Heritable retinoblastoma and pinealoma
G. T. Lueder, G. F. Judisch and B. C. Wen
Department of Ophthalmology, University of Iowa Hospitals and Clinics, Iowa City 52242.
A fourth case of bilateral retinoblastoma (RB) associated with pinealoma
was identified among 56 patients with heritable RB. Using life-table
analysis, the association was 5.6% 2 years after RB diagnosis.
Retinoblastoma-pinealoma (RB-P) was uniformly fatal in this series and in
40 additional patients described in the literature. Ninety-five percent of
patients with RB-P had bilateral RB, a positive family history of RB, or
both. All patients with pinealoma presented with symptoms of increased
intracranial pressure due to obstructive hydrocephalus. The advanced stage
of the tumors at diagnosis may contribute to their uniform lethality. The
incidence of a positive family history of RB in patients with pinealoma was
higher than expected (62% of patients), which may be related to earlier
surveillance and treatment of patients with positive family histories of
RB.
