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Darier-Ferrand dermatofibrosarcoma: three pediatric cases

S. El Batti*, N. Cardot-Leccia***, J. Bréaud* , JP. Lacour**, F. Pedeutour****, JY. Kurzenne

*Pediatric Unit*, ** Department of Dermatology, ***Anatomopathology, **** Cytogenetics - CHU Nice, France

 

ABSTRACT

Aim: To present 3 cases (3 boys) of Darier-Ferrand dermatofibrosarcoma (DFS)

Material and methods: First case: 7 years old boy, DFS located in the left areolar area. It was performed biopsy-excision and secondary histogenetic diagnosis. Secondary excision (nipple) was necessary and a cutaneous graft was applied. Second case: 10 years old boy, DFS located in the genito-crural area. It was performed biopsy- excision and secondary histogenetic diagnosis. The excision was recommended and the cutaneous graft applied. Third case: 5 years old boy, left calf DFS. Minimal excision was done, and the area was covered by artificial derm (Intégra*).

Results: Good cicatrisation; relaspe= 0. Follow-up 13 years (case 1), 5 years (case 2), 2 months (case 3). discussion: The DFS (0.1% of the malignant cutaneous tumors), very rare in children, is located especially at the level of the trunk and the upper limbs (pinky fibrous plate, dermic nodule). The diagnosis is histogenetic (translocation t (17, 22)). It has intermediate malignancy, high-risk local relapse, it imposes a large excision (edges: 5 cm in surface, 3 cm depth until the aponeurosis).The secondary substances losses are difficult to treat..

Conclusion: The DFS, with frequently delayed diagnosis, needs a multidisciplinary management (dermatologist, geneticist and surgeon). The artificial derm can be a satisfactory solution for the substance loss.

 

Key words: Darier-Ferrand dermatofibrosarcoma,