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Clear‐cell sarcoma of the soft tissue – a rare diagnosis with a fatal outcome
Author(s) -
IPACH I.,
MITTAG F.,
KOPP H.G.,
KUNZE B.,
WOLF P.,
KLUBA T.
Publication year - 2012
Publication title -
european journal of cancer care
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.849
H-Index - 67
eISSN - 1365-2354
pISSN - 0961-5423
DOI - 10.1111/j.1365-2354.2011.01318.x
Subject(s) - medicine , soft tissue , sarcoma , soft tissue sarcoma , surgery , medical record , lesion , disease , metastasis , radiology , cancer , pathology
IPACH I., MITTAG F., KOPP H.‐G., KUNZE B., WOLF P. & KLUBA T. (2012) European Journal of Cancer Care 21 , 412–420 Clear‐cell sarcoma of the soft tissue – a rare diagnosis with a fatal outcome 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.