Early detection, improved treatment methods help more patients survive retinoblastoma

In nations with advanced medical care, infants with retinoblastoma have a 95% chance of survival, but the worldwide survival rate is approximately 50%, according to a literature review by two ocular oncology experts. That difference is due to improved early clinical detection and a changed approach to management, said Carol L. Shields, MD, and Jerry A. Shields, MD, the authors of the review. These changes have allowed clinicians in the United States and other countries to save the eyes of many infants in recent years, they said.

“Retinoblastoma continues to be a challenge both diagnostically and therapeutically,” the authors said.

Changes in management that have led to greater eye survival rates have included more effective chemotherapy regimens, improved focal treatment modalities and the desire to avoid loss of the globe or exposure to external radiotherapy, the authors said.

Retinoblastoma is primarily a hereditary condition, the Drs. Shields noted in their review. Patients with retinoblastoma are at risk for three life-threatening problems: tumor metastasis, intracranial neuroblastic malignancy and second primary tumors, they said.

Clinical manifestations vary with the stage of the disease, they said. Tumor growth patterns can be classified as intraretinal, endophytic and exophytic. Intraretinal retinoblastomas are small and limited to the retina. Endophytic retinoblastoma grows from the retina towards the vitreous cavity. Exophytic retinoblastoma spreads from the retina outward to the subretinal space.

Treatment should be tailored to the patient, and tumor reduction should be targeted according to tumor size and risk for growth, the authors said.

Eyes with very advanced retinoblastoma still require enucleation in about 50% of cases, they noted.

The review is published in the journal Cancer Control.