Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma characterized by the proliferation of spindle cells arranged in a storiform pattern. Here we report a case of DFSP presenting as a bluish-black atrophic plaque. The tumor had the histopathologic characteristics of both the pigmented and atrophic variants of DFSP. We describe the histopathologic, molecular, and imaging features of this patient and review other cases of DFSP that have been reported in the literature. Early identification of this DFSP variant can help clinicians implement clinical management strategies to prevent recurrence.
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http://dx.doi.org/10.3389/fonc.2021.669754 | DOI Listing |
Cureus
December 2024
Pathology, Avalon University School of Medicine, Willemstad, CUW.
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally invasive cutaneous sarcoma with a high propensity for recurrence, even following complete surgical excision. DFSP exhibits a low metastatic potential and is characterized by a distinctive honeycomb-like architecture composed of uniformly arranged spindle cells that frequently show CD34 immunostaining. Common surgical approaches include wide local excision (WLE), Mohs micrographic surgery (MMS), and, in severe cases, amputation.
View Article and Find Full Text PDFCancers (Basel)
January 2025
Unit of Dermatology, Department of Medicine, University of Padova, 35122 Padua, Italy.
J Cancer Res Ther
December 2024
Department of Plastic and Reconstructive Surgery, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Background: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade fibrohistiocytic tumor with malignant potential. It is considered to have a high local recurrence rate due to the characteristic invasion of the finger-like lesion into the soft tissues.
Method: This retrospective study presents details of 20 DFSP patients with a history of surgery and a long follow-up period.
J Craniofac Surg
January 2025
Department of Pathology, Pathohistology and Medical Cytology, University Clinical Center of Serbia, Belgrade, Serbia.
Introduction: Sarcomas are relatively rare malignant tumors of mesenchymal origin, representing only about 1% of tumors in the head and neck region.
Materials And Methods: A retrospective study involved patients with sarcomas of the head and neck region who were diagnosed and treated over a 5-year period.
Results: Nine patients were included, 4 men and 5 women.
Diseases
November 2024
Department of Gynecology and Gynecologic Oncology, Medical University of Białystok, Skłodowskiej Str. 24A, 15-276 Białystok, Poland.
Background: Dermatofibrosarcoma protuberans (DFSP) is a rare proliferative condition representing skin sarcomas known to locally recur yet very rarely known to metastasize. A typical characteristic is trunk localization, making vulvar occurrences an exception. Complete resection is the primary treatment.
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