1. Clear-cell sarcoma of the soft tissue--a rare diagnosis with a fatal outcome.
- Author
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Ipach I, Mittag F, Kopp HG, Kunze B, Wolf P, and Kluba T
- Subjects
- Adult, Aged, Female, Follow-Up Studies, Germany, Humans, Kaplan-Meier Estimate, Male, Middle Aged, Neoplasm Recurrence, Local, Outcome Assessment, Health Care, Rare Diseases mortality, Rare Diseases pathology, Rare Diseases surgery, Sarcoma, Clear Cell mortality, Sarcoma, Clear Cell pathology, Sarcoma, Clear Cell surgery, Soft Tissue Neoplasms mortality, Soft Tissue Neoplasms pathology, Soft Tissue Neoplasms surgery
- 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., (© 2011 Blackwell Publishing Ltd.)
- Published
- 2012
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