Authors: Hajar Hanguir*, Imane Gouzi, Amal Akammar, Ahmed Bennis, Fouad Chraibi, Meriem Abdellaoui, Idriss Benatiya Andaloussi, Afaf Amarti
Malignant rhabdoid tumors are rare, poorly differentiated tumors which usually affect children under the age of three. These tumors have a predilection for the kidney, central nervous system and soft tissue. The definition classically relies on a characteristic morphology and the inactivation of the hSNF5/INI1 tumor suppressor gene. The diagnosis is based on radiological explorations, as well as anatomopathological and immuno-histochemical studies.Whatever the location of the tumor, the therapeutic protocol is only decided after multidisciplinary consultation meeting, while resorting to a triad of chemotherapy, surgery and radiotherapy. The prognosis remains poor and the survival rate is below 30%. We report a rare case of retro-orbital malignant rhabdoid tumor of a new born girl. Keywords: Ocular, Congenital tumor
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