Dermatofibrosarcoma protuberans (DFSP) is very rare low-grade neoplasm that arises from the dermal fibroblasts. DPFS has an incidence less than 1 per 100,000 people annually (1). Few authors succeeded to link this a translocation between chromosome 7 and 22 leading to up-regulation of derived growth factor subunit B (PDGFB) gene (2, 3). Although it is common to see local recurrence following excision, this tumor rarely metastasizes. Clinically, it appears as a subcutaneous nodule that grows slowly without any pain (2). Despite its indolent behavior with three years survival rate nearly 90%, DPFS usually resistant to chemotherapy and radiotherapy, thus, complete surgical resection is the gold standard aim of the treatment. The commonest site for DFSP are head, neck and extremities, however, few authors reported the affection of other sites such as the breast, other body parts. This publication discusses the management of a case of DFSP in the breast (2).
Case Presentation45 years old female presented to our department with a red-coloured subcutaneous right breast mass, 3 centimetres in maximal dimension and is located at the right lower quadrant of patient's breast. Her workup excluded any systemic metastasis (Figure 1; Sono-mammographic imaging of the tumor). At this stage, the provisional diagnosis was breast cancer with skin involvement. Biopsy from the nodule raised the suspicion of DFSP rather than breast cancer.So, the patient was informed about the nature of her disease, the risk of its recurrence and the possible complications of the treatment in addition to that a written consent was taken from her before further steps in the treatment process. She was subjected to wide resection with sentinel lymph node sampling which excluded any nodal involvement. Final pathological examination showed a tumour composed of malignant spindle cells (figure 2 and 3; spindle shaped cells are arranged in storiform pattern) that are CD 34 positive (Figure 4; diffuse expression of CD34) and CK, S100, ER, PR and CD 68 negative which suggest the diagnosis of low-grade dermatofibrosarcoma protuberans. The surgical resection margin was free of malignant cell involvement but less than 1.5 cm in width.Due to high risk of local recurrence, she was planned to receive adjuvant external beam radiotherapy at a dose of 60 Gy using threedimensional conformal radiotherapy, 3DCRT. She has CT simulation on breast board, supine position, in 5 mm thickness extending from the base of the skull to the level of the iliac crest. The organs at risk; both lungs, heart, right and left ventricles, liver, both kidneys and spinal cord were contoured.
