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)

Purpose: The aim of this survey was to review the different studies regarding the occurrence of second primary tumors (s PT) among survivors of retinoblastoma. Methods: Ovid (Medline, Current contents life, Psychlit, Embase) was searched for the years 1966 -1995 using the mesh headings: 'retinoblastoma', 'second primary neoplasms', and 'multiple primary neoplasms'. The inclusion criteria were: the study should involve 50 patients or more and should not be limited to one specific SPT. A checklist with criteria regarding the study design and the results was applied to each study. Results: Eleven studies were identified which met the inclusion criteria. Thirtyfive different types of S P T (Ntotal=z43) were reported. Most of them were osteosarcomas (37.0%), followed by melanomas (7.4%), soft-tissue sarcomas (6.9%), brain tumors (4.5%), fibrosarcomas (3.3%), chondrosarcomas (3.3%), and sarcomas (3.3%). Less frequently reported were leukemias (2.4%), sebaceous cell carcinomas (1.6%), and non-Hodgkin lymphomas (1.6). Pineoblastoma, which in fact is a trilateral retinoblastoma and not an S P T , was found in 2.4%. Despite the differences, all 11 studies showed a higher incidence of S P T compared to the general population. Only 4 studies were judged to be free from selection bias, reporting a cumulative incidence of S P T of 8.4% 18 years after diagnosis, 15.7% at the age of 20 years, 19% at the age of 35 years, and a relative risk of 15.4 for s P T, respectively. Conclusion: s P T is a serious problem for the survivors of hereditary retinoblastoma and its importance shoud be recognized in (genetic) counseling of patients.

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The aim of this register-based follow-up study was to evaluate the long-term cumulative incidence of second primary tumors (SPT) among survivors of hereditary retinoblastoma, with special interest for the incidence of pineoblastoma in retinoblastoma patients born after 1970. The Dutch Retinoblastoma Register was completed and updated: in the period 1945-1994, 639 retinoblastoma patients were registered. The vital status of each patient was obtained from the municipal registries and the Central Office of Genealogy. SPT were traced and histopathologically confirmed. Survival curves and cumulative incidence of SPT were calculated by the Kaplan-Meier method. The survival of patients with hereditary retinoblastoma was significantly shorter than that of patients with non-hereditary retinoblastoma. The cumulative incidence of SPT in hereditary patients was 3.7 and 17.7% at the ages of I0 and 35 years, respectively.Long-term follow-up revealed a high proportion of melanomas (7 melanomas out of 28 SPT). In the sub-cohort of the hereditaryretinoblastoma patient group born after 1970, the cumulative incidence of pineoblastomas at the age of 5 years was 9.3%. Our results suggest that patients with hereditary retinoblastoma should have careful follow-up, and procedures for diagnosing SPT and pineoblastomas at an early and potentially treatable stage should be developed.o 1996 Wiley-Lin, OK. Retinoblastoma is a rare pediatric eye tumor which occurs in both a hereditary and a non-hereditary form. An estimated 30 to 40% of retinoblastomas are hereditary (Vogel, 1979). In some o f the hereditary cases, deletion of chromosome 13q14 was shown to be present in all cells of the body (Knudson etaf., 1976). All bilateral (familial and/or sporadic) cases and the familial unilateral cases can be considered to be hereditary. In familial cases, the patient inherits the retinoblastoma mutation from a parent, and, in sporadic bilateral cases, from a healthy parent in whom a fresh germinal mutation has occured. In the non-hereditary patients (60-70% of all cases), including all sporadic unilateral cases, the tumors are thought to arise as a consequence of somatic mutation in the embryonic retinal cells during fetal life (Vogel, 1979).Although the cure rate of retinoblastoma is excellent after enucleat ion or irradiation, the likelihood of long-term survival in the hereditarygroup is reduced because of the occurrence of second primary tumors (SPT) years after treatment; nonhereditary-retinoblastoma patients do not seem to be at risk for SPT (Draper et al., 1986;DerKinderen et al., 1988;Eng et al., 1993). SPT may appear inside or outside the radiation field (Abramson er al., 1984; DerKinderen et al., 19SQ and adjuvant chemotherapy may enhance their frequency (Draper et al., 1986). Reports on the cumulative incidence of SPT in retinoblastoma are conflicting, ranging from 8.4% 18 years after diagnosis (Draper et al., 1986) to 90% after 30 years (Abramson et al., 1984). In order to resolve this conflict, we instituted an in-depth, retrospective...

