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Dermatofibrosarcoma protuberans in childhood

M L Bouyssou-Gauthier1, F Labrousse, B Longis

  • 1Department of Dermatology, Centre Hospitalier Régional et Universitaire Dupuytren, Limoges,France.

Pediatric Dermatology
|January 22, 1998
PubMed
Summary
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Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor that can present atypically in children. Early diagnosis is challenging, as illustrated by this case of a pediatric patient initially misdiagnosed.

Area of Science:

  • Dermatology
  • Pediatric Oncology
  • Surgical Pathology

Background:

  • Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing malignant neoplasm of the skin.
  • DFSP typically affects adults, with occurrence in childhood being exceptionally rare.
  • The clinical presentation of DFSP can be variable and may mimic benign dermatological conditions.

Observation:

  • An 18-month-old child presented with an acquired atrophic plaque on the left ankle.
  • Initial clinical diagnoses included localized scleroderma and lipoatrophy.
  • By age 5, the lesion enlarged, developed a purplish hue, and exhibited peripheral firm nodules.

Findings:

  • Histological confirmation established the diagnosis of Dermatofibrosarcoma protuberans (DFSP).
  • The pediatric case highlights the diagnostic challenges of DFSP in early life.

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  • Surgical resection was necessary to manage the tumor.

    • Implications:

    • This case underscores the importance of considering rare diagnoses like DFSP in pediatric patients with persistent or evolving skin lesions.
    • Delayed diagnosis of DFSP in children can lead to more extensive disease and complex treatment.
    • Increased awareness and vigilance are crucial for timely diagnosis and management of pediatric DFSP.