Dermatofibrosarcoma protuberans

Case contributed by Dr Brian Gilcrease-Garcia

Presentation

Left chest mass, slowly enlarging over 2-3 years. On physical exam, there is an indurated hyperpigmented mass at left chest/abdomen wall

Patient Data

Age: 30 years
Gender: Female
Ultrasound

At the right inframammary fold, there is a 2.9 x 2.1 x 2.8 cm heterogeneously hyperechoic solid mass centered in subcutaneous tissue, with overlying hypoechoic region in the dermis. There is increased color Doppler flow, with arterial waveform on spectral Doppler evaluation.

Differential diagnosis includes neoplasm and inflammatory mass.

CT

Two year follow-up

At the left anterior thoracoabdominal wall there is a 3 cm heterogenous soft tissue mass with lobulated margins, which corresponds to the lesion previously evaluated by ultrasound. The mass is centered within the subcutaneous tissue, although there is also thickening of the overlying dermal tissue.

Separately, a left upper breast nodule is indeterminate, although likely unrelated. No suspicious nodes or pulmonary nodule.

Patient eventually underwent core needle biopsy:

Surgical pathology

Microscopic

Sections of the biopsy material show a hypercellular spindle cell lesion growing in short fascicles with a predominant storiform pattern that appears to be infiltrating through subcutaneous fat. The cells appear cytologically bland and no mitoses or necrosis is observed. A panel of immunohistochemical stains (single antibody procedure with appropriate controls) were performed to better characterize lesion. They show the tumor cells to be positive for CD34 and negative for pancytokeratin (AE1/AE3) and desmin. There is no nuclear staining for beta-catenin. An S-100 stain highlights rare scattered positive cells. In aggregate, the morphologic and immunophenotypic profile is most consistent with a dermatofibrosarcoma protuberans (DFSP).

Impression

Soft tissue, left chest wall, core needle biopsy
- Dermatofibrosarcoma protuberans (DFSP)

Case Discussion

Dermatofibrosarcoma protuberans, commonly referred to as DFSP, are histologically-benign skin sarcomas which nonetheless exhibit malignant behavior manifesting as local invasion and recurrence.

A rare entity to begin with, they are perhaps even more rarely seen by radiologists, as they are not typically imaged. Apart from the imaging, this case exemplifies a typical presentation of DFSP: an indolent dermal/subdermal mass occurring along the trunk.

Due to a relatively high rate of local recurrence, management critically depends on resection with clean margins. This patient was treated by Mohs procedure, which was followed by deeper resection due to a positive deep margin.

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Case information

rID: 64660
Published: 3rd Dec 2018
Last edited: 14th Aug 2019
Tag: sarcoma
Inclusion in quiz mode: Included
Institution: Mallinckrodt Institute of Radiology at Washington University

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