Clinical and pathological analysis of dermatofibrosarcoma protuberans with long-term follow-up.

BACKGROUND

Dermatofibrosarcoma protuberans (DFSP) is a rare dermal sarcoma characterised by low-grade malignancy, slow infiltrative growth, local recurrence and rare distant metastases.

OBJECTIVE

Many international studies exist on DFSP, but limited data on Asian patients are available. This article fills this gap.

METHODS

A retrospective and observational patient series based on records of 99 patients diagnosed with DFSP and treated with wide local excision and assessments of complete circumferential peripheral and deep margin from 2002 to 2018. The margin width, pathological characteristics and surgical methods of 99 patients were described. Follow-up outcomes of 43 patients were evaluated.

RESULTS

In total, 106 DFSP resections were performed in 99 Asian patients (mean age: 44 years, SD: 14 years). Locations of DFSP are the trunk (63%), extremities (23%), head and neck (9%) and groin (5%). The average margin width was 3.5 ± 0.5 (SD) cm. CD34, vimentin and AOPD positivity was found in 98%, 100% and 86% of specimens, respectively. The median value of Ki67 expression was 5% + (range 1% + ∼ 50% +). Primary closure was used for 69% of all excisions, while flaps, grafts or other closures were used for the other excision procedures. A total of 43 patients were followed up for a mean duration of 53±36 (SD) months. Six patients exhibited recurrence. The eight-year recurrence-free survival rate was 94%.

CONCLUSION

Wide local excision and margin assessments can remove DFSP tumours and control recurrence.