Authors : Hajar Hanguir, Imane Gouzi, Amal Akammar, Ahmed Bennis, Fouad Chraibi, Meriem Abdellaoui, Idriss Benatiya Andaloussi, Afaf Amarti
DOI : 10.18231/j.ijooo.2023.020
Volume : 9
Issue : 2
Year : 2023
Page No : 98-101
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