PAPER 180: FUNGATING SOFT TISSUE TUMORS OF LIMB AND LIMB GIRDLE

Abstract

Purpose: The literature on management of advanced soft tissue tumours is limited because of the rarity of cases following increased awareness and improved diagnostic resources.

Method: Our experience of managing 18 patients with fungating soft tissue tumours of the extremities and one patient with a sarcoma involving the scapular region (limb girdle) is presented. There were 14 males and 5 females. Average age was 70.6 yrs ranging between 37 – 98 years. 13 tumours involved lower limb and 6 the upper limb.

Results: The follow-up ranged from a minimum of 6 months to 10 years from the initial referral. The histological diagnosis was Sarcoma in 15 patients (Spindle cell sarcoma in 4, Fibrous Histiocytoma in 2, Pleomorphic sarcoma in 3, liposarcoma in 2, leiomyosarcoma in 2, Fibrosarcoma in 1 and 1 Round cell sarcoma). In the remaining 3 patients immunohistochemistry studies confirmed a Metastatic Squamous cell Sarcoma, a Metastatic Malignant Melanoma and a Metastases from a poorly differentiated upper GI malignancy each. Primary wide local excision was performed in 15 patients and primary amputation was performed in two patients. In 2 patients when tumour was unresectable due to the location and local spread, an embolisation was performed in both for palliation. Lung Metastases were present at the time of referral in 6 patients and developed later during follow-up in 4 patients. A histologically proven recurrence occurred in 6 patients after an average of 15.83 (4 to 41) months. Revision surgery was needed in 9 patients for either a positive margin on histology or a recurrence, including 3 secondary amputations. Local adjuvant Radiotherapy was given for 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 32 months of follow-up.

Conclusion: Fungation in soft tissue tumours is rare and often a sign of locally advanced disease and a high grade nature, patients either have systemic spread by the time or develop later inspite of good local disease control. Primary wide local excision in such patients is difficult and has a high chance of a positive margin hence primary amputation may be better for local clearance. Recurrence of tumour and revision surgery is common and the mortality was > 50% at the end of 3 years from presentation to treatment in our series.