67 index was remarkably higher in the cellular variant (mean, 30%). EWSR1-related rearrangement was detected in 12 of 14 cases tested by fluorescence in situ hybridization using break-apart probes. The overall 5- and 7-year survival was 71% and 60%, respectively. Awareness of the imaging features may help pathologists in the diagnosis of EMC. Fluorescence in situ hybridization also serves as a useful diagnostic tool for EMC, especially in the distinction from its mimics.
authors.affiliation: Department of Pathology, Zhenjiang First People's Hospital, Jiangsu University, Zhenjiang 212001, China.
authors.forename: Rui
authors.initials: R
authors.lastname: Shao
authors.affiliation: Department of Pathology, Fudan University Shanghai Cancer Center, Fudan University, Shanghai 200032, China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China.
authors.forename: I Weng
authors.initials: IW
authors.lastname: Lao
authors.affiliation: Department of Pathology, Fudan University Shanghai Cancer Center, Fudan University, Shanghai 200032, China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Pathology, Fudan University Shanghai Cancer Center, Fudan University, Shanghai 200032, China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China.
authors.forename: Lin
authors.initials: L
authors.lastname: Yu
authors.affiliation: Department of Pathology, Fudan University Shanghai Cancer Center, Fudan University, Shanghai 200032, China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China. Electronic address: softtissuetumor@163.com.
authors.forename: Jian
authors.initials: J
authors.lastname: Wang
authors.affiliation: Department of Pathology, Jiangsu Province Hospital, The First Affiliated Hospital with Nanjing Medical University, Nanjing 210000, China.
authors.forename: Qinhe
authors.initials: Q
authors.lastname: Fan
mesh_terms: D000328:Adult; D000368:Aged; D002148:Calmodulin-Binding Proteins / Q000378:metabolism*; D002681:China; D002813:Chondrosarcoma / Q000175:diagnosis* / Q000378:metabolism / Q000401:mortality; D004268:DNA-Binding Proteins / Q000378:metabolism; D005260:Female; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D008875:Middle Aged; D018204:Neoplasms, Connective and Soft Tissue / Q000175:diagnosis* / Q000378:metabolism / Q000401:mortality; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000378:metabolism*; D012189:Retrospective Studies; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000401:mortality
chemical_list: D002148:Calmodulin-Binding Proteins; D004268:DNA-Binding Proteins; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins
keywords: Extraskeletal myxoid chondrosarcoma; Fluorescent in situ hybridization; Prognostic factor; Radiology