Pleomorphic dermal sarcoma presenting in a child with Li-Fraumeni syndrome: A case report and review of the literature

Pediatr Dermatol. 2024 Mar-Apr;41(2):311-314. doi: 10.1111/pde.15452. Epub 2023 Nov 28.

Abstract

Pleomorphic dermal sarcoma (PDS) is an uncommon malignant soft-tissue tumor that occurs mostly in elderly patients, with only 5% of cases occurring in children. However, pediatric patients with Li-Fraumeni syndrome (LFS) can develop several types of cancer, particularly sarcomas. Here, we describe a young LFS patient who presented with early-onset PDS and review the literature.

Keywords: Li-Fraumeni syndrome (LFS); pediatrics; sarcoma.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Aged
  • Child
  • Genetic Predisposition to Disease
  • Histiocytoma, Malignant Fibrous*
  • Humans
  • Li-Fraumeni Syndrome* / complications
  • Li-Fraumeni Syndrome* / diagnosis
  • Li-Fraumeni Syndrome* / genetics
  • Sarcoma* / complications
  • Sarcoma* / diagnosis
  • Skin Neoplasms* / complications
  • Skin Neoplasms* / diagnosis
  • Soft Tissue Neoplasms*