Chemoreduction improves eye retention in patients with retinoblastoma: a report from the German Retinoblastoma Reference Centre.

Background: Retinoblastoma is the most common intraocular childhood tumour. Although mortality is low in Western countries, long-term sequelae, including secondary tumours, compromised vision or loss of one or both eyes are common. Chemoreduction combined with focal treatment is currently the leading conservative treatment for retinoblastoma, with success rates of 50-75% reported.

[Intraocular medulloepithelioma - series of 10 cases and review of the literature].

Intraocular medulloepithelioma is an extremely rare unilateral intraocular tumor arising from the nonpigmented ciliary epithelium. Medulloepitheliomas may be classified as benign and malignant and as teratoid and nonteratoid tumors. As a rule a long latency period occurs after first symptoms until the final diagnosis of a medulloepithelioma is made.

Beta-ray brachytherapy with 106Ru plaques for retinoblastoma.

Purpose: A retrospective analysis of 134 patients who received (106)Ru brachytherapy for retinoblastomas (175 tumors in 140 eyes). Treatment and follow-up were analyzed with special emphasis on tumor control organ, preservation, and late complications.

Results: Treated tumors had a mean height and diameter of 3.

De novo intraocular retinoblastoma development after chemotherapy in patients with hereditary retinoblastoma.

Objective: Identification of incidence and risk factors for recurrence of de novo retinoblastomas after chemotherapy treatment in patients with hereditary retinoblastoma.

Methods: A retrospective, case-control study of 32 patients (50 eyes) with sporadic or familial bilateral retinoblastomas was conducted. Patients received a systemic chemotherapy regimen applying three courses of a combination of three drugs (including vincristine, etoposide, carboplatin, or cyclophosphamide) followed by additional local therapy.

[Retinoblastoma].

Retinoblastomas are the most frequent intraocular tumors in childhood. Untreated, the tumor is almost always fatal. Using a multidisciplinary approach combining the efforts of ophthalmologists, radiation oncologists, pediatric oncologists and geneticists a survival rate of more than 95% can be achieved.

Beta-ray brachytherapy of retinoblastoma: feasibility of a new small-sized ruthenium-106 plaque.

Unlabelled: A non-comparative case observation study estimated the feasibility of brachytherapy for retinoblastoma with a newly designed ruthenium-106 plaque (label: CXS) with an 8-mm diameter of the irradiation zone.

Methods: The new CXS plaque was used between 2001 and 2003 for brachytherapy of 13 retinoblastomas. Indications were recurrences after preceding local treatment or endophytic retinoblastoma with an impending vitreous tumour cell seeding.

Age at diagnosis of isolated unilateral retinoblastoma does not distinguish patients with and without a constitutional RB1 gene mutation but is influenced by a parent-of-origin effect.

Patients with hereditary cancer are usually diagnosed earlier than patients with non-hereditary tumours. In children with isolated unilateral retinoblastoma, some of whom have a hereditary predisposition, this rule has been subject to debate. We have analysed the clinical manifestation of disease in 188 children with completely resolved mutational status.

T1 Gd-enhanced compared with CISS sequences in retinoblastoma: superiority of T1 sequences in evaluation of tumour extension.

Background: As adequate therapy for retinoblastoma in young children depends on infiltration of extra-retinal structures, diagnostic modalities play an essential role.

Methods: In this widely extended study, 80 children with retinoblastoma were studied with MRI (standard fat-suppressed Gd-enhanced T1, T2 thin-slice sequences (additionally with small loop surface coil), constructive interference in steady state (CISS) sequence covering the orbita). The images were analysed by two blinded neuroradiologists.

Thermochemotherapy in hereditary retinoblastoma.

Background/aim: The combination of chemotherapy and transpupillary thermotherapy, thermochemotherapy (TCT) has become an established part of the treatment plan in advanced retinoblastoma. The aim of this study was to identify safe indications, the complications as well as the limitations of this new treatment for retinoblastoma.

Methods: Tumour response and side effects of TCT with an indirect laser ophthalmoscope (spot size about 400 micro m) in 55 tumours of 26 children with bilateral retinoblastoma were analysed.

[Corneoretinal dystrophy (Bietti)-- Long-term course of one patient over a period of 30 years, and interindividual variability of clinical and electrophysiological findings in two patients].

Background: A tapetoretinal dystrophy with crystalline deposits of the retina and limbal cornea was described by Bietti in 1937. To date, only a few cases with long-term follow up have been reported.

Patients And Methods: Two patients are presented including the clinical findings, fluorescein angiography, electrophysiology [electroretinography (ERG); electrooculography (EOG), multifocal electroretinography (MERG)], adaptometry, and transmission electron microscopy (TEM) of peripheral blood lymphocytes.