Dermatofibrosarcoma protuberans with fibrosarcomatous transformation: our experience, molecular evaluation of selected cases, and short literature review

Int J Dermatol. 2019 Nov;58(11):1246-1252. doi: 10.1111/ijd.14462. Epub 2019 Apr 10.

Abstract

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation (DFSP-FS) is a higher grade tumor arising from dermatofibrosarcoma protuberans (DFSP). Recent literature highlights its impact on recurrence rates, metastatic rates, and survival. In this article, we aim to describe our experience with 13 cases of DFSP-FS in terms of pathologic findings, molecular alterations, clinical outcomes, management, and also perform a short recent literature review.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Cell Transformation, Neoplastic / genetics*
  • Cell Transformation, Neoplastic / pathology
  • Chemotherapy, Adjuvant
  • Chromosomes, Human, Pair 22 / genetics
  • DNA Copy Number Variations
  • Dermatofibrosarcoma / genetics
  • Dermatofibrosarcoma / mortality
  • Dermatofibrosarcoma / therapy*
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Gene Rearrangement
  • Humans
  • Male
  • Middle Aged
  • Mohs Surgery
  • Neoplasm Recurrence, Local / epidemiology*
  • Neoplasm Recurrence, Local / pathology
  • Proto-Oncogene Proteins c-sis / genetics
  • Radiotherapy, Adjuvant
  • Registries / statistics & numerical data
  • Skin / pathology*
  • Skin Neoplasms / genetics
  • Skin Neoplasms / mortality
  • Skin Neoplasms / therapy*
  • Young Adult

Substances

  • Proto-Oncogene Proteins c-sis