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Darier-Ferrand Dermatofibrosarcoma Protuberans: A Rare Soft Tissue Tumor Of The Breast And A Review Of The Literature

Introduction

Darier-ferrand dermatofibrosarcoma protuberans: a rare soft tissue tumor of the breast and a review of the literature. Darier-Ferrand Dermatofibrosarcoma Protuberans (DFSP) is a rare soft tissue tumor of the breast. This report details a case in a 38-year-old female and reviews literature.

Abstract

In cases where a mass in the breast is diagnosed and a tru-cut or excisional biopsy is performed, mesenchymal lesions are rarely encountered. Dermatofibrosarcoma protuberans is a rare mesenchymal tumour originating from the dermis in clinical practice. It is generally a low-grade sarcoma and rarely metastasizes. Other malignant mesenchymal lesions observed in the breast include leiomyosarcoma, liposarcoma, chondrosarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma, spindle cell mesenchymal tumour, and angiosarcoma. Primary mesenchymal tumours of the breast make up less than 1% of all breast malignancies. In this report, a 38-year-old female patient with a painless mass measuring approximately 7x6 cm, protruding from the upper inner quadrant of the right breast, was presented. The tru-cut biopsy performed on the lesion revealed a spindle cell mesenchymal tumour. A PET-CT scan showed a mass with increased SUV-max in the right breast and reactive lymph nodes in the right axilla. No distant metastasis was observed. The patient underwent a wide local excision with a 1 cm clear surgical margin. She was discharged on the 2nd postoperative day without complications. The pathology result of the main specimen was dermatofibrosarcoma protuberans, with negative surgical margins, and the patient was placed under clinical and radiological follow-up.

Review

This paper presents a highly relevant case report of Darier-Ferrand Dermatofibrosarcoma Protuberans (DFSP) originating in the breast, an anatomical location where such mesenchymal tumors are exceedingly rare. The abstract effectively highlights the infrequent nature of mesenchymal lesions in breast biopsies, emphasizing that primary mesenchymal tumors account for less than 1% of all breast malignancies. Given the general rarity of DFSP itself, documenting this specific presentation is crucial for expanding the current diagnostic and clinical understanding of unusual breast pathologies, making this report a valuable contribution to the medical literature. The case presentation is clear and concise, detailing the clinical scenario of a 38-year-old female with a painless breast mass and the initial tru-cut biopsy findings of a spindle cell mesenchymal tumor. The subsequent diagnostic pathway, including a PET-CT scan to stage the disease and rule out metastasis, reflects a comprehensive approach to an atypical breast lesion. The successful management through wide local excision with clear surgical margins, followed by an uncomplicated postoperative course and ongoing clinical and radiological follow-up, provides practical insights into the definitive treatment of this low-grade sarcoma in an unusual site. The ultimate pathological confirmation of DFSP adds significant educational value for clinicians and pathologists. While the abstract provides a strong summary of the case, a fuller discussion in the complete paper on the specific immunohistochemical markers crucial for confirming the DFSP diagnosis and distinguishing it from other spindle cell lesions of the breast would be beneficial. Despite this, the report significantly enriches the body of knowledge regarding rare breast tumors, serving as an important reminder for healthcare professionals to consider DFSP in the differential diagnosis of breast masses, especially when initial findings suggest a mesenchymal lesion. This case report, coupled with the implied literature review, strongly supports publication to enhance awareness and guide future diagnostic and therapeutic strategies for this rare and unusual presentation.

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