BACKGROUND: Retinoblastoma is the most common pediatric intraocular neoplasm. The association of uni- or bilateral retinoblastoma with synchronic or metachronic ectopic midline intracranial tumor [trilateral retinoblastoma (TRB)] is uncommon. MATERIALS AND METHODS: MR examinations of 202 children with retinoblastoma treated at our institute were retrospectively reviewed. MR images and clinical data of children with TRB were evaluated for the patient's age at diagnosis of the intracranial tumor and intraocular lesions, tumor size, signal characteristics, and further course in follow-up MR examinations. RESULTS: There were three patients with TRB in our group of patients. All three children had had a negative family history. Two of them had a primary midline intracranial tumor and intraocular lesions at the time of the first diagnosis. In the third case, the first diagnosis was intracranial midline primitive neuroectodermal tumor. Diagnosis of lesions in both eyes was confirmed in ophthalmologic examination 1 month later. In one case, the intracranial tumor was in the pineal region and, in the other two cases, in the sellar and suprasellar regions. There was no evidence of leptomeningeal spread of the tumors in any patient. CONCLUSIONS: Patients with uni- and bilateral intraocular tumors should receive brain screening by MR imaging. We also recommend that patients under the age of 4 years with midline tumors should be carefully diagnosed for ocular neoplasms.
BACKGROUND:Retinoblastoma is the most common pediatric intraocular neoplasm. The association of uni- or bilateral retinoblastoma with synchronic or metachronic ectopic midline intracranial tumor [trilateral retinoblastoma (TRB)] is uncommon. MATERIALS AND METHODS: MR examinations of 202 children with retinoblastoma treated at our institute were retrospectively reviewed. MR images and clinical data of children with TRB were evaluated for the patient's age at diagnosis of the intracranial tumor and intraocular lesions, tumor size, signal characteristics, and further course in follow-up MR examinations. RESULTS: There were three patients with TRB in our group of patients. All three children had had a negative family history. Two of them had a primary midline intracranial tumor and intraocular lesions at the time of the first diagnosis. In the third case, the first diagnosis was intracranial midline primitive neuroectodermal tumor. Diagnosis of lesions in both eyes was confirmed in ophthalmologic examination 1 month later. In one case, the intracranial tumor was in the pineal region and, in the other two cases, in the sellar and suprasellar regions. There was no evidence of leptomeningeal spread of the tumors in any patient. CONCLUSIONS:Patients with uni- and bilateral intraocular tumors should receive brain screening by MR imaging. We also recommend that patients under the age of 4 years with midline tumors should be carefully diagnosed for ocular neoplasms.
Authors: S H Friend; R Bernards; S Rogelj; R A Weinberg; J M Rapaport; D M Albert; T P Dryja Journal: Nature Date: 1986 Oct 16-22 Impact factor: 49.962
Authors: Firazia Rodjan; Pim de Graaf; Hervé J Brisse; Sophia Göricke; Philippe Maeder; Paolo Galluzzi; Isabelle Aerts; Claire Alapetite; Laurence Desjardins; Regina Wieland; Maja Beck Popovic; Manuel Diezi; Francis L Munier; Theodora Hadjistilianou; Dirk L Knol; Annette C Moll; Jonas A Castelijns Journal: J Neurooncol Date: 2012-07-18 Impact factor: 4.130