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Objective: Central nervous system tumors are the most common solid neoplasm in children, 60%-70% occurring in the posterior fossa. Surgery is the mainstay of treatment but surgery in the pediatric population is associated with a high risk of perioperative complications. We aimed at analyzing the perioperative complications after posterior fossa surgery in a pediatric population and identifying the associated risk factors., Methods: Retrospective study of all pediatric patients undergoing surgery for resection of a posterior fossa tumor between 1999 and 2019, at the University Hospital of Lausanne. Data were collected including age, clinical presentation, tumor localization, presence of preoperative hydrocephalus, timing of surgery, surgical approach, surgical team, extent of surgical resection, perisurgical complications, and histopathological diagnosis. Statistical analysis was performed to correlate the data with the risk of complications., Results: Sixty-seven patients were included. Perisurgical complications were identified in 39 patients (58.2%), of which 14 (35.9%) required corrective interventions. The perioperative mortality rate was zero. In the univariate analysis, surgery performed under emergency conditions, transvermian and telovelar approaches were statistically correlated with an increased rate of complications. Extent of resection, hydrocephalus, and Lansky index at presentation were not predictive of perioperative complications. Midline tumor, tumor volume >25 cm 3 , and surgery performed by a nonspecialized pediatric onconeurosurgeon were found to be independent risk factors in the multivariate analysis., Conclusions: Surgery in the posterior fossa in the pediatric population harbors a high risk of complications. Identifying the variables contributing to these complications is important in order to improve surgical management of these patients., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Retinoblastoma is the most common paediatric neoplasm of the retina, and one of the earliest model of cancer genetics since the identification of the master tumour suppressor gene RB1. Tumorigenesis has been shown to be driven by pathogenic variants of the RB1 locus, but also genomic and epigenomic alterations outside the locus. The increasing knowledge on this "mutational landscape" is used in current practice for precise genetic testing and counselling. Novel methods provide access to pre-therapeutic tumour DNA, by isolating cell-free DNA from aqueous humour or plasma. This is expected to facilitate assessment of the constitutional status of RB1, to provide an early risk stratification using molecular prognostic markers, to follow the response to the treatment in longitudinal studies, and to predict the response to targeted therapies. The aim of this review is to show how molecular genetics of retinoblastoma drives diagnosis, treatment, monitoring of the disease and surveillance of the patients and relatives. We first recap the current knowledge on retinoblastoma genetics and its use in every-day practice. We then focus on retinoblastoma subgrouping at the era of molecular biology, and the expected input of cell-free DNA in the field., Competing Interests: Declaration of competing interest The authors declare no conflict of interest., (Copyright © 2022. Published by Elsevier Masson SAS.)

Purpose: To identify risk factors for acute choroidal ischemia (ACI) after intra-arterial chemotherapy (IAC) for retinoblastoma., Design: Retrospective cohort study., Participants: Two hundred twenty patients (248 eyes) treated with IAC in Lausanne between November 2008 and September 2019 (665 procedures). All patients were evaluated on a monthly basis with fundus photography and fluorescein angiography before and after each IAC injection., Methods: Acute choroidal ischemia, defined as any new choroidal ischemia clinically diagnosed within 35 days after an IAC injection, were noted. Eyes with choroidal complications diagnosed later than 35 days after the last IAC injection (n = 7) or those for which the status of the choroid was not assessable (n = 35) were excluded. Specific procedure parameters and treatment regimens were compared between the group of eyes with and without ACI., Main Outcome Measures: Procedure-related risk factors for ACI after IAC injection and visual acuity assessment in the group of eyes with ACI., Results: Acute choroidal ischemia developed in 35 of 206 included eyes after a mean of 2 injections. No differences were found between the two study groups regarding age at first IAC injection, disease grouping at diagnosis, previously administered treatments, number of IAC injections, drug dose, mean injection time, injection method (pulsatile vs. continuous), or concomitant intravitreal melphalan use. Treatment regimen (melphalan vs. combined melphalan plus topotecan; P < 0.05), catheterization route (internal carotid artery vs. external carotid or posterior communicating artery; P < 0.001), and catheterization type (occlusive into the ophthalmic artery [OA] vs. nonocclusive; P < 0.001) were included in multivariate analysis, and occlusive catheterization was identified as an independent risk factor for ACI (P < 0.001). In the subgroup