RESUMO
INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare neoplasm that exceptionally can affect the breast, always originating from skin and dermis, so imposing large sacrifice of skin. Only few cases have been reported of intraparenchymal DFS. We describe a unique case of giant intraparenchymal DFS that required removal of all the gland and reconstructive surgery. CASE REPORT: A 34 years old woman presents with a quickly growing breast mass, about 12 cm, with radiological features suggestive for giant fibroadenoma or mesenchymal neoplasm. The histology showed a Dermatofibrosarcoma protuberans infiltrating the surrounding parenchyma. The need for radicalization imposed, due to the size of the tumour, a nipple sparing mastectomy. The reconstruction has been performed using a new type of Acellular Dermal Matrix (ADM) mesh to wrap the prosthesis that has been placed and fixed over the great pectoral muscle. RESULTS: The final histologic report showed that the residual parenchyma and the skin removed were free from neoplastic infiltration. The patient is free from recurrence at 24 months from the surgery and the cosmetic result is excellent. DISCUSSION AND CONCLUSIONS: The treatment of DFSP should be aimed to prevent local recurrence, that are usually located in the scar or very close to it. Large size DFS can impose even mastectomy. If skin is not compromised like in this case, a nipple sparing mastectomy is suitable and the one time reconstruction with ADM wrapping of the prosthesis and fixation over the muscle can help to spare time, avoid complications and pain medication and reach excellent cosmetic resu. KEY WORDS: Acellular Dermal Matrix (ADM) mesh, Protuberans, Breast neoplasms, Dermatofibrosarcoma, Mastectomy, Nipple sparing, reconstructive surgery.
