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Dermatofibrosarcoma protuberans presenting in infancy and childhood.
1Department of Histopathology, St. Thomas's Hospital (UMDS), London, England.
Journal of Cutaneous Pathology
|August 1, 1991
Summary
Dermatofibrosarcoma protuberans (DFSP) is rare but occurs in children. This study highlights eight pediatric cases, emphasizing the need for increased awareness and accurate diagnosis in young patients.
Area of Science:
- Oncology
- Dermatology
- Pediatric Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a low-grade cutaneous sarcoma typically affecting adults.
- DFSP is traditionally considered rare in infants and children, potentially leading to diagnostic challenges in this age group.
Purpose of the Study:
- To investigate the occurrence and characteristics of dermatofibrosarcoma protuberans in pediatric patients.
- To raise awareness of DFSP in childhood to ensure timely and appropriate management.
Main Methods:
- Retrospective review of eight pediatric cases diagnosed with DFSP.
- Analysis of patient demographics, tumor location, clinical presentation, and histopathological features.
- Short-term follow-up data collection.
Main Results:
- Eight pediatric DFSP cases were identified, representing 5.9% of the institution's DFSP cases.
- Patients ranged from 14 months to 12 years old, with two congenital cases.
- Tumors predominantly arose on the trunk and often presented as plaque-like lesions, differing from the typical nodular pattern in adults.
- Initial misdiagnoses included unclassified sarcoma, fibromatosis, and diffuse neurofibroma.
Conclusions:
- Dermatofibrosarcoma protuberans is not as rare in childhood as traditionally believed.
- Pediatric DFSP may present with distinct histological features, such as a plaque-like growth pattern.
- Increased recognition of pediatric DFSP is crucial for accurate diagnosis and effective treatment planning.