Dermatofibrosarcoma Protuberans of the Breast—a Rare Entity

Abstract

Dermatofibrosarcoma protuberans (DFSP) represents about 1% of soft-tissue sarcomas with an estimated incidence of 0.8 to 5.0 cases per million per year. This lesion may occur anywhere in the body but more than 50% occur on the trunk, 20% on the head and neck and 30% on the extremities. DFSP of the breast is an extremely uncommon site of presentation. Data regarding DFSP of the breast is limited and mostly in the form of case reports. Clinical presentation is not uniform and may mimic benign skin lesions [1]. However, it typically presents as a nodular cutaneous mass in early or mid-adult life. We herein report a case of DFSP of the breast in a 33-year-old lady who was managed successfully in our institute and review the literature associated with it.

Dermatofibrosarcoma Protuberans Mimicking Primary Breast Neoplasm: A case report and literature review

“…These mammography findings are not specific and hence, breast DFSP can mimic a benign breast lesion such as fibroadenoma, sebaceous cyst or an abscess. 1 , 4 , 5 , 9 , 15 In the current case, the mammography findings were consistent with findings reported in the literature with a skin-based well-defined lesion with no microcalcification nor architectural distortion, mimicking a sebaceous cyst.…”

“…Another useful ultrasonographic finding that can help to distinguish DFSP is the presence of a peripheral hyperechoic rim in the subcutaneous tissue that represents a mixture of tumour cells and fibrous tissue infiltrating the subcutaneous fat. 1,2,4,5,9,11 On magnetic resonance imaging (MRI), DSFP has no specific features and appears as a well-defined nodule or mass that is T1 isointense to the muscle and shows homogenous or heterogenous enhancement on post-contrast examination. However, owing to its high spatial resolution and high tissue characterisation, MRI is very helpful in delineation of tumour extent and evaluating the anatomical relation of the tumour to adjacent structures.…”

“…On physical examination, there was a nodular pinkish skin lesion over the upper-inner quadrant D ermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue sarcoma that commonly affects young to middle aged adults. 1 It has intermediate-to lowgrade malignancy with a high propensity for local infiltration as well as having a high local recurrence rate after excision. 2 DFSP most commonly involves the trunk, followed by the extremities, and the head and neck area.…”

“…3 However, DFSP very rarely involves the breast, mimicking primary breast tumour. 1,2,4,5 Awareness of this rare entity and its typical imaging features is crucial to avoid the misdiagnosis of DFSP with a benign breast lesion and to suggest a preoperative diagnosis. However, reports of imaging features have been scarce in the literature compared to histopathological features, with few reported cases of the left breast, which was mobile and non-tender, measuring 3 × 3 cm.…”

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

“…Most cases of breast DFSP followed guidelines. A few cases indicated no local recurrence or metastasis with a surgical margin width of less than 2 cm [ 29 , 45 , 46 ]. Nevertheless, most of them reported a short follow-up period.…”