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[Dermatofibrosarcoma protuberans of the breast: a case report]
1Dipartimento di Scienze Morfologiche, Sezione di Anatomia Patologica, Università di Modena e Reggio Emilia, Policlinico, Via del Pozzo 71, I-41100 Modena, Italia.
Pathologica
|January 24, 2003
Summary
Dermatofibrosarcoma protuberans (DFSP) of the breast is a rare tumor. This case highlights the diagnostic challenges, emphasizing the need for careful histopathological evaluation to distinguish it from other spindle cell lesions.
Area of Science:
- Oncology
- Dermatopathology
- Surgical Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue tumor.
- Mammary DFSP is exceptionally rare, posing diagnostic challenges due to its insidious presentation.
- Distinguishing DFSP from other spindle cell neoplasms of the breast is critical for appropriate management.
Observation:
- A 27-year-old woman presented with a rapidly growing breast nodule during pregnancy, initially suspected to be a fibroadenoma.
- Imaging revealed a well-defined intraparenchymal lesion, further complicating preoperative diagnosis.
- Histopathological examination demonstrated a monomorphic spindle cell proliferation with a storiform pattern, infiltrating mammary tissue.
Findings:
- Immunohistochemistry revealed tumor cells positive for vimentin and CD34, and negative for markers like CD99, bc-2, desmin, S100, and cytokeratins.
- The diagnosis of mammary dermatofibrosarcoma protuberans (DFSP) was confirmed.
- Microscopic examination showed a low mitotic rate and absence of necrosis.
Implications:
- Mammary DFSP requires careful surgical management, often necessitating wide local excision to achieve clear margins.
- Accurate preoperative diagnosis is challenging but crucial for guiding treatment strategies.
- This case underscores the importance of considering rare tumors in the differential diagnosis of breast lesions.