FUNGATING SOFT TISSUE TUMOURS OF LIMB AND LIMB GIRDLE: A 10-YEAR SINGLE CENTRE EXPERIENCE IN THE NORTH WEST OF ENGLAND

Abstract

Background: The literature on description and management of advanced fungating soft tissue tumours (FSST) is limited because of the rarity of cases. Recent advances in diagnostic resources and an increased awareness of the disease has made early recognition easier. Manchester Royal Infirmary is a Regional Sarcoma Centre in the North West of England. We describe our experiences in managing patients with FSST of the extremities.

Patients and Methods: Between 1997 and 2007, 18 patients presented with FSST of the extremities (13 involving the lower limb, and 5 involving the upper limb), and 1 patient with a sarcoma involving the scapular region (limb girdle). The cohort included 14 males and 5 females with a mean average age of 68.5 ± 13.7 years. Follow-up ranged from a minimum of 6 months to 10 years from the initial referral.

Results: The histological diagnosis was sarcoma in 15 patients, subclassified into spindle cell sarcoma (4), fibrous histiocytoma (2), pleomorphic sarcoma (3), liposarcoma (2), leiomyosarcoma (2), fibrosarcoma (1) and round cell sarcoma (1). In the remaining 3 patients immunohistochemistry studies confirmed a metastatic squamous cell sarcoma, a metastatic malignant melanoma and a metastasis from a poorly differentiated upper gastrointestinal malignancy. Lung metastases were present at the time of referral in 6 patients and developed later during follow-up in 4 patients.

For patients where curative surgery was an option, primary wide local excision (15 patients) or primary amputation (2 patients) was performed. The remaining 2 patients presented with unresectable disease due to the location and localised spread; an embolisation was performed for palliation in both cases. Revision surgery was needed in 9 patients for either a positive resection margin confirmed by histology, or a recurrence; these included 3 secondary amputations. A histologically proven recurrence occurred in 6 patients after an average of 15.8 (4 to 41) months. Local adjuvant radiotherapy was administered to 7 patients and a combination of radio–and chemotherapy was used in 2 patients for metastases. Mortality was 53% (9 patients) by the end of 36 months follow-up period.

Conclusion: Fungation in soft tissue tumours is a rare phenomenon and often a sign of locally advanced disease with a high grade nature. Patients present with either systemic spread, or have a tendency to develop metastases despite good local disease control. Primary wide local excision is difficult with a high chance of a positive margin; hence primary amputation may be better for local clearance. Tumour recurrence and revision surgery, however, is common. We report a mortality rate of > 50% at the end of 3 years from presentation to treatment.