TRILATERAL RETINOBLASTOMA - A REPORT OF 5 PATIENTS

BACKGROUND. Trilateral retinoblastoma is a well recognized, although r are, syndrome. Most of the reported cases have involved a family histo ry of retinoblastoma (RB) and the disease is almost always fatal. The authors chose to investigate the cases of trilateral retinoblastoma oc curring in the West Midlands, a region of the United Kingdom with an i ncreasing incidence of bilateral sporadic RB. METHODS. Five patients w ith trilateral retinoblastoma (including two were previously reported) , diagnosed in 146 consecutive patients with RB in the West Midlands H ealth Authority Region between 1957 and 1994, are presented (an incide nce of 3%). Their clinical presentation, treatment, and outcome are de scribed.RESULTS. There were 4 patients with pineoblastoma, only one of whom had a positive family history. The mean age at diagnosis of RB w as 6 months, whereas the patients with pineoblastoma were diagnosed at a mean age of 2 years 8 months. The tumors were not evident on the in itial computed tomography scans. One child presented with a calcified suprasellar mass 13 months before the bilateral sporadic RB was identi fied. Death occurred within 1 month of diagnosis of the intracranial t umor in 3 patients who did not receive any treatment. In the other 2 p atients who were treated, death occurred at 15 months and 2 years 7 mo nths, respectively, after diagnosis of intracranial tumor. CONCLUSIONS . Early diagnosis with regular neuro-imaging and more aggressive treat ment may improve prognosis in patients with RB and an intracranial tum or. (C) 1996 American Cancer Society.