Dermatofibrosarcoma protuberans: the diagnosis of high-grade fibrosarcomatous transformation
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SCIENTIFIC ARTICLE
Dermatofibrosarcoma protuberans: the diagnosis of high-grade fibrosarcomatous transformation Paul Choong 1 & Daniel Lindsay 2 & Michael Khoo 3 & Asif Saifuddin 3 Received: 19 July 2020 / Revised: 15 September 2020 / Accepted: 16 September 2020 # ISS 2020
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
Introduction Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous fibrohistiocytic tumour which accounts for ~ 1% of soft tissue sarcomas, having an annual incidence of 0.8–5 cases per million population per year [1, 2]. However, in a review of 12,370 malignant soft tissue tumours referred to The Armed Forces
* Michael Khoo [email protected] 1
Spinal Cord Injuries Unit, Royal National Orthopaedic Hospital, Stanmore, UK
2
Department of Histopathology, Royal National Orthopaedic Hospital, Stanmore, UK
3
Department of Radiology, Royal National Orthopaedic Hospital, Stanmore, UK
Institute of Pathology (Washington DC, USA) over a 10-year period, DFSP accounted for 6.2% of cases [3]. Patients usually present between the third to sixth decades, but cases of congenital and paediatric DFSP have also been reported [4, 5]. The trunk is involved in 40–50% of cases and the arms and thighs in 30–40% [1, 2]. The tumour is associated with a good prognosis, the 5-
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