Clinical diagnosis and treatment of retinoblastoma

  Retinoblastoma is the most common primary malignancy of the eye in childhood. The incidence is approximately 1:15,000 live births, with more than 1,000 new cases in China, and the clinical presentation of RB is dominated by white pupils and strabismus. The tumor may appear as single or multiple lesions on fundus examination, and may be of four types: endogenous, exogenous, mixed and diffuse infiltrative, and may be accompanied by intravitreal or subretinal tumor implantation and retinal detachment.  In addition to traditional ophthalmopexy, modern treatments for retinoblastoma include systemic intravenous chemotherapy, external radiation radiotherapy, localized scleral patch radiotherapy, freezing, laser photocoagulation, warm laser and local injectable chemotherapy (including subconjunctival injection, transcorneal arterial cannula perfusion and intravitreal injection chemotherapy). External radiation radiotherapy has been used less and less due to complications such as second malignant tumors and facial deformities.  The purpose of chemotherapy for RB, also known as chemo-reduction, is to first reduce the size of the tumor through chemotherapy, and then to control the tumor in combination with other local treatments such as cryotherapy, laser, and radiation therapy. The most commonly used systemic chemotherapy regimen is the VEC regimen, which includes vincristine, etoposide and carboplatin. Six dosing cycles are considered a course of treatment.  The choice of treatment for RB should be based on the international stage of RB (ICRB), which reflects the extent of the disease and divides RB into five stages: stage A can be treated with any of the applicable treatments alone, stage BCD usually requires chemical reduction combined with local therapy, and stage E still often requires removal of the eye. There are five types of degeneration in RB tumors after treatment: type O: complete disappearance of the tumor, type 1: complete calcification of the tumor, type 2: complete absence of calcification of the tumor, type 3: partial calcification of the tumor, and type 4: flat atrophic scar of the tumor. The type of degeneration is not related to tumor prognosis.