Cases reported "Retinal Neoplasms"

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1/5. osteosarcoma following retinoblastoma: age at onset and latency period.

    In order to assess the role of genetic predisposition in the induction of radiation-induced tumors, we performed statistical analysis on data from the literature and from our own Institute with regard to the age at onset and the latency period of osteosarcoma as the second primary tumor for retinoblastoma with or without subsequent radiotherapy. In retinoblastoma survivors who subsequently developed osteosarcoma, the age at onset of retinoblastoma was young (average of 12 months) in both unilateral and bilateral forms. This suggests that all or almost all of the patients were genetically predisposed by a mutation of one allele of the RB1 gene. For retinoblastoma patients, osteosarcomas occurred 1.2 years earlier inside than outside the radiation field. The latency period between radiotherapy and osteosarcoma onset was 1.3 years shorter inside than outside the radiation field. Interestingly, a bimodal distribution of latency periods was observed for osteosarcomas arising inside, but not outside the radiation field: 40% occurred after a short latency, while the latency of the remaining 60% was comparable to that of osteosarcoma occurring outside the radiation field. This suggests that different mechanisms may be involved in radiocarcinogenesis. A radiation-induced mutation of the second RB1 allele may be the cause of osteosarcomas occurring after a short delay, while other genes may be affected in those occurring after a longer delay.
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2/5. Reconstruction of a complex hemifacial deformity with multiple simultaneous free-flap transfers: case report.

    Severe disfigurement, facial paralysis, abnormal continuity of oral and nasal passages, and velopharyngeal incompetence resulted, following maxillectomy and cranial-base resection for a radiation-induced sarcoma. Oral lining, bone support, facial muscle, and outer skin were provided with a single-staged transfer of radial forearm, scapular bone, scapular skin, and serratus anterior muscle with anastamoses to the contralateral neck. This combination of flaps may be considered in complex facial deformities, and may obviate the need for staged prefabrication.
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3/5. Cataract surgery and intraocular lens implantation in a retinoblastoma case treated by external-beam radiation therapy.

    We report a case of extracapsular cataract extraction with posterior chamber intraocular lens implantation in a pediatric patient with a radiation-induced cataract that developed after combined external-beam radiation therapy and chemotherapy for retinoblastoma. A 12-year follow-up revealed effective tumor regression and sustained visual recovery.
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4/5. phacoemulsification surgery and foldable intraocular lens implantation in a child with regressed retinoblastoma.

    PURPOSE: The management of retinoblastoma has been shifting toward conservative treatment. Visual preservation has become a concern as tumor regression is achieved. To report the surgical approach and outcome of a radiation-induced cataract in an only eye with regressed retinoblastoma. methods: Single case report and review of literature. RESULTS: Small incision sutureless phacoemulsification and implant of an acrylic foldable intraocular lens was performed. Best-corrected visual acuity 1 year after surgery was 20/20. No tumor recurrence has been noted after 27 months of follow-up. CONCLUSIONS: phacoemulsification and foldable intraocular lens implant seemed to be an appropriate therapeutic option for this case.
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5/5. Uncommon presentation of pediatric ruptured intracranial aneurysm after radiotherapy for retinoblastoma. Case report.

    OBJECTIVE: radiation-induced intracranial aneurysms are a rare entity with high mortality. Their pathogenesis is still in debate. Their unique anatomy and behavior should be considered when deciding the proper management. A background of radiation, uncommon anatomic aspects, age of presentation, and location guide us to suspect a radiation-induced etiology. We report the case of a pediatric patient with a ruptured intracranial aneurysm, who previously received radiation therapy to the orbits. We aim to contribute to the literature of this uncommon condition and stress the importance of its prompt diagnosis and treatment. CASE DESCRIPTION: A 12-year-old boy, who received radiation therapy for recurrent bilateral retinoblastomas at age 4 months, suddenly developed severe headache associated with nausea and vomiting. A computed tomography scan revealed subarachnoid hemorrhage. A 4-vessel cerebral angiogram revealed a 2-mm aneurysm in the right A1 segment. The aneurysm was clipped successfully with excellent outcome. After 3 years of follow-up, the patient remains neurologically intact and asymptomatic. A new computed tomography angiogram revealed no new aneurysms. CONCLUSION: Vascular abnormalities develop after radiation injury to the brain. Aneurysm formation after radiation therapy has been previously reported, probably secondary to endothelial injury. In this case, early presentation, unusual anatomy, location, and small size at rupture, in contrast with saccular aneurysms, suggest a radiation-induced etiology.
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