Cases reported "Eye Neoplasms"

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1/6. Fine structure of a radiation-induced osteogenic sarcoma.

    An osteogenic sarcoma arose in the right orbit of a 7-year-old boy some 5 years after the right orbit had been treated by four courses of radiotherapy (total dose approximately 13,000 rads) for a multicentric retinoblastoma. death occurred 6 months after the orbital tumor was first detected. Study of the orbital tumor by electron microscopy revealed a cell population of varied morphology in which two main types were identified. In one group, the cells were large with radiolucent cytoplasm, which contained long branching segments of rough endoplasmic reticulum. In the second group, the cells were smaller with irregular nuclei and an electron-dense cytoplasm, which contained short segments of dilated rough endoplasmic reticulum and numerous mitochondria. The first group of cells closely resembled osteoblasts, while the second group had some features of osteoclasts or their percursors. The branching processes of the tumor cells were separated by an amorphous ground substance, which contained collagen-like fibrils and hydroxyapatite crystals. Crystal deposition was in some instances in close relation to extracellular membrane-bound vesicles.
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2/6. New findings in the chromosome 13 long-arm deletion syndrome and retinoblastoma.

    New clinical and pathologic findings in patients with deletion of the long arm of chromosome 13 (13q-) include optic nerve hypoplasia and retinal dysplasia. fibroblasts derived from patients with a 13q- syndrome with and without retinoblastoma, as well as from familial and sporadic retinoblastoma, are a useful model for the study of genetic susceptibility to the development of spontaneous and radiation-induced cancers. fibroblasts from patients with hereditary retinoblastoma appear more radiosensitive than fibroblasts from patients with sporadic retinoblastoma or normal control patients.
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3/6. Computed tomography in the evaluation of patients with bilateral retinoblastomas.

    Six patients with histologically proven retinoblastomas were examined by computed tomography, two at the time of initial diagnosis and four from 2 to 30 years following treatment, which consisted of radiation therapy alone or with surgery. The spectrum of computed tomographic findings includes: calcified retinal tumor, intracranial subarachnoid spread of tumor, radiation-induced tumors (papillary adenocarcinoma and undifferentiated sarcoma) in the field of prior radiation, and second, primary central nervous system tumor, a pineoblastoma.
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4/6. A new familial cancer syndrome? A spectrum of malignant and benign tumors including retinoblastoma, carcinoma of the bladder and other genitourinary tumors, thyroid adenoma, and a probable case of multifocal osteosarcoma.

    An 11-year-old Caucasian girl who had been cured of bilateral retinoblastoma developed non-radiation-induced osteosarcoma in multiple sites of the extremities. Investigation of the medical histories of 36 of her family members through six generations revealed that 8 relatives on the maternal side (22%) had malignant tumors, predominately genitourinary carcinomas, 2(6%) had benign tumors only, and 2(6%) had both benign and malignant neoplasms. The histologic variety of these tumors, the predominance of genitourinary carcinoma, the higher than expected frequency of tumor appearance over six generations, and the occurrence of malignant tumors in direct lineage suggest that the case of retinoblastoma followed by osteosarcoma is part of a familial cancer syndrome.
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5/6. radiation-induced meningiomas in pediatric patients.

    radiation-induced meningiomas rarely have latency periods short enough from the time of irradiation to the clinical presentation of the tumor to present in the pediatric patient. Three cases of radiation-induced intracranial meningiomas in pediatric patients are presented. The first involved a meningioma of the right frontal region in a 10-year-old boy 6 years after the resection and irradiation of a 4th ventricular medulloblastoma. review of our pediatric tumor cases produced a second case of a left temporal fossa meningioma presenting in a 15-year-old boy with a history of irradiation for retinoblastoma at age 3 years and a third case of a right frontoparietal meningioma in a 15-year-old girl after irradiation for acute lymphoblastic leukemia. Only three cases of meningiomas presenting in the pediatric age group after radiation therapy to the head were detected in our review of the literature.
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6/6. Immunodiagnosis of late, recurrent retinoblastoma.

    The clinical, pathological, and immunohistochemical features of a patient with bilateral retinoblastoma who developed a nasopharyngeal mass seven years following irradiation treatment of the left eye is described. diagnosis of the new tumor was difficult by routine light microscopy. Immunohistochemical staining of the original globes, the nasopharyngeal mass, and a cervical lymph node biopsy specimen with an antiserum prepared against tissue cultured retinoblastoma cells suggested the diagnosis of metastatic retinoblastoma rather than a "radiation-induced" sarcoma.
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