Dermatofibrosarcoma protuberans in childhood: two case reports and review of the literature.

Pediatr Hematol Oncol

Plastic Surgery Unit-Regional Melanoma Referral Center, Tuscan Tumor Institute (ITT), Santa Maria Annunziata Hospital, Florence, Italy.

Published: September 2008

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.

Download full-text PDF

Source
http://dx.doi.org/10.1080/08880010802235066DOI Listing

Publication Analysis

Top Keywords

dermatofibrosarcoma protuberans
8
dfsp
5
protuberans childhood
4
childhood case
4
case reports
4
reports review
4
review literature
4
literature dermatofibrosarcoma
4
protuberans dfsp
4
dfsp rare
4

Similar Publications

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 PDF
Article Synopsis
  • Dermatofibrosarcoma protuberans (DFSP) is a rare type of low-grade cancer that can be difficult to diagnose.
  • It often looks similar to benign skin lesions, which can lead to confusion in diagnosis.
  • Prompt recognition and differentiation from other skin conditions are important for effective treatment.
View Article and Find Full Text PDF

Dermatofibrosarcoma protuberans: A clinical and pathological observational report of Asian samples from one center.

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.

View Article and Find Full Text PDF

Sarcomas of the Head and Neck Region.

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.

View Article and Find Full Text PDF

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.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!