Abstract

Primary intraocular malignant rhabdoid tumor is classified as a malignant extrarenal rhabdoid tumor. It is extremely rare, highly aggressive, and, so far, only one case (in a newborn) has been described in the medical literature. The authors report a second case of primary intraocular malignant rhabdoid tumor, this time without extrascleral involvement and in a teenager, and describe its histological, immunohistochemical, and radiological characteristics along with clinical correlations. [J Pediatr Ophthalmol. 2018;55:e7-e9.].

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