A Rare Breast Tumor: Dermatofibrosarcoma Protuberans

Abstract

Dermatofibrosarcoma protuberans is a slow-growing, local aggressive fibrous tumor of the subcutaneous tissue, frequently seen in the proximal extremities and the trunk. Its occurrence in the breast is very rare. Herein, we present a female who presented with a breast mass, and aim to discuss pathological features and differential diagnosis of dermatofibrosarcoma protuberans. A 44-year-old female presented to our clinic with a mass on her breast. Physical examination revealed a 8x5.5 cm mass with multilobular nodules on the skin in the lower inner quadrant of her right breast. Her mammography revealed a hyperdense, 7.5x6.5 cm, well-demarcated, lobulated mass in the right breast, which caused nodules on the lower paraareolar portion of the breast skin. There was no axillary lymphadenopathy on both clinical and radiologic examinations. A core needle biopsy had been performed prior to her referral to our center, which revealed a 'spindle cell lesion'. The patient underwent simple mastectomy. On macroscopic examination; the skin over the lesion appeared ulcerated, and there was a well-defined solid mass, which was pale white-tan on the cut surface. Microscopic examination revealed monotonous spindle cell proliferation arranged in storiform pattern within the collagenous stroma with irregular extensions into deep adipose tissue. There were no necrosis or nuclear pleomorphism. The mitotic rate was 2-3/10 HPF. Immunohistochemically tumor cells showed diffuse CD34 positivity, and S100, EMA and SMA negativity. Based on histopathological and immunohistochemical findings, the lesion was diagnosed as dermatofibrosarcoma protuberans. Local recurrence is expected in 20-50% of these cases. Its treatment requires complete surgical excision with wide margins. Distant metastases, although rare, have been reported.

Diagnostic Challenges of Dermatofibrosarcoma Protuberans (DFSP), a Rare Spindle Cell Tumor of Breast

“…Sometimes histological variants amount to some confusion in diagnosis. The histological variants can be pigmented; with focal myofibroblastic areas; myxoid, with sclerosing nodules; atrophic, or granular cell, fibrosarcoma-like changes; or giant cell fibro blastoma-like areas [ 8 - 12 ]. Differential diagnosis includes fibroadenomas and phyllodes tumors as the most common, followed by pseudo angiomatous stromal hyperplasia, hamartoma, adenomyoepithelioma, metaplastic carcinoma, and fibromatosis [ 7 , 9 , 10 ].…”

Dermatofibrosarcoma Protuberans Mimicking Primary Breast Neoplasm: A case report and literature review

“…Dermatofibroma is a tumour of the dermis, whereas DFSP frequently infiltrates the subcutaneous adipose tissue. 13 Bednar tumour is the pigmented variant of DFSP and has similar morphologic properties as DFSP. However, Bednar tumour has melanin containing dendritic cells.…”

“…However, Bednar tumour has melanin containing dendritic cells. 13 Another differential diagnosis is malignant fibrous histiocytoma (MFH). Marked nuclear pleomorphism, increased mitotic activity and necrosis are detected in MFH and tumour cells are negative for CD34.…”

Clinical and imaging features of dermatofibrosarcoma protuberans in the breast

“…Mohs micrographic surgery has become an alternative to wide local excision that allows to remove the entire tumor while conserving healthy tissue 2 . Recurrence rate has been described in up to 50% of cases and is most common for tumors of the head and neck, likely because it is difficult to achieve wide margins 9 . For the treatment of inoperable primary tumors or metastatic disease, systemic therapy with Imatinib mesylate can be utilized 10 …”