Chronic medical conditions in adult survivors of retinoblastoma: Results of the Retinoblastoma Survivor Study

Abstract

Background Limited data are available on long-term morbidity in adult retinoblastoma (Rb) survivors. Methods The Rb Survivor Study is a retrospective cohort of adult Rb survivors diagnosed between 1932 and 1994. Participants completed a comprehensive questionnaire, adapted from the Childhood Cancer Survivor Study (CCSS) surveys. Chronic conditions were classified using the Common Terminology Criteria for Adverse Events v4.03. Multivariate Poisson regression was used to compare Rb survivors with 2,377 non-Rb controls, consisting of the CCSS sibling cohort, and survivors with bilateral versus unilateral disease. Results Rb survivors (53.6% with bilateral disease) and non-Rb controls had mean ages of 43.3 (SD 11) and 37.6 (SD 8.6) years, respectively, at study enrollment. At a median follow-up of 42 years (range, 15-75), 86.6% of Rb survivors had at least one condition and 71.1% had a severe/life-threatening (grade 3-4) condition. The adjusted relative risk of a chronic condition in survivors, compared to non-Rb controls, was 1.4 (95% confidence interval [CI], 1.3–1.4, p<0.01); for a grade 3-4 condition, the risk was 7.6 (95% CI, 6.4–8.9, p<0.01). Survivors were at excess risk regardless of laterality. After stratifying by laterality and excluding ocular conditions and SMN, only those with bilateral disease were at increased risk for any non-ocular, non-SMN condition (RR 1.2; 95% CI, 1.1–1.2) and for grade 3-4 non-ocular, non-SMN conditions (RR 1.7; 95% CI, 1.2–2.5). Conclusions Rb survivors have an increased risk of chronic conditions compared to non-Rb controls. After excluding ocular conditions and SMN, this excess risk persists only for those with bilateral disease.

Metabolite systems profiling identifies exploitable weaknesses in retinoblastoma

“…Additionally, patients with germ line mutation are prone to secondary cancers compared to the general population [8]. However, both enucleation and chemotherapy have severe side effects, including intellectual and developmental disabilities [11][12][13]. Currently, intravitreal chemotherapy with melphalan and intra-arterial chemotherapy with carboplatin are commonly practiced to save the vision of these children.…”

“…If these measures fail, enucleation is done [9,10]. However, both enucleation and chemotherapy have severe side effects, including intellectual and developmental disabilities [11][12][13]. Recently, metabolomics on RB samples identified a number of metabolites (majorly from amino acid and lipid metabolism), concentrations of which significantly differed from healthy tissue [14].…”

Residential Pesticide Exposures in Pregnancy and the Risk of Sporadic Retinoblastoma: A Report From the Children's Oncology Group

“…5 In adulthood, these children have an increased risk of chronic medical conditions including a second malignant neoplasm. 6 …”

Psychosoziale Versorgung pädiatrisch-onkologischer PatientInnen

“…Pädiatrisch-onkologische PatientInnen gehören zudem zur Risiko-bzw. Hochrisikogruppe für die Entwicklung eines Zweitmalignoms [6,8]; dreißig Jahre nach Erstdiagnose beträgt das Risiko durchschnittlich etwa 6,6 % für alle Krebsarten [11]. Diese Tatsache kann eine erhebliche psychische Belastung darstellen [31].…”