Dermatofibrosarcoma Protuberans: A comprehensive review on the spectrum of clinico‐radiological presentations

Abstract

Dermatofibrosarcoma Protuberans (DFSP) is a rare malignant soft-tissue neoplasm which is often misdiagnosed due to its indolent clinical course and non-specific radiological appearances. An observation case series was conducted with retrospective review of clinical and radiological data of DFSP patients presenting to a major tertiary hospital in Hong Kong for radiological assessment between November 2006 and February 2016. Seven patients with confirmed histological diagnosis of DFSP were included. Tumour sizes at presentation ranged from 1 to 5 cm, most commonly (n = 6) occurred over chest wall and abdominal wall. History of previous local trauma or surgery was identified in the majority of cases (n = 4). There was poor correlation between pre-imaging clinical diagnoses and pathological diagnoses. Local recurrence and tumour de-differentiation with sarcomatous changes occurred in the minority of cases (n = 2). A common radiological 'claw' sign at the lesion/skin interface formed by elongated appendages of the tumour superficially was appreciated in most cases (n = 6). A history of previous local trauma or surgery serves as a possible etiological factor for the development of DFSP. High clinical suspicion for the entity is essential in its detection and differentiation from simple wound complications and local recurrence of other benign lesions. The radiological 'claw' sign at the lesion/skin interface might serve as a tell-tale sign for cutaneous tumour involvement. A comprehensive analysis of imaging findings in conjunction with individual clinical presentations is the key to accurate diagnoses and proper management.

Role of Ultrasound In The Diagnosis of Primary And Recurrent Dermatofibrosarcoma Protuberans

“…2, 3). This is in agreement with Sung et al's results [23]. They observed a characteristic radiological "claw" sign featuring the interface between the lesion and the skin in various imaging modalities, including US, CT, and MRI.…”

“…Surgical excision of the lesion is the standard treatment for DFSP. Some reports [23] have noted that the recurrence rate after local excision was signi cantly higher than that after wide-margin excision (margins over 3 cm), while the recurrence rate after wide-margin excision was signi cantly higher than that after Mohs surgery. Preoperative US imaging may assist in surgical planning and help to prevent local recurrence.…”

Usefulness of ultrasound in dermatofibrosarcoma protuberans and correlation with histopathological findings: A series of 30 cases

“…This is the largest series to date to describe the ultrasound The ultrasound findings for this series of 30 patents are consistent with those described to date. [9][10][11][12][14][15][16] In a study of nine patients with DFSP published in 2013, Lee et al 13 reported round or ovoid tumors with a hypoechoic matrix and small echogenic foci. Although oval lesions, on occasions with lobulated margins, seem to be a consistent ultrasound finding, they are not specific to DFSP, as similar morphologies have been described for other skin lesions such as neurogenic and myxoid tumors, epidermal inclusion cysts, and nodular fascitis.…”

“…1 Our ultrasound analysis showed that 20 of the 30 tumors analyzed had finger-like projections extending into the subcutaneous tissue. These projections, which have also been described as a claw sign, 15 a jellyfish-like image, or tentacle-like projections, 16 are a well-recognized ultrasound finding in DFSP, and some authors have even suggested that they should be considered highly suggestive or even pathognomonic for DFSP. 15,16 Correlations have been previously reported between finger-like projections on ultrasound and radial or vertical wedge-shaped extensions of DFSP along the septa on histopathology.…”

“…Subsequent reports have confirmed these findings and reported occasional variants. [10][11][12][13][14][15][16] No large series to date, however, have analyzed the ultrasound features of DFSP. In addition, evidence is lacking on the usefulness of ultrasound as a preoperative planning tool for tumor resection or its accuracy for determining depth of invasion and identifying the deepest level of penetration.…”