Abstract

Trilateral retinoblastoma (TRb) is a rare syndrome associating hereditary bilateral or unilateral retinoblastoma (Rb) with an intracranial neuroblastic tumor. The latter arises in the midline, most often in the pineal gland, less frequently in the suprasellar or parasellar region. The outcome is usually fatal because of secondary spinal dissemination. We report 10-year-old boy presented with a right eye proptosis and leukocoria, and the magnetic resonance imaging (MRI) showed right orbital mass lesion infiltrating optic nerve and diagnosis of retinoblastoma was made. He was referred for fluorodeoxyglucose-positron emission tomography/computed tomography (FDG PET/CT) to find out the extent of the disease. PET/CT showed abnormal FDG-uptake within right orbital mass lesion, suprasellar enhancing lesion and drop metastasis in the cervical spinal canal level. He was diagnosed as a case of TRb with spinal canal drop metastasis. He underwent chemotherapy and craniospinal irradiation.

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