Dermatofibrosarcoma protuberans in the forearm

Clinical Cases 15.04.2013
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 20 years, female
Authors: Javier Fernandez-Jara, Rafael Félix Ocete Pérez2, Dimitry Hikin3, Faisal Shah3
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Details
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AI Report

Clinical History

20-year-old female patient with a history of soft tissue mass in the posterior forearm for one year showing no change on size. On examination it was not movable and there was overlying skin discoloration.

Imaging Findings

MRI shows a dermal nodular mass extending to the skin surface, measuring 1 x 1 cm. The lesion is isointense relative to muscle on T1WI and isointense/intermediate signal relative to muscle on fluid-sensitive sequences.
On post-contrast images the lesion demonstrates moderate enhancement.

Discussion

Superficial soft-tissue masses are common in clinical practice, and the expanding availability of the radiologic imaging has increased radiologists' familiarity with these entities [1].
Dermatofibrosarcoma protuberans is an uncommon spindle cell tumour, which typically arises in the dermis as a nodular mass, which then can spread into the subcutaneous tissue and muscle [2].
Dermatofibrosarcoma protuberans accounts for about 6% of all soft-tissue sarcomas [1, 3]. This lesion occurs between the third and the fifth decade of life. Lesions most commonly involve the trunk; up to half occur in this location. The extremities followed by the head and neck are the next most common sites, but tumours may occur on any part of the body [2, 3].
Patients often ignore these tumours because of their slow growth [2].
MR imaging is not routinely performed as part of the workup of patients with these neoplasms because of their characteristic appearance and superficial location [2, 3].
MR imaging is used to evaluate larger or atypical primary lesions or recurrent disease [3]. The lesions usually are manifested at MR imaging as unmineralised nodular masses with nonspecific signal intensity and moderate enhancement [1-3]. Areas of haemorrhage may be seen within the tumour [1].
Dermatofibrosarcoma protuberans has an excellent prognosis after complete resection but has a marked tendency to recur locally if inadequate surgical resection margins are obtained. Metastases are rare [2]. In our case the lesion was completely removed, histology was performed and established the diagnosis.
In conclusion, dermatofibrosarcoma protuberans is a rare, usually superficial soft-tissue sarcoma, which may be suspected on the basis of the tumour's clinical appearance. MR imaging helps to characterise atypical or difficult cases and may be important for pre-surgical planning.

Differential Diagnosis List

Dermatofibrosarcoma protuberans
Deep benign fibrous histiocytoma
Giant cell fibroblastoma
Dermatofibroma
Undifferentiated pleomorphic sarcoma
Neurofibroma

Final Diagnosis

Dermatofibrosarcoma protuberans

Liscense

Figures

Axial (A) and Sagittal (B) T1 FS post-contrast images

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Axial (A) and Sagittal (B) T1 FS post-contrast images

Microscopic findings

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Microscopic findings
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Microscopic findings

Axial T1WI (A) and T2WI (B)

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Axial T1WI (A) and T2WI (B)
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Axial T1WI (A) and T2WI (B)