Incidence and survival of retinoblastoma in the Netherlands: a register based study 1862-1995

Abstract

Aim-The aim of this study was to determine the (time trends in) incidence and survival of hereditary (familial and sporadic) and non-hereditary retinoblastoma for male and female patients born in the Netherlands between 1862 and 1995. Method-The national retinoblastoma register was updated and now consists of 955 patients. The missing dates of death were obtained from the municipal registers and the Central Bureau of Genealogy in The Hague. Mortality was compared with the Dutch vital statistics. Results-From 1862 to 1995 no significant diVerences in incidence for retinoblastoma were found in the hereditary subgroups. Further, no significant differences between males and females were found, both overall and in the hereditary subgroups. The average incidence of retinoblastoma increased untill 1944, probably due to incompleteness of the register, and stabilised after 1945 (1 per 17 000 live births). From 1900 to 1995 the standardised mortality ratio increased for hereditary retinoblastoma patients from 2.9 to 9.0 and decreased for non-hereditary retinoblastoma patients from 1.9 to 1.0. Conclusion-Although survival for retinoblastoma was significantly better after 1945 than before, in comparison with the Dutch population the mortality between 1900 and 1990 increased for the hereditary and decreased for the non-hereditary retinoblastoma patients. (Br J Ophthalmol 1997;81:559-562)

Genes and environment: effects on the development of second malignancies in retinoblastoma survivors

“…Eng et al analyzed the long term follow-up study of patients from New York and Boston (USA) and found that the cumulative probability of death from second cancers was 26% at 40 years after bilateral retinoblastoma diagnosis [1]. Moll et al reported similar findings from a large patient registry in The Netherlands with a cumulative probability of death among hereditary retinoblastoma patients of 18% at 35 years [13]. Deaths from second tumors are also more frequent among females for unclear reasons [1].…”

“…By clinical observation, patients with unilateral retinoblastoma who should be considered high risk for carrying the germinal mutation include: patients with a family history of retinoblastoma (including a family history of retinocytoma); patients diagnosed under the age of 6 months; and patients who present with multifocal disease. An excess of mortality from second cancers has also been found among unilateral retinoblastoma patients compared with the general population, implicating the germinal mutation in a small percentage of these patients as the cause [1,13].…”

“…Treatment approaches for retinoblastoma in developed countries are now so effective that in the USA, Europe, and developed countries throughout the world, the leading cause of death among retinoblastoma survivors is not retinoblastoma itself, but second malignancies [1]. The incidence of second malignancies in survivors of retinoblastoma who carry the rb1 mutation has been reviewed extensively [1][2][3][4][5][6][7][8][9][10][11][12][13]. Cumulative incidence reports of second malignancies have varied, at least in part because of the wide range in design and population size of these reports.…”

Genomic gains on chromosome 1q in retinoblastoma: Consequences on gene expression and association with clinical manifestation

“…[1][2][3] Mutations resulting in loss of function of both alleles of the RB1 gene, a tumor-suppressor gene located on 13q14, are a prerequisite for the development of this tumor. [4][5][6][7] About 60% of affected children develop Rb in one eye only (unilateral Rb).…”

Familial retinoblastoma: fundus screening schedule impact and guideline proposal. A retrospective study

“…1 Most cases are sporadic and only 10% are familial (defined by a positive family history of RB). All patients having presented a bilateral RB and up to 10% having presented a unilateral RB are expected to carry a germinal mutation and can thus transmit the disease to their offspring.…”