Dermatofibrosarcoma protuberans of the breast: A rare breast tumor mimicking a cutaneous lesion

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor which generally affects people from the second to the fifth decades of life, with the same incidence in both sexes. DFSP can appear as a slow-growing, flesh-colored or erythematous plaque or nodule, often becoming protuberant if untreated.

We report two cases of DFSP. The first case regards a 22-year-old woman with a 1.5-centimeter, mobile nodule of hard-elastic consistency in the left breast initially suspected to be a sebaceous cyst. Ultrasound and MRI suggested benign features, but histopathological examination post-excision confirmed DFSP. The second patient come to our attention is a 54-year-old woman with a 6 mm erythematous lump in the right breast, which reached 22 mm after two years. Mammography and histological examination post-excision confirmed DFSP. Both patients underwent to wide local excision and after four years of follow-up no recurrence or complications are observed.

DFSP is a soft tissue sarcoma with low metastatic potential but requires early diagnosis and surgical excision to avoid malignant transformation. There are no standardized guidelines for its diagnosis and treatment. Imaging techniques, including ultrasound, CT, and MRI, are crucial to define tumor extension and planning surgical intervention. Surgical excision with clear margins is the primary treatment, but there are also emerging treatments as Mohs micrographic surgery or Imatinib Mesylate therapy for unresectable cases.

Our cases highlight the importance of an accurate diagnostic evaluation and effective surgical management to achieve favorable outcomes.