Dermatofibrosarcoma of The Breast – Case Report and Review of Literature

Abstract

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.

Localization on the palmar face of the hand of a Darier-Ferrand dermatofiborosarcoma. About a case

“…It sends fine extensions sometimes very deep into the hypodermis, which would explain the occurrence of recurrence even with wide resection margins, the epidermis is respected, the cells are elongated, spindle-shaped, with more or less abundant cytoplasm, the oval nucleus, regular, mitoses are variable with rare atypia, the stroma is variable from one area to another (Hammas et al, 2014). In doubtful cases, Muhamed et al had published that immunohistochemistry makes it possible to distinguish DFSP from other spindle cell tumors, it shows an intense and diffuse positivity of CD34 in 90% of cases and negativity of actin, of desmin, and the S-100 protein (Muhammed et al, 2018).…”

Dermatofibrosarcoma Protuberans and Adjuvant Radiotherapy: A Case Report with Uncertain Surgical Margins in The Breast

“…CTV was created by giving a 3 cm margin to the tumor bed, and PTV was created by giving a 1 cm margin to the CTV. 19 Although the cases of DFSP in the breast are limited, the first priority issue is the removal of the tumor with wide local excision, as understood from the published cases. Although different information has been presented, surgical margins of 2-4 cm should be obtained.…”

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

“…Most cases of breast DFSP followed guidelines. A few cases indicated no local recurrence or metastasis with a surgical margin width of less than 2 cm [ 29 , 45 , 46 ]. Nevertheless, most of them reported a short follow-up period.…”