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It is generally stated in elementary lectures on sensory systems that the limits of the visible spectrum are 400 nm (violet) and 700 nm (red), although there are several exceptions to this generalization. Recently, the properties of ultraviolet (UV) vision in invertebrates, as well as vertebrates, have been better characterized, and it is worthwhile to examine visual phenomena in the UV region of the spectrum. The purpose of this review is to integrate what is known about UV vision in invertebrates, which have been particularly well studied, with a growing literature on UV sensitivity and UV effects in the visual systems of humans and other vertebrates.

We wished to determine the influence of parental age at the birth of a retinoblastoma patient on the risk of sporadic hereditary retinoblastoma. The parental age at birth of 941 patients of the Dutch retinoblastoma register was identified and compared between sporadic hereditary and nonhereditary patients. In a subcohort , a comparison was made with parental age at birth in the general population, as obtained from the Central Bureau of Statistics. Missing birth dates of the parents of retinoblastoma patients were traced with the help of the municipal registries and the Central Bureau of Genealogy. The mean paternal age was 10.7 months higher and the mean maternal age was 11.0 months higher in the sporadic hereditary retinoblastoma patients than in parents of nonhereditary patients. In the subcohort, the mean paternal and maternal ages of sporadic hereditary patients were also higher (12.4 and 11.5 months, respectively) than those of the general population. All differences were statistically significant. This study shows that a high parental age is associated with an enhanced risk of sporadic hereditary retinoblastoma. IntroductionRetinoblastoma is a malignant pediatric tumor affecting the retina and occurring about once in 20 000 live births. Retinoblastoma occurs in a hereditary form (30%-40% of the patients) and a nonhereditary form (60%-70%; Vogel 1979). It develops following at least two (Knudson 1971; DerKinderen 1987) mutational events, namely, inactivation of both alleles of the retinoblastoma (RB1) gene at 13q14 (Cavenee et al. 1983;Dryja et al. 1986).The hereditary form results from a germline mutation which is present in all body cells. Among hereditary cases, a familial hereditary form and a sporadic hereditary form may be distinguished. An indication for the familial hereditary form is a parent with retinoblastoma or a family member with this disease, indicating that one of the parents must be a carrier of the RB1 gene. In the sporadic hereditary form, no other family members are affected, and the patient is the first person in the family with retinoblastoma. In the nonhereditary form, the retinoblastoma mutation is exclusively found in the tumor cells of the retina (Knudson 1971;Vogel 1979).Epidemiological observations suggest that parental age is related to the genesis of sporadic hereditary disorders such as Down's syndrome and achondroplasia. This is understandable, since the number of new mutations in germline cells increases with age (Penrose 1955;Vogel and Rathenberg 1975;Vogel 1979). The existence of an association of sporadic hereditary retinoblastoma with parental age is still controversial (Falls and Neel 1951;Stevenson and Martin 1957;Smith and Sorsby 1958;Macklin 1960;Matsunaga 1965;Fraser and Friedmann 1967;Tünte 1972;Pellié et al. 1973;Bunin et al. 1989;Matsunaga et al. 1990; DerKinderen et al. 1990). Furthermore, DNA investigations on some patients suggest that new germline mutations are principally of paternal orgin (Ejima et al. 1988;Dryja et al. 1989;Zhu et al. 1989). Th...