Retinoblastoma in the state of Oklahoma: a clinicopathologic review

This is a retrospective review of 56 patients diagnosed with retinoblastoma (Rb) from 1970 to 1990 at either the Dean A. McGee Eye Institute or Children's Hospital of Oklahoma. Twenty-six (46%) patients had somatic Rb and thirty (54%) had germinal Rb. On average, somatic disease presented at 20.5 months and germinal disease at 11.7 months. Nine (16%) of our patients have died; of these, metastatic disease was the cause of death in six patients, whereas a midline pineal tumor (trilateral retinoblastoma) was the cause of death in three patients. Metastatic disease presented an average of 17.5 months after the diagnosis of Rb, and death occurred an average of 10.3 months after the diagnosis of metastasis. Trilateral Rb presented an average of 18 months after the diagnosis of retinoblastoma, and on average death occurred 2 months later. Histopathology was available on forty-nine of our patients. Poorly differentiated tumor histology, optic nerve invasion of tumor cells past the surgical plane of transection, and choroidal invasion of tumor cells appear to be significant histological markers for developing metastatic disease. The cytogenetics, current therapy, and related management of retinoblastoma are reviewed.