Abstract
Clear cell sarcoma of soft tissues is a rare, poorly understood tumour with little written about it in peer reviewed literature. The aim of this paper is to present a consecutive series of patients treated at our institution.
All patients were staged using the system of the musculo-skeletal tumour society (MSTS). The aim of surgery was to achieve a wide excision. Adjuvant chemotherapy or radiotherapy was used in some patients depending on the margins, age and general health of the patient. Follow-up comprised clinical examination, magnetic resonance imaging (MRI) of the tumour bed and chest x-rays. Patients were seen 3 monthly for the first 2 years and then 6 monthly.
Between 1997 and 2003 14 patients were included. There were 5 males and 9 females with a mean age of 49 years (21–82). Mean follow-up was 42 months (1–84). Seven tumours occurred in the upper limb and 7 in the lower limb. Four patients were lymph node positive at presentation. The mean maximum diameter of the tumour was 5.6 cm (2–8). Ten patients were referred prior to excision but 4 patients had already undergone inadvertent excision biopsy elsewhere. Four patients developed local recurrence and 3 patients developed metastases. Seven patients remain disease free, 2 have no active disease, 1 is alive with disease and 4 have died of the disease.
The 2 year survival in this series is 71%. Poor prognostic factors include positive lymph nodes at diagnosis, maximum diameter of the tumour greater than 5cm and incomplete initial excision. It is important that these patients are treated early and that wide excision is achieved. We recommend early referral to a recognised musculo-skeletal tumour centre.
The abstracts were prepared by Mr Roger Tillman. Correspondence should be addressed to BOOS at the Royal College of Surgeons, 35–43 Lincoln’s Inn Fields, London WC2A 3PN
