Fibroblastome à cellules géantes de présentation atypique

International audienceBACKGROUND: Giant cell fibroblastoma is a specific entity that belongs to the dermatofibrosarcoma protuberans spectrum. We report an original case with an atypical clinical presentation. CASE REPORT: A four-year-old male child presented with a perineoscrotal mass, present since the age of one year. This lesion was initially a bluish perineal macule that grew rapidly after a traumatic injury. Physical examination showed a large flaccid bi-lobed tumour originating from the posterior border of the left of the scrotum to the anal margin. A haemolymphangioma was clinically suspected and the results of ultrasound and MRI were consistent with this diagnosis. Because of the discomfort and the atypical clinical presentation, local surgical resection was performed. Histological examination did not confirm the clinical assumption but revealed a giant cell fibroblastoma. Because of the location of this tumour, a secondary surgical procedure was carried out using the "Slow-Mohs" technique. DISCUSSION: This case is particularly interesting because of the clinical pseudo-angiomatous presentation of this tumour. Use of the "Slow-Mohs" technique allowed sparing of tissue. No recurrence was noted after 3 years of follow-up