title: Dedifferentiated chondrosarcoma in patients with multiple osteochondromatosis: report of a case and review of the literature.
abstract: Multiple osteochondromatosis (MOS) is a familial disorder of autosomal dominant transmission characterized by the development of multiple exostoses and often derangements of epiphyseal cartilage, sometimes resulting in long bone growth retardation. Patients with the disorder appear to be at increased risk for developing secondary chondrosarcomas. Rarely, dedifferentiated chondrosarcomas may also occur. We report a single case of a 27-year-old man with multiple osteochondromatosis who developed a fatal dedifferentiated chondrosarcoma. Radiographically, the neoplasm arose from the pelvis completely destroying the left pubic ramus. Subsequently, the patient underwent preoperative chemotherapy followed by a left external hemipelvectomy. On pathologic examination, the tumor was characterized by high-grade pleomorphic sarcoma sharply juxtaposed to a low-grade chondrosarcoma. The patient ultimately died of widespread metastatic sarcoma.
authors.affiliation: Department of Pathology, North Carolina Baptist Hospitals, Bowman Gray School of Medicine of Wake Forest University, Winston-Salem, NC 27157-1072, USA.
authors.forename: S E
authors.initials: SE
authors.lastname: Kilpatrick
authors.forename: E J
authors.initials: EJ
authors.lastname: Pike
authors.forename: W G
authors.initials: WG
authors.lastname: Ward
authors.forename: T L
authors.initials: TL
authors.lastname: Pope
mesh_terms: D000328:Adult; D001707:Biopsy, Needle; D002813:Chondrosarcoma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D018216:Osteochondromatosis / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy; D014057:Tomography, X-Ray Computed