1/27. Advanced bilateral retinoblastoma treated conservatively with lens sparing external beam radiation therapy: report of three cases.From 1995 through 1998, 3 children with bilateral advanced retinoblastoma were treated primarily with external beam radiation therapy; 6 eyes were irradiated with a lens sparing technique, doses varied from 5500 to 5700 cGy, and follow-up period ranged from 14 to 36 months. Recurrent tumors were found in 3 eyes, and a new growing tumor in one eye. Three eyes underwent enucleation eventually; one eye refused enucleation and finally developed optic nerve extension. The overall ocular cure rate was 2/6 (33.3%). One eye sustained visual acuity of 20/30, the other eye retained some peripheral vision; both eyes were blind in one patient. There were no deaths, metastasis, or secondary malignant tumors in our study. Advanced bilateral retinoblastoma with simultaneous radiation therapy instead of bilateral enucleation does not increase the risk of death, and more children will enjoy the benefits of retaining some vision in the affected eye through the use of this conservative therapeutic regimen.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/27. Vascular anastomoses between the iris and persistent hyperplastic primary vitreous.Prominent radial iris blood vessels created small but definite pupillary notches in five children with persistent hyperplastic primary vitreous and persistence of the tunica vasculosa lentis. Detection of these abnormal iris vessels suggested the presence of persistent hyperplastic primary vitreous and related syndromes even when the diagnosis was obscured by the presence of an opaque lens. These vessels represented early developmental arrest and may have been associated with abnormal development of the macula and optic nerve. Surgical and amblyopia therapy, therefore, may not provide useful vision, and should be undertaken with caution.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
3/27. genome instability in secondary solid tumors developing after radiotherapy of bilateral retinoblastoma.genome alterations of seven secondary tumors (five osteosarcomas, one malignant peripheral sheath nerve tumor, one leiomyosarcoma) occurring in the field of irradiation of patients treated for bilateral retinoblastoma have been studied. These patients were predisposed to develop radiation-induced tumors because of the presence of a germ line mutation in the retinoblastoma gene (RB1). Tumor cells were characterized by a high chromosome instability whereas microsatellites and minisatellites were found to be stable. In all tumors, the normal RB1 allele was lost with the corresponding chromosome 13, whereas the germ line mutated allele was retained. The two alleles of TP53 were inactivated, one by deletion of the short arm of chromosome 17, the other by mutation. As compared with non-radiation-induced tumors, the observed panel of TP53 mutations was uncommon with sites not recurrently found otherwise and a high rate of deletions (3/7). In these predisposed patients, the loss of the single normal allele of RB1 is rather due to the radiation-induced chromosome instability than a direct effect of ionizing radiation.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
4/27. 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 = 1keywords = nerve (Clic here for more details about this article) |
5/27. visual fields in a successfully radiated retinoblastoma patient.1. Computerized visual fields have recently been used to study patients before and after treatment of choroidal melanoma with plaque therapy. Such information is unavailable for patients diagnosed with retinoblastoma. 2. There is no information on the long term visual fields of successfully treated retinoblastoma; therefore, no comparisons can be made between the effects of photocoagulation, cryotherapy, plaques, and external beam radiation on the visual field defects. 3. External beam radiation to tumors around the optic nerve caused widespread patchy sparing because of partial destruction of the nerve fiber layer. Whether the destruction was related to the position of the tumors or the effect of the radiation is unknown.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
6/27. A unique case of retinoblastoma masked by retinal detachment diagnosis and management.A 4-year-old Latin American boy presented with a history of loss of vision of his left eye for 7 months. He was followed by different ophthalmologists and diagnosed with a retinal detachment. Computerized tomograms and magnetic resonance imaging demonstrated a large calcified left eye and markedly enlarged optic nerve. Based on these radiologic findings diagnosis of retinoblastoma was strongly suspected. Initially enucleation was not done because it was thought that cutting through the involved optic nerve would cause dissemination. The patient was treated with chemotherapy which resulted in marked response. Following normalization of optic nerve and after irradiation the eye was enucleated. The diagnosis of retinoblastoma was confirmed. No tumor was found in the optic nerve. The importance of imaging studies in diagnosis of retinoblastoma and the role of chemotherapy in the treatment of primary retinoblastoma are emphasized.- - - - - - - - - - ranking = 4keywords = nerve (Clic here for more details about this article) |
