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Dermatofibrosarcoma protuberans in a child: a case report
Prashant Goyal1, Shelly Sehgal, Sompal Singh
1Department of Pathology, Swami Dayanand Hospital, Shahdara, New Delhi 110095, India.
Abstract:
Background. Dermatofibrosarcoma protuberans (DFSP) is an intermediate grade soft tissue neoplasm originating from the dermal layer of the skin. It usually occurs in adults; however, it can rarely be seen in infancy and childhood. Diagnosis of DFSP in children is quite difficult-given-rarity of this lesion, its variegated appearance, and its presentation sometimes at unusual sites. Case. We present the case of five-year-old boy who came with painless lesion on a forehead. Fine needle aspiration cytology (FNAC) suggested possibility of mesenchymal neoplasm. Patient was advised excision biopsy. Final diagnosis of DFSP was made based on histopathological findings. The patient was then advised reexcision surgery with wide margins. The patient was lost to followup and later turned up after two months with recurrence of a similar swelling at the same site. Conclusion. DFSP in children is rare and difficult to diagnose. Treatment of childhood DFSP is often delayed leading to incomplete excision. Hence, there is need to recognize and appropriately manage this uncommon childhood neoplasm.
Insights
Dermatofibrosarcoma protuberans (DFSP) is a rare skin neoplasm in children, often misdiagnosed. Early recognition and complete surgical excision are crucial for effective management and preventing recurrence in pediatric patients.
Area of Science:
- Pediatric Oncology
- Dermatopathology
- Surgical Oncology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is an intermediate-grade soft tissue neoplasm originating in the dermis.
- While typically occurring in adults, DFSP can rarely manifest in infants and children.
- Diagnosis in pediatric patients is challenging due to rarity, varied appearance, and potential for unusual presentations.
Purpose of the Study:
- To highlight the diagnostic challenges of pediatric Dermatofibrosarcoma protuberans.
- To emphasize the importance of timely and complete surgical management.
- To underscore the need for increased awareness of this rare childhood neoplasm.
Main Methods:
- Case presentation of a five-year-old boy with a forehead lesion.
- Initial diagnosis suggested by Fine Needle Aspiration Cytology (FNAC).
- Definitive diagnosis confirmed via histopathological examination and subsequent wide margin reexcision surgery.
Main Results:
- Histopathology confirmed Dermatofibrosarcoma protuberans (DFSP) in a pediatric patient.
- The patient experienced a recurrence after initial treatment due to lost follow-up and presumed incomplete excision.
- Delayed treatment and incomplete excision contributed to the unfavorable outcome.
Conclusions:
- Pediatric Dermatofibrosarcoma protuberans (DFSP) is rare and diagnostically difficult.
- Delayed treatment and incomplete excision are common issues in childhood DFSP.
- Prompt recognition and appropriate surgical management are essential for managing this uncommon pediatric neoplasm.
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