Fact-sheet: Dermatofibrosarcoma protuberans
Updated on 09/27/2024 at 9:34 AM
View all RADEOS cases associated with this fact-sheet
Definition
Dermatofibrosarcoma protuberans is a rare infiltrative soft tissue sarcoma of dermal origin, likely fibroblastic, associated with a specific chromosomal translocation t(17;22).
It is a tumor that is essentially locally malignant, low-grade in 85-95% of cases, suggested on pathology by a proliferation of CD34+ spindle cells with little or no cytologic atypia. Dedifferentiation into high-grade fibrosarcoma is seen in 5-15% of cases.
Clinical features
Prevalence: 1/10,000
Occurs at any age, with a predominance between 20 and 50 years.
Indurated pink or violaceous-red fibrous plaques, or dermal nodules of the head, neck, trunk, or proximal extremities.
Tendency for local recurrence after surgery. Metastases are rare.
MRI
Loco-regional staging
Infiltrative dermal or dermo-hypodermal lesion, hyperintense on T2, hypointense on T1, with marked enhancement after injection.
Specify contact with or extension to the underlying fascia, muscles, bone, and adjacent vascular-nervous structures.
Management
Netsarc+ MDT meeting
Surgical treatment at a sarcoma reference center, either by the slow-Mohs technique or with lateral margins ideally of 1 cm and deep excision of the aponeurotic plane.
Locally advanced or unresectable forms: Imatinib