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<p>Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue sarcoma arising from dermal fibroblasts. Breast involvement is extremely rare, posing diagnostic and therapeutic challenges.&#xa0;We retrospectively reviewed eight cases of breast DFSP managed at our center over a 10-year period.&#xa0;The cohort included seven females and one male with a median age of 39.1 years. Clinical presentations were diverse: one patient had multiple areolar nodules, five presented with breast lumps including three with recurrent lumps, which is an uncommon presentation for DFSP, one had an ulceroproliferative growth with bleeding, and one presented following excision of a recurrent lump elsewhere with histopathology showing positive margins. Histological diagnosis was confirmed via core biopsy or block review. Four patients had classic DFSP, while three had fibrosarcomatous transformation. All cases of DFSP showed CD34 positivity. Wide local excision was performed in seven patients, and one underwent mastectomy. Two patients received adjuvant radiotherapy. No recurrence was observed within six months.&#xa0;Breast DFSP is rare and requires histopathological confirmation. Core biopsy with appropriate histologic features and CD34 immunostaining aids in diagnosis. Wide local excision remains the primary treatment approach. Radiotherapy is recommended in cases with close or positive margins, recurrence, metastatic disease, or when surgery is not feasible. Targeted therapy with Imatinib Mesylate, a tyrosine kinase inhibitor, is reserved for unresectable or metastatic lesions, provided the COL1A1::PDGFB translocation involving chromosomes 17 and 22 [t (17; 22)] is confirmed. Long term stringent follow up is required as recurrence in breast has been noted 26 years following primary treatment in literature.</p>

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Dermatofibroasarcoma Protuberans (DFSP) of the Breast: An Eight Case Series From A Single Institution

  • 期刊论文

  • 发表日期：

    2026年1月7日

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  • Shreya Surendra,
  • Shalini Sahu,
  • Santhosh Raj,
  • Nirmal Daniel,
  • Supriya Sen,
  • Thomas Shawn Sam,
  • Meskerem Abera,
  • Rajesh Balakrishnan,
  • Anish Jacob Cherian,
  • Mazhunvanchary Jacob Paul

摘要

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue sarcoma arising from dermal fibroblasts. Breast involvement is extremely rare, posing diagnostic and therapeutic challenges. We retrospectively reviewed eight cases of breast DFSP managed at our center over a 10-year period. The cohort included seven females and one male with a median age of 39.1 years. Clinical presentations were diverse: one patient had multiple areolar nodules, five presented with breast lumps including three with recurrent lumps, which is an uncommon presentation for DFSP, one had an ulceroproliferative growth with bleeding, and one presented following excision of a recurrent lump elsewhere with histopathology showing positive margins. Histological diagnosis was confirmed via core biopsy or block review. Four patients had classic DFSP, while three had fibrosarcomatous transformation. All cases of DFSP showed CD34 positivity. Wide local excision was performed in seven patients, and one underwent mastectomy. Two patients received adjuvant radiotherapy. No recurrence was observed within six months. Breast DFSP is rare and requires histopathological confirmation. Core biopsy with appropriate histologic features and CD34 immunostaining aids in diagnosis. Wide local excision remains the primary treatment approach. Radiotherapy is recommended in cases with close or positive margins, recurrence, metastatic disease, or when surgery is not feasible. Targeted therapy with Imatinib Mesylate, a tyrosine kinase inhibitor, is reserved for unresectable or metastatic lesions, provided the COL1A1::PDGFB translocation involving chromosomes 17 and 22 [t (17; 22)] is confirmed. Long term stringent follow up is required as recurrence in breast has been noted 26 years following primary treatment in literature.