Characteristics, Treatment Patterns and Clinical Outcomes of Patients diagnosed with Fungating Soft Tissue Sarcomas

Objective: Fungating Soft Tissue Sarcoma is a rare type of tumor that has limited literature to describe its clinical characteristics, treatment, and outcomes. This study aims to describe important clinical characteristics, treatment patterns and outcomes of a local series of twenty patients diagnosed with Fungating Extremity Soft Tissue Sarcoma (F-ESTS) and treated by a single surgeon at a sarcoma unit. 

Methodology: We did a retrospective clinical case series study on twenty F-ESTS patients treated by the senior investigator at a sarcoma unit over a period of 25 years (1993-2018). 

Results: There is a 10.9% local incidence of F-ESTS which commonly occurred in middle-aged group with a mean age of 48.7 years old, predominantly in women. The most common site of the tumor was on the thigh (50%). The average size of the tumor on presentation at the clinic was 11.8cm, mostly were deep and 85% were high grade with liposarcoma (31%) as the most common histological diagnosis. Eleven patients (55%) had unplanned excision and two underwent diagnostic biopsies prior to consult at the sarcoma unit.  Limb salvage was done in 60% of the patients. Reconstructive procedure was done in 50% of the patients (skin graft in seven patients, flap coverage in three patients). Outcome wise, three patients developed complications (two SSI/wound dehiscence, one wound dehiscence), there was 25% local recurrence, 65% distant metastases and the mean survival was 47.8 months ranging from 2 months to 13.5 years. Sixty percent of the patients died of the disease.

Conclusion: The clinical characteristics in our F-ESTS series include patients < 65y.o., with deep and high-grade tumors, usually liposarcomas, with a predilection for the thigh, and with a previous history of surgery or biopsy. Most F-ESTS patients underwent limb salvage surgery, receiving RT if they had both deep and high-grade tumors. Our series had the following outcome: five (25%) patients developed local recurrence, thirteen (65%) patients had distant metastases, twelve (60%) died of disease with a mean time to death of 21 months from diagnosis. Most F-ESTS patients were still able to undergo limb salvage surgery. In the last 10 years, limb salvage surgery has become the treatment of choice for patients in this series. This upends the notion that fungating tumors require an amputation and may allay fears of contamination from “tumor leakage”.