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. 2025 Jan 2;17(1):e76786.
doi: 10.7759/cureus.76786. eCollection 2025 Jan.

Dermatofibrosarcoma Protuberans of the Breast: A Clinicopathological Study of a Rare Cutaneous Low-Grade Sarcoma

Fatima Safdar  1 , Nasir Ud Din  2 , Abida K Sattar  3 , Syeda Samia Fatima  1 , Romana Idress  1
Affiliations

Dermatofibrosarcoma Protuberans of the Breast: A Clinicopathological Study of a Rare Cutaneous Low-Grade Sarcoma

Fatima Safdar et al. Cureus. .

Abstract

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.

Keywords: breast; dermal lesion; dermatofibrosarcoma protuberans; low grade; sarcoma.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Aga Khan University Ethics Review Committee issued approval 2022-6912-21068. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. (A) The image shows a multilobulated lesion ulcerating the overlying skin. (B) The image shows a circumscribed, multinodular, tan-white lesion.
Figure 2
Figure 2. (A) Proliferation of spindle cells with characteristic honeycomb appearance and breast parenchyma at the periphery (H&E, X10). (B): Classic storiform pattern of dermatofibrosarcoma protuberans (DFSP) (H&E, X20). (C): DFSP transition with fibrosarcomatous areas (H&E, X20). (D): Loss of CD34 in fibrosarcomatous areas (H&E, X20).
H&E: hematoxylin and eosin; DFSP: dermatofibrosarcoma protuberans
See this image and copyright information in PMC

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