undergoing an occlusive procedure, placement of the catheter tip into the OA distal third versus medial and proximal thirds (P = 0.04) and a mean catheter diameter-to-OA lumen ratio of 0.6 or more (P < 0.001) were correlated significantly with ACI. Complete vision loss was noted in 27% of the eyes with ACI that were old enough for visual assessment (n = 9/33), whereas 33% maintained a useful vision ranging between 0.1 and 0.8 (n = 11/33)., Conclusions: Catheterization of the OA should be attempted from an ostial position or an external carotid approach to minimize the risk of potentially vision-threatening choroidal complications., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Background & Aims: Poor diet may increase the risk that childhood cancer survivors (CCS) will suffer from chronic disease. We compared adherence to national dietary recommendations between CCS, their siblings and the Swiss population, identified determinants of adherence, and assessed the association of adherence with cardiovascular disease (CVD) risk profiles., Methods: As part of the Swiss Childhood Cancer Survivor Study (SCCSS), a questionnaire was sent to all Swiss resident CCS aged <21 years at diagnosis, who survived ≥5 years and were 16-45 years old at the time of the survey. We compared dietary adherence between CCS, their siblings and participants in the Swiss Health Survey (SHS), a representative survey of the general population. A multivariable logistic regression was used to assess characteristics associated with dietary adherence. We sorted CCS into four kinds of CVD risk groups based on type of treatment (anthracyclines, chest irradiation, a combination, or neither)., Results: We included 1864 CCS, 698 siblings and 8258 participants of the general population. Only 43% of the CCS met the recommended dietary intakes for meat, 34% for fruit, 30% for fish, 18% for dairy products, 11% for vegetables, and 7% for combined fruit and vegetables. Results were similar for both control groups. In all groups, dietary adherence was associated with gender, parental education, migration background, language region in Switzerland, smoking, alcohol consumption and sport participation. CCS with a higher CVD risk profile because of cardiotoxic treatment had no better adherence., Conclusions: CCS have similar food patterns as their siblings and the general population, and poorly adhere to current recommendations. Awareness of the importance of a healthy diet should be raised among CCS, to prevent chronic diseases like CVD., (Copyright © 2016. Published by Elsevier Ltd.)

Purpose: To evaluate the clinical characteristics of the 3 classifications of vitreous seeds in retinoblastoma-dust (class 1), spheres (class 2), and clouds (class 3)-and their responses to intravitreal melphalan., Design: Retrospective, bi-institutional cohort study., Participants: A total of 87 patient eyes received 475 intravitreal injections of melphalan (median dose, 30 μg) given weekly, a median of 5 times (range, 1-12 times)., Methods: At presentation, the vitreous seeds were classified into 3 groups: dust, spheres, and clouds. Indirect ophthalmoscopy, fundus photography, ultrasonography, and ultrasonic biomicroscopy were used to evaluate clinical response to weekly intravitreal melphalan injections and time to regression of vitreous seeds. Kaplan-Meier estimates of time to regression and ocular survival, patient survival, and event-free survival (EFS) were calculated and then compared using the Mantel-Cox test of curve., Main Outcome Measures: Time to regression of vitreous seeds, patient survival, ocular survival, and EFS., Results: The difference in time to regression was significantly different for the 3 seed classes (P < 0.0001): the median time to regression was 0.6, 1.7, and 7.7 months for dust, spheres, and clouds, respectively. Eyes with dust received significantly fewer injections and a lower median and cumulative dose of melphalan, whereas eyes with clouds received significantly more injections and a higher median and cumulative dose of melphalan. Overall, the 2-year Kaplan-Meier estimates for ocular survival, patient survival, and EFS (related to target seeds) were 90.4% (95% confidence interval [CI], 79.7-95.6), 100%, and 98.5% (95% CI, 90-99.7), respectively., Conclusions: The regression and response of vitreous seeds to intravitreal melphalan are different for each seed classification. The vitreous seed classification can be predictive of time to regression, number, median dose, and cumulative dose of intravitreal melphalan injections required., (Copyright © 2015 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Unlabelled: Undifferentiated sarcoma of the liver is a rare primary tumor of childhood: only about 150 cases have been reported in the literature., Case-Report: A 10 year-old girl was admitted because of diarrhea and weight loss. Sonography, then CT-scan and MRI showed a large tumor of the liver., Comments: In the differential diagnosis of primary liver tumors in children, one should think about undifferentiated sarcoma of the liver, especially if imaging shows haemorrhagic foci and if sonography and CT/MRI display a discordant appearance. Survival has improved in the last decade due to agressive surgery and intensive chemotherapy.