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Published on: May 18, 2018
Dermatofibrosarcoma protuberans occuring in a child
D Peschos1, P Dallas, A Doulis
1Dept. of Forensic Medicine, Medical School, University of Ioannina, Greece.
Abstract:
Dermatofibrosarcoma protuberans (DFSP) usually occurs in adults, however it can also occur in infancy and childhood. Diagnosis of DFSP in children is quite difficult given the rarity of the tumor and its variegated appearance. The behavior of this neoplasm is of borderline or intermediate malignancy with a high rate of recurrence but limited metastatic potential. We present the case of an eleven-year old boy who presented with a slightly raised, not tender lesion on his right shoulder. It had appeared two years before as a red-brown plaque and since then it had gradually grown to a nodule. An excisional biopsy was performed, and the diagnosis of DFSP was made based on the histological and immunohistochemical findings.
Insights
Dermatofibrosarcoma protuberans (DFSP) is rare in children, making diagnosis challenging. This case highlights the importance of considering DFSP in pediatric patients with persistent skin lesions.
Area of Science:
- Pediatric Oncology
- Dermatopathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a rare skin neoplasm.
- DFSP typically affects adults but can occur in pediatric patients.
- Diagnosis in children is challenging due to rarity and varied presentation.
Observation:
- An 11-year-old boy presented with a 2-year history of a gradually growing lesion on his right shoulder.
- The lesion initially appeared as a red-brown plaque and evolved into a nodule.
- The lesion was slightly raised and non-tender.
Findings:
- Histological and immunohistochemical examination confirmed the diagnosis of DFSP.
- DFSP is characterized as a borderline or intermediate malignancy.
- It has a high recurrence rate but low metastatic potential.
Implications:
- This case underscores the diagnostic difficulties of pediatric DFSP.
- Early recognition and accurate diagnosis are crucial for appropriate management.
- Understanding DFSP behavior in children is vital for long-term patient outcomes.
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