Dermatofibrosarcoma protuberans: the diagnosis of high-grade fibrosarcomatous transformation

Skeletal Radiol. 2021 Apr;50(4):789-799. doi: 10.1007/s00256-020-03617-3. Epub 2020 Oct 1.

Abstract

Objectives: Dermatofibrosarcoma protuberans (DFSP) is an intermediate-grade tumour which may undergo fibrosarcomatous transformation to a high-grade sarcoma (DFSP-FST). DFSP-FST requires wide local resection, and therefore, pre-operative identification is important. The aims of this study are to see if DFSP and DFSP-FST can be differentiated based on MRI appearances, and to determine the ability of ultrasound-guided core needle biopsy (US-CNB) to identify DFSP-FST.

Materials and methods: Retrospective review of patients with a histological diagnosis of DFSP with/without transformation to DFSP-FST. Patient age, gender, lesion location and maximal size were recorded, as were several MRI features. MRI studies were reviewed independently by 2 musculoskeletal radiologists and the assessed features were then compared with final surgical resection histology. Histological results of US-CNB were also compared with final surgical pathology.

Results: A total of 42 patients were included, 26 males and 16 females with a mean age of 41.3 years (range 3-78 years). The upper limb was involved in 12 cases, the lower limb in 17 and the trunk in 13. Final surgical histological diagnosis was DFSP in 21 (50%) cases and DFSP-FST in 21 (50%) cases. Mean tumour dimension for DFSP was 32 mm and DFSP-FST 68 mm (p < 0.001). MRI features indicative of DFSP-FST included multi-lobular morphology (p = 0.03), T2W hypointensity compared with fat (p = 0.03), internal flow voids (p = 0.03) and peri-tumoral oedema (p < 0.001). Only 3 cases of DFSP-FST were correctly diagnosed on US-CNB.

Conclusions: Various MRI findings can suggest a diagnosis of DFSP-FST, but US-CNB is unreliable at identifying high-grade fibrosarcomatous transformation.

Keywords: Dermatofibrosarcoma protuberans; Fibrosarcoma; MRI; Needle biopsy.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Child, Preschool
  • Dermatofibrosarcoma* / diagnostic imaging
  • Female
  • Fibrosarcoma*
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Skin Neoplasms* / diagnostic imaging
  • Ultrasonography
  • Young Adult