Literature DB >> 33001221

Dermatofibrosarcoma protuberans: the diagnosis of high-grade fibrosarcomatous transformation.

Paul Choong1, Daniel Lindsay2, Michael Khoo3, Asif Saifuddin4.   

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.

Entities:  

Keywords:  Dermatofibrosarcoma protuberans; Fibrosarcoma; MRI; Needle biopsy

Mesh:

Year:  2020        PMID: 33001221     DOI: 10.1007/s00256-020-03617-3

Source DB:  PubMed          Journal:  Skeletal Radiol        ISSN: 0364-2348            Impact factor:   2.199


  1 in total

1.  The clinical application of lateral chest flap transfer combined with relay skin flap transfer to repair soft tissue defects of the chest wall after tumor resection.

Authors:  Fangfang Guo; Lu Wang; Lei Wang; Xinyang Yu; Zexin Chen
Journal:  J Thorac Dis       Date:  2022-09       Impact factor: 3.005

  1 in total

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