Dermatofibrosarcoma protuberans (DFSP) is a rare skin cancer with intermediate malignancy, characterized by a progressive local growth and a propensity for local recurrence. DFSP is most frequent in adults; however, in recent years, DFSP in childhood emerged to be more common than previously believed. Unfortunately DFSP in children may be misdiagnosed, leading to a delay in the treatment. The authors report two cases of childhood DFSP with unusual clinical presentation: a congenital nodular variant and an atrophic variant developed at 2 years of age, both with acral localization. They highlight the importance of an early diagnosis by pediatricians and dermatologists to ensure an appropriate complete excision and reduce the risks of recurrences.
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http://dx.doi.org/10.1080/08880010802235066 | 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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