Le neurofibrome plexiforme est une tumeur bénigne rare des nerfs périphériques aux dépens des cellules conjonctives du périnevre. Il est pathognomonique de la neurofibromatose de type 1 (NF1 ou maladie de Von Recklinghausen). L'IRM est d'une grande aide au diagnostic de cette pathologie. La confirmation anatomopathologique est parfois nécessaire en particulier en dehors d'un contexte évocateur d'une NF1. Nousrapportonsl'observation d'une petite fille atteinte de neurofibrome plexiforme cervical révélateur d'une neurofibromatose Type 1.
Renal angiomyolipomas are rare type of benign renal neoplasm. They are composed of vascular, smooth and fat elements and can be associated to phacomatosis as Tuberous Sclerosis disease. Symptomatic presentation is most frequently spontaneous retroperitoneal hemorrhage, which can be fatal. The risk of bleeding is proportional to the size of the lesion (>4 cm of diameter). Typical angiomyolipomas are benign but may have alarming properties: nuclear pleomorphism and mitotic activity, extension into the vena cava, and spread to regional lymph nodes without malignant progression. We report a Computed Tomography finding of a rare giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombus in a patient with tuberous sclerosis, emphasizing the importance of imagery in the positive and etiologic diagnosis.
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