1/305. Sporadic bilateral retinoblastoma and 13q- chromosomal deletion.Unilateral retinoblastoma (Rb) is usually a sporadic occurrence while bilateral (multifocal) cases are often familial. Sporadic bilateral Rb associated with a long-arm deletion of a D-group chromosome has been reported in 8 children. We have studied a 6-year-old female with bilateral sporadic retinoblastoma, treated during infancy by enucleation and radiotherapy. chromosome banding studies on peripheral lymphocytes revealed an interstitial deletion from the long arm of a chromosome 13: del(13) (q12q14). Three additional patients reported in the literature had interstitial 13q- deletions, involving slightly different though overlapping regions. The only chromosomal region consistently missing in all of these 4 cases appears to be part of the lightly staining band 13q14. We, therefore, propose this site as the precise location of a gene (or genes) involved in retinal development. Our patient lacked features of the classic 13q- or 13-ring syndrome, which involves deletion of a more distal portion of the 13 long arm. When compared to reported patients with Rb and 13q-, it became apparent that there may be a separate recognizable syndrome consisting of moderate growth and developmental delay, characteristic facies and external ears, and bilateral sporadic Rb, which is associated with an interstitial 13q- deletion.- - - - - - - - - - ranking = 1keywords = retinoblastoma (Clic here for more details about this article) |
2/305. 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.- - - - - - - - - - ranking = 0.16666666666667keywords = retinoblastoma (Clic here for more details about this article) |
3/305. Nonocular cancer in retinoblastoma survivors.From a review of the records of 2,302 patients with retinoblastoma collated from CPMC and AFIP, it was found that retinoblastoma patients who survived their original eye cancer have a high incidence of second nonocular malignancies. The second neoplasms occur almost exclusively (97.5%) in patients who have had bilateral retinoblastoma, although retinoblastoma is much more commonly unilateral. The second neoplasms have appeared between 1 and 42 years after the successful treatment of retinoblastoma and have been fatal in approximately 85% of cases. Seventy-one percent of patients develop tumors in the field of the radiation beam; many of these were following treatment with low doses of radiation and after short latent periods. Nineteen percent of patients develop tumors clearly out of the field of radiation (eg, osteogenic sarcoma of the femur). retinoblastoma patients appear to be unusually radio-sensitive to low doses of radiation and develop tumors in the field of radiation following treatment of 3,500 rads. They have a high incidence of nonocular tumors clearly distant from the radiation beam develop nonocular malignancies even if no radiation has been given. The incidence of multiple malignancies in patients successfully treated for retinoblastoma appears to be higher than for any other primary malignancy whether they receive radiation or not.- - - - - - - - - - ranking = 1.6666666666667keywords = retinoblastoma (Clic here for more details about this article) |
4/305. Osteogenic sarcoma and phthisis bulbi: a case report.PURPOSE: To describe a case of osteogenic sarcoma (osteosarcoma) that developed within a phthisical eye. METHOD: Case report. RESULTS: An 86-year-old woman with a 20-year history of phthisis bulbi developed pain and proptosis. Tumor was identified by computed tomography. An exenteration was performed, and osteogenic sarcoma was identified. CONCLUSION: Osteogenic sarcoma is the most common primary malignant tumor of bone. In the orbit it frequently is associated with prior irradiation for retinoblastoma. We describe the first case of osteogenic sarcoma that developed de novo from bone within a phthisical eye.- - - - - - - - - - ranking = 0.16666666666667keywords = retinoblastoma (Clic here for more details about this article) |
5/305. uveitis and reticulum cell sarcoma of brain with bilateral neoplastic seeding of vitreous without retinal or uveal involvement.A 65-year-old woman had been observed for more than four years with bilateral chronic nongranulomatous uveitis and vitreous clouding of unknown cause. Her death was from reticulum cell sarcoma of the brain (microglioma). Both eyes were obtained post mortem. Histopathologic examination revealed malignant cell in the vitreous of both eyes, but no other tumor was demonstrable in the ocular tissues. Reticulum cell sarcoma should be suspected in middle-aged or older persons with chronic unilateral or bilateral uveitis of unknown cause who develop cerebral manifestations during the course of the disease.- - - - - - - - - - ranking = 5.1618114876693E-6keywords = glioma (Clic here for more details about this article) |
6/305. Osteogenic sarcoma after retinoblastoma radiotherapy.Development of osteogenic sarcoma after retinoblastoma radiotherapy in three patients, two of whom were siblings, is reported. Pluridirectional tomography and plain skull radiography demonstrated soft tissue masses, sinus opacification, and bone destruction and sclerosis in all three patients. Computed tomography reliably indicated the presence or absence of intracranial tumor extension in the two patients in whom it was performed. Radionuclide bone scanning was a useful adjunct for osteosarcomatous detection.- - - - - - - - - - ranking = 0.83333333333333keywords = retinoblastoma (Clic here for more details about this article) |
7/305. A high-grade primary leiomyosarcoma of the bladder in a survivor of retinoblastoma.Second nonocular malignancies develop with increased incidence in patients with hereditary retinoblastoma. osteosarcoma is by far the most common type with an incidence of up to 50%, followed by soft tissue sarcomas. Visceral leiomyosarcoma is extremely rare and only 2 cases have been reported in the past 2 decades, one in the liver and another one in the urinary bladder, both of which developed after cyclophosphamide therapy. Here we report a case of vesical leiomyosarcoma that was diagnosed in a 49-year-old woman 47 years after the diagnosis of a hereditary retinoblastoma. The patient's retinoblastoma was treated with unilateral enucleation without adjuvant radiation or chemotherapy. We believe that this is the first report of vesical leiomyosarcoma occurring in a patient with retinoblastoma without a prior history of radiation or chemotherapy. This report is significant not only because of the rarity of vesical leiomyosarcoma as a second nonocular tumor in retinoblastoma patients, but also because of the infrequency of vesical leiomyosarcoma in general. We also investigated the potential molecular pathogenesis of the leiomyosarcoma.- - - - - - - - - - ranking = 1.5keywords = retinoblastoma (Clic here for more details about this article) |
8/305. scleral buckling in retinoblastoma: case report.In the course of treating retinoblastoma in a monocular child, a progressive retinal detachment occurred. Although no retinal break was demonstrated, several clinical features suggested that the detachment was rhegmatogenous. A successful scleral buckling procedure was performed without adverse sequelae. The rationale for this approach is discussed.- - - - - - - - - - ranking = 0.83333333333333keywords = retinoblastoma (Clic here for more details about this article) |
9/305. 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.- - - - - - - - - - ranking = 1.3333333333333keywords = retinoblastoma (Clic here for more details about this article) |
10/305. Cystic retinoblastoma.A grossly cystic retinoblastoma, presumably a form of the diffuse infiltrating type, mimicked uveitis in an 81/2-year-old boy. A review of 44 cases indicated that grossly (clinically) detectably cysts are rare in this neoplasm, although microcysts are relatively common. Lesions of this nature, especially if presenting with a history of trauma, as in our patient, may lead the ophthalmologist to diagnose uveitis. The diagnosis of retinoblastoma may thus be unduly delayed.- - - - - - - - - - ranking = 1keywords = retinoblastoma (Clic here for more details about this article) |
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