Lipofibromatosis-like neural tumors: Report of a case and review of 73 reported cases

Pediatr Dermatol. 2023 Jul-Aug;40(4):664-668. doi: 10.1111/pde.15218. Epub 2023 Jan 8.

Abstract

Lipofibromatosis-like neural tumors (LPF-NTs) are a recently discovered group of spindle cell tumors defined by the presence of a lipofibromatosis-like pattern, CD34 and/or S100 reactivity, and frequent neurotrophic receptor tyrosine kinase 1 (NTRK1) gene rearrangements. As new cases emerge, the spectrum of features observed in LPF-NTs continues to evolve. Here we describe the case of an 11-year-old with LPF-NT with a dermatofibrosarcoma protuberans-like honeycomb pattern, CD34 and S100 co-expression, and an NTRK1 rearrangement. We also review the clinical and molecular features of the 73 cases of LPF-NT previously described in the literature.

Keywords: connective and soft tissue; dermatology; neoplasms; pathology; review; surgical oncology..

Publication types

  • Case Reports

MeSH terms

  • Biomarkers, Tumor / genetics
  • Child
  • Fibroma* / diagnosis
  • Fibroma* / genetics
  • Fibroma* / surgery
  • Humans
  • Skin Neoplasms* / diagnosis
  • Skin Neoplasms* / genetics
  • Skin Neoplasms* / surgery
  • Soft Tissue Neoplasms* / pathology

Substances

  • Biomarkers, Tumor