7/27. Pleomorphic adenoma of the lacrimal gland in an 18-year-old girl irradiated for bilateral retinoblastoma.PURPOSE: To report a case of pleomorphic adenoma of the lacrimal gland following irradiation for bilateral retinoblastoma. MATERIALS AND methods: Case report. A 4-month-old girl, with bilateral retinoblastoma, underwent enucleation of the right eye, systemic chemotherapy and bilateral external beam irradiation with a lateral field. The right anophthalmic socket (because of infiltration of the optic nerve) and the left eye (for relapse) were irradiated;. 17 years later, she developed a mass in the superotemporal quadrant of the left orbit. The mass was completely excised. RESULTS: Pleomorphic adenoma is rare in children and teenagers; it usually presents as a painless, slow growing mass in healthy adults. In this case, it developed as a second primary tumor after irradiation for retinoblastoma.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
8/27. Late-onset retinoblastoma in a well-functioning fellow eye.PURPOSE: To describe the documented growth, clinical course, and histopathology of retinoblastomas in an untreated and otherwise normal right eye of a 27-year-old white male with a g.153211T>A (p.Tyr606X) mutation in the retinoblastoma 1 gene, whose left eye was enucleated at age 2 years for 2 retinoblastomas. DESIGN AND PARTICIPANTS: Retrospective interventional case report. INTERVENTIONS: Over the years, the right eye was irradiated twice and underwent trans-pars plana vitrectomy, transscleral cryocoagulation, argon laser photocoagulation of tumors and their feeder vessels, extracapsular cataract extraction with posterior chamber lens implantation, and neodymium:yttrium-aluminum-garnet laser treatment of after-cataract in the form of Elschnig's pearls. Finally, the patient received combination chemotherapy with etoposide, methotrexate, actinomycin D, cisplatin, and vincristine. RESULTS: The eye finally had to be removed 12 years later due to tumor recurrences and seeding, pseudohypopyon, and elevated intraocular pressure. Histopathology showed microcellular retinoblastoma cells in the anterior chamber angle and trabecular meshwork without subconjunctival extension and in the nasal ciliary body, pars plana, internal limiting membrane, and optic nerve head anterior to the cribriform plate. The patient is without local or systemic recurrences at age 50, 11 years after the last eye was enucleated. CONCLUSIONS: This report shows that retinoblastoma patients may have tumor growth in their fellow eye 25 years after the first eye and also that Elschnig's after-cataract pearls still can arise after irradiation of a lens with 45 Gy.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
9/27. Phthisis bulbi and buphthalmos as presenting signs of retinoblastoma: a report of two cases and literature review.PURPOSE: To report two cases of bilateral retinoblastoma (RB) with unusual presentations. methods: The medical records of 321 patients from the retinoblastoma Referral Center in Siena were reviewed. A total of 111 patients had bilateral RB, 2 of them presenting with phthisis bulbi and buphthalmos. Both patients underwent bilateral enucleation. Clinical features, imaging studies, and histopathology were reviewed. RESULTS: These 2 cases represent 0.62% (2/321) in our series. Histopathology did not reveal viable tumor cells in the phthisical eyes; in both buphthalmic eyes the tumor was active, infiltrating the choroid and optic nerve. CONCLUSIONS: Phthisis bulbi and buphthalmos are unusual presenting signs of RB. This very rare combination of these two signs in different eyes of the same patient is probably due to a delay in diagnosis.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/27. Ischemic necrosis and atrophy of the optic nerve after periocular carboplatin injection for intraocular retinoblastoma.PURPOSE: To report four cases of optic nerve neuropathy in three children treated with periocular carboplatin injections for unilateral or bilateral intraocular retinoblastoma. DESIGN: Retrospective, observational case series. methods: Setting: University-based ophthalmology Practice. Study population: Four eyes of three children with retinoblastoma enucleated after nonsuccessful multimodality treatment including periocular carboplatin injections. observation procedures: The enucleated eyes were routinely processed and evaluated by light microscopy. A retrospective chart review of all four cases was performed. RESULTS: Three enucleated eyes (Reese-Ellsworth groups III and VB) were obtained from two children with bilateral multifocal retinoblastoma, and one eye (Reese-Ellsworth group IIB) was harvested from a child with unilateral retinoblastoma. All affected eyes underwent three to seven periocular carboplatin injections before enucleation. Additional treatment modalities included systemic chemotherapy, transpupillary thermotherapy, transscleral cryotherapy, and external beam radiotherapy. Histopathologic evaluation of the enucleated eyes revealed focal areas of ischemic necrosis or atrophy of the optic nerve along with dystrophic calcification and mild inflammation in the surrounding fibrovascular adipose tissue. CONCLUSIONS: Periocular injections of carboplatin may be a useful treatment approach in the management of patients with advanced intraocular retinoblastoma and may minimize systemic side-effects. However, ophthalmologists and pediatric oncologists should be aware of potential marked local complications with periocular carboplatin delivery, including ischemic optic neuropathy. Modifying the injection site/location (for example, subtenon space) or adding other delivery routes adjuncts (for example, fibrin sealant) deserves further study.- - - - - - - - - - ranking = 6keywords = nerve (Clic here for more details about this article) |
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