JXG is a benign lesion of unknown incidence. It is the most frequent type of non-langerhans histiocytosis with a median age of 2 years. It usually presents as isolated cutaneous lesions. Multiple lesions, especially over the head and neck, may occur. The skin lesions tend to regress slowly with time. Extra-cutaneous and visceral involvements have been observed, the most common site being the eye. When the lesions are numerous, they may persist, hence the need for treatment with corticosteroids or chemotherapy. Histologically, the lesion consists of histiocytes admixed with an inflammatory infiltrate of variable density. The lesions are initially monomorphic and very cellular, progressively enriched with multinucleated giant cells of Touton and foamy cells, followed by spindle cells. We report an 8-year old girl with JXG of early type without multinucleated and foamy cells. This case presented as a tumour in the inferior meatus of nasal cavity, clinically simulating a rhabdomyosarcoma. This atypical clinical and histological presentation with benign evolution should be recognized since it requires only local treatment.
Child Female