Dermatofibrosarcoma Protuberans – A rare type of sarcoma.

This poster is published under an open license. Please read the disclaimer for further details.

Congress:

ECR 2016

Poster Number:

C-0365

Type:

Educational Exhibit

Keywords:

Cancer, Surgery, Staging, Education, Ultrasound, MR, Catheter arteriography, Soft tissues / Skin

Authors:

D. De Witte¹, C. S. Nabarro², D. Vroegindeweij³, J. J. Hensen³; ¹Rotterdam, Nederland/NL, ²Den Haag/NL, ³Rotterdam/NL

DOI:

10.1594/ecr2016/C-0365

DOI-Link:

https://dx.doi.org/10.1594/ecr2016/C-0365

Fig. 1: Ultrasound of DFSP

Fig. 2: MRI of abdominal DFSP

Fig. 3: MRI of DFSP on the right shoulder

Fig. 4: Digital Substraction Angiography (DSA) of DFSP on the right shoulder

Learning objectives

To describe imaging manifestations of dermatofibrosarcoma protuberans (DFSP).

Background

DFSP is a rare (3:1.000.000) and locally aggressive (sub)cutaneous soft tissue sarcoma. It most often arises in adults in the age between 20 to 50 years, (M=F), but it can be seen amongst all ages including children and elderly. Approximately 85-90% of DFSP are low grade, the remainder contains a high grade sarcomatous component (usually a fibrosarcoma, called DFSP-FS). It usually presents as an asymptomatic, reddish plaque that enlarges over time (months to years). When progressive, it becomes raised, firm and nodular and the surrounding...

Findings and procedure details

Thetumor orginates in the cutaneous tissues, “splits” the skin and proliferates in the underlying tissues. Ultrasound shows a hypoechoic (sub)cutaneous mass with internal reflections. A hyperechoic rim surrounds the mass.Color Doppler revealedflow in the lesion indicating hypervascularity (figure 1). MRI characteristics are alow signal intensity on T1-weighted images, a high signal intensity (but lower than fat) on T2-weighted images, no fat suppression and avid enhancement after IV gadolineum administration (figures 2 and 3). In one case atight relation to the muscle seen, however no invasion...

Conclusion

DFSP is a rare soft tissue sarcoma which can be locally aggressive. Clinical diagnosis can be difficicult with a broad differential diagnosis. Inimaging, MRI plays an important role in stratifying the differential diagnosis and is especially essential in determining the extend of tumor in muscle, fascia or bone. Whenmetastasized disease is suspected, (PET) CT scan is the modality of choice. Surveillance is strongly recommended after radical resection.

Personal information

D. de Witte, MD Radiology resident Maasstad Hospital Rotterdam The Netherlands

References

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