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Dermatofibrosarcoma Protuberans of the Breast: A Clinicopathological Study of a Rare Cutaneous Low-Grade Sarcoma
Fatima Safdar1, Nasir Ud Din2, Abida K Sattar3
1Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, PAK.
Background:
Dermatofibrosarcoma protuberans (DFSP) of the breast is a rare entity. It is a slow-growing soft tissue tumor of low to intermediate grade. The risk of metastasis is low, but its likelihood of local recurrence is significant.
Objective:
Our study aims to present the clinical and histological features of DFSP of breast and follow-up.
Material And Methods:
Patients with histologically proven DFSP between 01 January 2010 and 31 October 2023 were identified from a prospectively maintained pathology database. Two senior pathologists reviewed the clinical data and histological slides, and a follow-up was obtained.
Results:
Twenty-six cases of DFSP breast were diagnosed between 01 January 2010 and 31 October 2023. Out of 26, 10 (38.5%) were male and 16 (61.5%) were female. The mean age of presentation was 37.2 years in females and 40.7 years in males. The mean tumor size in females was 4.7 cm and in males was 5.4 cm. Histologically, the 15 DFSP cases (58%) showed spindle cells arranged in a storiform pattern with honeycomb-type fat infiltration. Fibrosarcomatous transformation was noted in 11 (42%) cases comprising a fascicular pattern. The median follow-up period was 6.1 years. Three (12%) patients experienced recurrence. No recurrence was observed in 23 (88%) patients with complete surgical resection.
Conclusions:
We present the largest series of breast DFSP. The recurrence rate of 12% aligns with the DFSP of other common sites. Fibrosarcomatous transformation in breast DFSP (42%) is higher as compared to DFSP in other common locations and its long-term clinical behavior cannot be reliably predicted due to lack of long-term follow-up.
