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Dermatofibrosarcoma protuberans: the diagnosis of high-grade fibrosarcomatous transformation

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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.

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Acknowledgments

The authors would like to acknowledge Mr. Paul Bassett for statistical input.

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Authors and Affiliations

  1. Spinal Cord Injuries Unit, Royal National Orthopaedic Hospital, Stanmore, UK

    Paul Choong

  2. Department of Histopathology, Royal National Orthopaedic Hospital, Stanmore, UK

    Daniel Lindsay

  3. Department of Radiology, Royal National Orthopaedic Hospital, Stanmore, UK

    Michael Khoo & Asif Saifuddin

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  1. Paul Choong
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  2. Daniel Lindsay
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Correspondence to Michael Khoo.

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Choong, P., Lindsay, D., Khoo, M. et al. Dermatofibrosarcoma protuberans: the diagnosis of high-grade fibrosarcomatous transformation. Skeletal Radiol 50, 789–799 (2021). https://doi.org/10.1007/s00256-020-03617-3

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  • DOI: https://doi.org/10.1007/s00256-020-03617-3

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