Adequate surgical margins for dermatofibrosarcoma protuberans - A multi-centre analysis.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive tumour. Adequate margins have a positive impact on recurrence rates. The aim of this study is to assess how adequate margins are achieved and secondly which additional treatment modalities might be necessary to achieve adequate margins. MATERIAL & METHODS: Patients with DFSP treated between 1991 and 2016 at three tertiary centres were included. Patient- and tumour characteristics were obtained from a prospectively held database and patient files. RESULTS: A total of 279 patients with a median age of 39 (Interquartile range [IQ], 31-50) years and a median follow-up of 50 (IQ, 18-96) months were included. When DFSP was preoperatively confirmed by biopsy and resected with an oncological operation in a tertiary centre, in 86% was had clear pathological margins after one excision. Wider resection margins were significantly correlated with more reconstructions (p = 0.002). A substantial discrepancy between the primary surgical macroscopic and the pathological margins was found with a median difference of 22 (range, 10-46) mm (Fig. 1). There was no significant influence of the width of the pathological clear margins (if > 1 mm) and the recurrence rate (p = 0.710). CONCLUSION: The wider the resection margins, the more likely it is to obtain clear pathological margins, but the more likely patients will need any form of reconstruction after resection. The aim of the primary excision should be wide surgical resection, where the width of the margin should be balanced against the need for reconstructions and surgical morbidity.

Isolated limb perfusion is an effective treatment modality for locally advanced Kaposi sarcoma of the extremities.

INTRODUCTION: Kaposi sarcoma (KS) is a rare soft tissue sarcoma. In case of locally advanced disease, mutilating surgery such as amputations or major reconstructive procedures are sometimes inevitable. The aim of this study was to evaluate the effectiveness of isolated limb perfusion (ILP) in patients with locally advanced KS of the extremities. MATERIAL AND METHODS: All patients who underwent ILP for KS between 1996 and 2018 at Erasmus MC, Rotterdam were identified. Clinical data was obtained from either a prospectively maintained database or retrospective assessment of patient files. RESULTS: A total of 14 primary ILP's were performed in 11 patients. Median follow-up from primary ILP was 30 months (range, 5-98). The overall response rate of primary ILP was 100%, with a complete response (CR) rate of 50%. Only minimal local toxicity (Wieberdink I-III) was observed. Local progressive disease occurred after eight primary ILP's (57%) with a median local progression free survival (PFS) of 18 months (95% confidence interval [CI]: 7.0-28.9). Subsequently, four (46%) patients received a total of 5 recurrent ILP's. After the recurrent ILP on the same leg, the overall response rate was 75% and a CR-rate of 50%. One patient needed amputation post-operatively resulting in a limb salvage rate of 91%. One (9%) patient developed metastases four months after ILP. CONCLUSIONS: ILP is a highly effective treatment modality with very limited morbidity rates for patients with locally advanced KS of the extremity. ILP should be considered as a treatment modality for locally advanced KS of the extremities.

Isolated limb perfusion for locally advanced angiosarcoma in extremities: A multi-centre study.

BACKGROUND: Angiosarcomas are rare and aggressive soft-tissue sarcomas. The only potential curative treatment is complete surgical excision. This study reports the outcome of isolated limb perfusion (ILP) with high-dose melphalan and tumour necrosis factor α for locally advanced angiosarcoma. MATERIAL AND METHODS: All patients who underwent an ILP for angiosarcomas between 1991 and 2016 in three tertiary referral centres were identified from prospectively maintained databases. RESULTS: A total of 39 patients were included, with a median follow-up of 18 months (interquartile range 6.1-60.8). Of these patients, 23 (58.9%) patients had a complete response (CR) after ILP, 10 (25.6%) had a partial response, 4 (10.3%) had stable disease and 2 (5.1%) patients had progressive disease immediately after ILP. A total of 22 patients developed local progression (56.4%), whereas nine (23.1%) developed distant metastases. The patients with CR had a significantly prolonged median local progression-free survival (PFS) (15.4 versus 7.3 months, p = 0.015) when compared with non-CR patients, and a trend towards better median overall survival (81.2 versus 14.5 months, p = 0.054). Six patients underwent multiple ILPs, whereby the CR rate of the first, second and third ILPs were 60%, 80% and 67%, respectively. Thirteen (33.3%) patients needed further surgical intervention, consisting of resection in eight patients (20.5%) and amputation in five patients (12.8%). CONCLUSION: ILP is an effective treatment option for patients with locally advanced angiosarcoma in the extremities, resulting in a high number of CRs, a high limb salvage rate and prolonged local PFS.