Article type :
Case Report
Author :
Hajar Hanguir*, Imane Gouzi, Amal Akammar, Ahmed Bennis, Fouad Chraibi, Meriem Abdellaoui, Idriss Benatiya Andaloussi, Afaf Amarti
Volume :
9
Issue :
2
Abstract :
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.
Keyword :
Ocular, Congenital tumor