[Retinoblastoma]. / Retinoblastom.

Retinoblastomas represent 6% of all malignant tumors in children under 5 years old, which untreated lead to blindness in the affected eye and death due to metastases. The main symptoms are leukocoria and strabismus, which if possible, always necessitate a clarification within 1 week for exclusion of a retinoblastoma. The most frequent differential diagnoses are Coats' disease and persistent fetal vasculature (PFV) as well as other intraocular tumors, in particular astrocytomas. Systemic chemotherapy, if necessary in combination with laser hyperthermia, local chemotherapy and brachytherapy are the most important methods for eye-preserving treatment. Advanced cases mostly necessitate enucleation.

[Primary meningioma of the optical nerve sheet in infancy as initial presentation of neurofibromatosis type 2]. / Primäres Meningeom des Nervus opticus im Säuglingsalter als Erstmanifestation einer Neurofibromatose Typ 2.

Intraorbital meningiomas are rare tumors, making up less than 4% of all intraorbital tumors. Intraorbital meningiomas of childhood are curiosities with only few documented cases. We present the case of an 8­month-old male infant, presenting with strabismus and nystagmus. Magnetic resonance imaging showed a long segment thickening of the optical nerve and an intraocular tumor. The tumor was suspicious for retinal dysplasia and enucleation of the eye was performed to exclude malignancy. Histological examination revealed a meningothelial meningioma (WHO grade I), extending along the optical nerve and into the eye accompanied by retinal dysplasia and epiretinal membranes. Meningiomas of childhood, retinal dysplasia, and epiretinal membranes are regularly associated with neurofibromatosis type 2. Subsequent genetic analysis led to the final diagnosis. This case documents a very unusual early beginning of a neurofibromatosis type 2.

[Intraocular Tumors other than Retinoblastoma in Children]. / Intraokulare Raumforderungen im Kindesalter ohne Retinoblastom.

The diagnosis of an intraocular mass in children can be challenging as invasive procedures are not allowed, in particular if a retinoblastoma may be present. In eyes with a unilateral tumour mass and loss of function enucleation with subsequent histopathological processing might be the only option to establish a diagnosis and to exclude a malignant tumour. The present paper deals with intraocular tumours other than retinoblastoma in children, with a special focus on the correlation of modern imaging techniques and histopathological findings.

[New therapeutic strategies for retinoblastoma: non-systemic chemotherapy]. / Neue Therapiestrategien beim Retinoblastom: Nichtsystemische Chemotherapie.

Retinoblastoma is the most common primary intraocular malignancy worldwide. The known established standard therapies for bilateral disease, such as external beam radiation therapy or systemic chemotherapy often lead to a higher morbidity and increased risk of secondary malignancies, especially with radiation therapy. Therefore, new non-systemic chemotherapy strategies, such as the intra-arterial or intravitreal administration of melphalan are being revised with the aim of reducing systemic side effects.

[Value of transretinal tumor biopsy as diagnostic and predictive instrument]. / Stellenwert der transretinalen Tumorbiopsie als diagnostisches und prädiktives Instrument.

Transretinal biopsy of intraocular tumors plays a decisive role as a diagnostic tool in ocular oncology. A biopsy is indicated to confirm a clinical diagnosis before treatment and allows identification of high risk melanomas of the uvea with a high potential of metastasis by molecular genetic evaluation of the specimen. This review will focus on the various biopsy techniques and indications for this method.

[New developments in molecular genetics and treatment of retinoblastoma]. / Neue Entwicklungen zur Genetik und Therapie des Retinoblastoms.

Retinoblastomas are the most frequent primary malignant intraocular tumours worldwide. Conventional and new treatment modalities have significantly improved the chance for survival and preservation of vision. The armamentarium of treatment modalities has been broadened recently by new techniques like intraarterial chemotherapy, which still has to be considered as experimental since long-term follow-up results are not yet available. The excellent prognosis for retinoblastomas in countries with a well developed health system is contrasted by the miserable prognosis for retinoblastomas in developing countries, which must be changed by a joint effort of all centres.