title: Clear-cell sarcoma of the soft tissue--a rare diagnosis with a fatal outcome.
abstract: Clear-cell sarcomas account for less than 1% of all soft tissue tumours. They most often occur in middle-aged adults as a deeply located lesion with predilection to the tendons and aponeuroses. The aim of the present study was to show possible influencing factors on the outcome after surgical treatment in a detailed case series. We reviewed the medical records of 11 patients with the diagnosis of a clear-cell sarcoma of the soft tissue. These cases were analysed with regard to age, gender, localisation, tumour size, recurrence free survival and overall survival. A minimum follow up of 12 months was achieved. The mean age at the point of diagnosis was 47.9 years. Metastases occurred after a mean of 19.2 months. In the cases with a tumour diameter >5 cm, metastases occurred earlier. When treated in a specialist centre, metastases occurred later. Patients died a mean of 18.4 months after developing metastatic disease. Patients with tumour size >5 cm at the point of primary diagnosis died earlier than patients with a tumour size <5 cm. It is important to detect clear-cell sarcomas as soon as possible and the final surgical treatment should be performed in a centre familiar with the treatment of soft tissue tumours not only to prolong overall survival, but also to treat the patient in a multiprofessional team.
authors.affiliation: Department of Orthopaedic Surgery, University Hospital of Tübingen, Hoppe-Seyler-Strasse 3, Tübingen, Germany.
authors.forename: I
authors.initials: I
authors.lastname: Ipach
authors.forename: F
authors.initials: F
authors.lastname: Mittag
authors.forename: H-G
authors.initials: HG
authors.lastname: Kopp
authors.forename: B
authors.initials: B
authors.lastname: Kunze
authors.forename: P
authors.initials: P
authors.lastname: Wolf
authors.forename: T
authors.initials: T
authors.lastname: Kluba
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D005858:Germany; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D017063:Outcome Assessment, Health Care; D035583:Rare Diseases / Q000401:mortality / Q000473:pathology / Q000601:surgery; D018227:Sarcoma, Clear Cell / Q000401:mortality / Q000473:pathology / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery