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[Intraocular retinoblastoma: new therapeutic options].

Retinoblastoma is the most frequently occurring primary intraocular malignant tumour in children (12-15 new patients per year in the Netherlands). It occurs in one or two eyes. Bilateral retinoblastoma, which occurs in 40% of the cases, is always hereditary; unilateral retinoblastoma, which is found in 60% of cases, is hereditary in 10% of these cases. The presenting symptoms are: leucocoria, strabismus or a red, painful eye. Early detection of retinoblastoma is important for the chance of survival, the visual prognosis and preservation of the eye. The choice of treatment is based on the risk of metastases, the diameter and the location of the tumour, the age of the patient, the heredity and the visual prognosis. Nowadays, treatment more often consists of a combination of techniques. Enucleation is carried out when a large tumour fills over half of the globe; often this is the only possible treatment. Small tumours (diameter and thickness < 2 mm) in the centre of the retina can be treated with laser therapy and those in the peripheral retina by cryotherapy. Small to medium-sized tumours (< 8 mm diameter) can be treated with thermochemotherapy: systemic chemotherapy and laser hyperthermia, if necessary with adjuvant laser therapy or brachytherapy. Medium-sized tumours (< 8 mm thick) can be treated with just brachytherapy, sometimes preceded by chemoreduction.

Antineoplastic Agents↗

[Studies on thymidine kinase gene (TK) and GCV system for treatment of human retinoblastoma (RB)].

The antitumor effect of herpes simplex virus thymidine kinase (HSV-TK)/GCV system on human retinoblastoma (RB) was studied here. A retroviral vector with tk gene (pLXSN-TK) was transduced into packaging cell line PA317. Recombinant retroviral was obtained and employed to infect human RB cells. The in vitro efficacy of TK/GCV was evaluated by survival rate of RB cells with and without TK transduced 5 days after treated with GCV. A nude mouse model with heteroplantation of human RB was established to examine the in vivo efficacy. Mice with RB were given an in situ injection of retrovirus followed by treatment with GCV for 14 days (50 mg/kg). The RB/TK cells in tissue culture dish showed far more sensitive to GCV than RB cells. The tumors in RB mice with TK gene transduced were much smaller than those in control. The results indicate that HSV-TK/GCV system can suppress growth of RB both in vitro and in vivo. It could be a valuable method for treatment of RB patients.

Animals↗

Clinical cases in AIDS. 2.

As the human immunodeficiency virus (HIV) epidemic continues, there are increasing numbers of patients with HIV-related disease. Those doctors studying for the MRCP exam will need to be familiar with the common manifestations of HIV infection and acquired immunodeficiency syndrome.

Adult↗

Preoperative embolization for meningeal tumors: evaluation of vascular supply with angio-CT.

When evaluating vascular supply, a combined angiography and CT (angio-CT) system provides more accurate vascular anatomy than digital subtraction angiography. To the best of our knowledge, however, the application for intracranial tumors has not been described. We herein describe a technique of an angio-CT system for diagnosis of vascular anatomy of the feeding artery for preoperative embolization of meningeal tumors.

Aged↗

[Meningiomas of the wings of the basilar bone in patients undergone a course of radiation therapy for retinoblastoma in infancy (analysis of 3 cases)].

The paper presents 3 cases of radio-induced meningiomas in patients who underwent irradiation for retinoblastoma in infancy. Meningiomas of the middle cranial fossa were diagnosed in women aged 16, 34, and 47 years. Radiation therapy was in the complex treatment of retinoblastoma in all patients in infancy (4 months to 3 years). The latent period between the irradiation and diagnosis of meningioma was 15 to 44 years. The location of detected meningiomas (the wings of the basilar bone on the side of exenteration or enucleation) corresponded to the irradiation fields. Surgical treatment yielded good results. Histological study has indicated that meningiomas were fibroblastic in the former two cases and atypical meningioma was verified in the third case when the tumor infitratively spread. Our observations support the fact that there may be radio-induced meningiomas in the area exposed to irradiation.

Adolescent↗

Outcome following initial external beam radiotherapy in patients with Reese-Ellsworth group Vb retinoblastoma.

OBJECTIVE: To describe patient and ocular outcomes following initial treatment with external beam radiotherapy (EBT) in eyes with Reese-Ellsworth group Vb retinoblastoma. METHODS: Retrospective case series (from January 1, 1979, to February 28, 2002). The Kaplan-Meier method was used to analyze survival (ocular and patient) and incidence (second cancer) data. RESULTS: Two hundred forty-three patients with 1 or more Reese-Ellsworth group Vb eyes were identified. Of 284 group Vb eyes, 63 (22.2%) initially received EBT, vs 172 (60.6%) that were initially enucleated. Of the 63 radiated group Vb eyes, 31 (49.2%) had no further tumor growth, 26 (41.3%) developed a recurrence, and 8 (12.7%) developed a new tumor. Of the 63 radiated group Vb eyes, 33 (52.4%) developed ocular complications. The ocular survival rate of radiated group Vb eyes was 81.4% at 1 year and 53.4% at 10 years. Twenty-eight radiated group Vb eyes survived to the last follow-up with visual acuity information. Thirteen patients developed second cancers, 11 in the field of radiation. The probability of developing a second cancer following initial EBT for group Vb disease in patients with bilateral disease was 29.7% by 10 years after diagnosis. Survival from second cancers in patients with bilateral disease initially receiving EBT for group Vb disease was 93.6% at 5 years and 52.6% at 18(1/4) years. No patient with unilateral disease developed a second cancer. Deaths due to metastatic retinoblastoma were uncommon. CONCLUSIONS: To our knowledge, this is the first study focusing exclusively on group Vb eyes treated initially with EBT, most of which were salvaged with vision. Outcome data provided herein are clinically relevant when choosing treatment options for advanced intraocular retinoblastoma.

Eye Enucleation↗

Macular retinoblastoma managed with chemoreduction: analysis of tumor control with or without adjuvant thermotherapy in 68 tumors.

OBJECTIVE: To evaluate the effectiveness of chemoreduction alone and chemoreduction with thermotherapy for macular retinoblastoma. DESIGN: Prospective, nonrandomized, single-center case series. SETTING: Ocular Oncology Service at Wills Eye Hospital of Thomas Jefferson University in conjunction with the Division of Oncology at the Children's Hospital of Philadelphia (Pa). PARTICIPANTS: There were 68 macular retinoblastomas in 62 eyes of 49 patients managed with chemoreduction from January 1995 through January 2003. Intervention All patients received 6 cycles of intravenous chemoreduction using vincristine, etoposide, and carboplatin. The patients were then treated according to 1 of 2 approaches: chemoreduction alone with no adjuvant focal therapy (group A) or chemoreduction combined with adjuvant foveal-sparing thermotherapy to each macular retinoblastoma (group B). Main Outcome Measure Tumor recurrence. RESULTS: Of the 68 tumors, 28 were in group A and 40 were in group B. A comparison of both groups revealed that the tumors were similar with regard to clinical features. The mean tumor basal dimension was 12.3 mm for group A and 12.1 mm for group B, and the mean tumor thickness was 6.8 mm for group A and 6.1 mm for group B. Tumors in group A occupied a mean of 71% of the macula, and those in group B occupied 74% of the macula. Following treatment, Kaplan-Meier estimates revealed that group A tumors showed recurrence in 25% by 1 year and 35% by 4 years whereas those in group B showed recurrence in 17% by 1 year and 17% by 4 years. All recurrences were treated with additional focal thermotherapy, cryotherapy, or plaque radiotherapy except for 1 that required external beam radiotherapy and 1 that required enucleation, both in group A. Univariate analysis revealed that predictors of tumor recurrence were intraretinal growth pattern (vs endophytic); small tumor basal dimension (less than 3 mm and occupying a smaller percentage of the macula); absence of subretinal fluid, subretinal seeds, and vitreous seeds; and chemoreduction response with less tumor calcification and tumor regression of type 0 (complete disappearance without a scar). By multivariate analysis, the most important factors predictive of tumor recurrence were smaller macular tumor size (judged by percentage of the macula occupied by the tumor), absence of subretinal or vitreous seeds, and unilateral disease. CONCLUSIONS: Treatment of macular retinoblastoma with chemoreduction plus adjuvant foveal-sparing thermotherapy provides tumor control of 83% by 4 years, and this is slightly more favorable than chemoreduction alone, which provides control of 65% by 4 years. Tumors most destined for recurrence are small tumors.

Antineoplastic Combined Chemotherapy Protocols↗

Retinoblastoma and deletion of the long arm of chromosome 13: an underestimated diagnosis?

We report an infant with normal neurological development and phenotype who developed bilateral retinoblastoma (RB). This patient, despite lack of dysmorphic features, demonstrated constitutional abnormality of the long arm of chromosome 13 on standard karyotype. We recommend systematic cytogenetic examinations complemented by fluorescent in situ hybridization as second-line screening in all patients suspected for hereditary RB despite negative RB1 molecular screening and normal phenotype.

Antineoplastic Combined Chemotherapy Protocols↗