Aggresomes are inclusion bodies for misfolded/aggregated proteins. Despite the role of misfolded/aggregated proteins in neurological disorders, their role in cancer pathogenesis is poorly defined. In the current study we aimed to investigate whether aggresomes-positivity could be used to improve the disease subclassification and prognosis prediction of pediatric medulloblastoma. Ninety three pediatric medulloblastoma tumor samples were retrospectively stratified into three molecular subgroups; WNT, SHH and non-WNT/non-SHH, using immunohistochemistry and Multiplex Ligation Probe Amplification. Formation of aggresomes were detected using immunohistochemistry. Overall survival (OS) and event-free survival (EFS) were determined according to risk stratification criteria. Multivariate Cox regression analyses were carried out to exclude confounders. Aggresomes formation was detected in 63.4% (n = 59/93) of samples. Aggresomes were non-randomly distributed among different molecular subgroups ( P = 0.00002). Multivariate Cox model identified aggresomes’ percentage at ≥20% to be significantly correlated with patient outcome in both OS (HR = 3.419; 95% CI, 1.30–8.93; P = 0.01) and EFS (HR = 3; 95% CI, 1.19–7.53; P = 0.02). The presence of aggresomes in ≥20% of the tumor identified poor responders in standard risk patients; OS ( P = 0.02) and EFS ( P = 0.06), and significantly correlated with poor outcome in non-WNT/non-SHH molecular subgroup; OS ( P = 0.0002) and EFS ( P = 0.0004).

Colorectal carcinoma (CRC) is rare in children with extremely poor prognosis. Proper management is obscure because of deficient data. The aim of the current study is to assess the clinical characteristics and to determine the different prognostic factors leading to dismal outcome. It is a retrospective study included all CRC patients, below 18 years, treated in Children's Cancer Hospital of Egypt (CCHE) between 2007 and 2016. Demographic data, clinical characteristics, diagnostics, histological subtypes, disease stage, treatment methods, and survival outcome were collected. The result showed that, 15 patients below 18 years. All had unfavorable histopathology (mucinous adenocarcinoma) and 10 cases had metastatic disease. Initial surgical resection was done in 8/15 cases, all patients received neo adjuvant/adjuvant chemotherapy. Four patients had rectal adenocarcinoma and were treated with chemo-radiotherapy while 11 had colonic adenocarcinoma. Ten patients had progression or relapse, while 12 died at the end of follow up period; 3-years Overall Survival (OS) and Event Free Survival (EFS) were 17.8% and 16.5% respectively, the only three surviving patients were of lower stage disease. In Conclusions, Clinical presentation of CRC in pediatrics is similar to adults but delayed diagnosis and advanced stages contribute to poor outcome which is due to the absence of familiarity to CRC. The high frequency of mucinous adenocarcinoma may explain the poor outcome. Surgery remains the mainstay of treatment as in adults. Preoperative radiotherapy for rectal adenocarcinoma, offers better prognosis. Chemotherapy plays a role in the metastatic disease and can downstage the primary tumor for better local control.

Background: This study explores the prognostic factors and outcomes of different treatment modalities in focal brain stem glioma (FBSG). Materials & methods: Pediatric FBSG patients diagnosed during 2010–2017 were retrospectively reviewed for clinical and therapeutic data. Results: A total of 71 cases were identified and the median age was 6.4 years. The 5-year overall- and progression-free survival were 74.5 and 70.6%, respectively. Radiotherapy was the main line of treatment (66.2%) and there were no survival differences between radiotherapy, chemotherapy and surveillance groups. Two independent poor prognostic factors were identified on multivariate analysis: age <8 years and cervicomedullary tumor site (p = 0.02 for both). Conclusion: Surveillance, radiotherapy and chemotherapy have comparable clinical outcomes in pediatric FBSG.

Background: This study aimed to investigate diffuse intrinsic pontine glioma-specific symptoms in the last 12 weeks before death and to describe current palliative care. Materials & methods: A retrospective study included 80 pediatric diffuse intrinsic pontine glioma patients diagnosed between January 2018 and December 2019. Results: The most frequently encountered symptoms were headache, gait disturbance, vomiting, dysphagia, sensory loss, seizures and constipation. Steroids were used in 96% of patients with a high success rate, as well as analgesics (67.5%), antiemetics (59%), neuropathic medication (42.5%) and anticonvulsants (37.5%). Re-irradiation improved symptoms in 50% of patients. Conclusion: Steroids were efficient in managing many symptoms, with tolerated side effects. The symptomatic treatment succeeded in relieving end-of-life symptoms. Re-irradiation should be considered a good palliative tool in addition to regular symptomatic treatment.