Primary atypical rhabdoid orbital tumor: An entity with aggressive behavior

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Case Report

Author Details : Hajar Hanguir*, Imane Gouzi, Amal Akammar, Ahmed Bennis, Fouad Chraibi, Meriem Abdellaoui, Idriss Benatiya Andaloussi, Afaf Amarti

Volume : 9, Issue : 2, Year : 2023

Article Page : 98-101

https://doi.org/10.18231/j.ijooo.2023.020

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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.
 

Keywords: Ocular, Congenital tumor

How to cite : Hanguir H, Gouzi I, Akammar A, Bennis A, Chraibi F, Abdellaoui M, Andaloussi I B, Amarti A, Primary atypical rhabdoid orbital tumor: An entity with aggressive behavior. IP Int J Ocul Oncol Oculoplasty 2023;9(2):98-101

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