Background: Clinical presentations of paraneoplastic syndromes in neuroblastoma may multiply. Review of the clinical data and the literature on this syndrome may help in the diagnosis of neuroblastoma. Objectives: In order to make more accurate diagnosis, we reviewed the clinical data and the literature on this syndrome. Patients and Methods: Between April 2007 and April 2012, 68 children were diagnosed with neuroblastoma or ganglioneuroblastoma in our institution, 9 of which presented exclusively with paraneoplastic syndromes and were not treated with chemotherapy prior to diagnosis. After the diagnosis, all patients received chemotherapy and operation on NB97 protocol. Results: Among 68 pediatric patients with neuroblastoma or ganglioneuroblastoma, 4 (5.9%) patients suffered from neurological complications at diagnosis, 2 (2.9%) patients had digestive tract disorders, 2 (2.9%) patients had immune diseases, and 1 (1.5%) suffered from hematological disorder (without bone marrow involvement). All paraneoplastic syndrome patients achieved complete remission on paraneoplastic syndrome before completion of chemotherapy. Conclusions: Neuroblastoma may present with a range of non-specific neurologic symptoms in addition to the well-known opsoclonus-myoclonus syndrome and cerebellar ataxia. In any case, the presence of unexplained neurologic manifestations and other common clinical presentations such as rash, constipation, diarrhea, and especially immune disorders in an otherwise healthy child had raised the possibility of paraneoplastic syndrome due to the presence of an undiagnosed tumor. Keywords: Paraneoplastic Syndromes; Neuroblastoma; Child; Ganglioneuroblastoma; Nervous System Diseases

Purpose: Chemotherapy is commonly used for treatment in children over three years old with high-risk medulloblastoma(MB). However, little is currently known about the therapeutic benefits and side effects of intrathecal methotrexate(MTX), warranting further research. Methods: In this retrospective study, patients who received intrathecal MTX during chemotherapy were included in the MTX group (n = 32), and patients that only underwent cerebrospinal fluid (CSF) cytology analysis were assigned to the control group (n = 14). Results: In the MTX group, 27(84.38%) patients had metastatic disease, 3(9.38%) had diffuse anaplasia, and 3(9.38%) had residual disease greater than 1.5 cm². Molecular subgroup classification was available for 28(87.5%) patients. In the control group, 8(57.14%) patients had metastatic disease, 3(27.27%) had diffuse anaplasia, and 6(42.86%) had residual disease greater than 1.5 cm². Molecular subgroup classification was available for 6(42.86%) patients. The 5-year progression-free survival was 70.99% and the 5-year overall survival was 72.99% for the MTX group, and the corresponding values were 41.67% and 50% for the control group, respectively. 6 (18.75%) patients in the MTX group with group 4 disease developed MTX-related acute leukoencephalopathy and one of them died. Conclusions: Our findings support the addition of intrathecal MTX during chemotherapy as the optimal management for children with group 3 and SHH high-risk MB. However, it is not recommended for group 4 MB patients, especially in resource-limited regions. Trial registration number: : Retrospective registered No.(2020 − 117).

Background: Accurate molecular and clinical stratification of patients with central nervous system (CNS) non‐germinomatous germ cell tumors (NGGCTs) remains challenging, impeding the development of personalized therapeutic approaches. Herein, we investigated the translational significance of cerebrospinal fluid (CSF) circulating tumor DNA (ctDNA) in pediatric NGGCTs to identify characteristic features of CNS NGGCTs and to identify a subset of patients for whom the presence of residual disease is a risk factor and an indicator of shorter progression‐free survival (PFS) and overall survival (OS).Methods: Medical records of patients with CNS NGGCTs between January 1, 2018 and December 31, 2022 were reviewed retrospectively.Results: The cohort consisted of 11 male and six female patients. Tumor markers were elevated in four of the five people who underwent surgery. The remaining 12 patients were diagnosed with malignant NGGCTs according to elevated tumor markers. Among them, ctDNA before chemotherapy as well as ctDNA clearance were consistently associated with PFS and OS (p < .05). By setting a ctDNA positivity threshold of 6%, patients with high ctDNA (above the threshold) levels, which had limitation due to the selection based on optimal statistic from the survival analysis, had significantly inferior 5‐year PFS and OS compared to those with low levels (below the threshold). ctDNA or ctDNA clearance combined with the presence of residual disease predicted significantly worse OS and PFS (p < .05).Conclusions: CSF ctDNA might allow the study of genomic evolution and the characterization of tumors in pediatric NGGCTs. CSF ctDNA analysis may facilitate the clinical management of pediatric NGGCT patients, and aid in designing personalized therapeutic strategies.

Currently, no available coherent management protocol exists for pediatric cancers associated with pleural effusion, ascites, and pericardial effusion. This study aimed to retrospectively present our experience in treating pediatric cancer patients with pleural effusion, ascites, and pericardial effusion using interleukin-2 (IL-2) and dexamethasone (DEX) intracavitary injections. Between January 1st, 2008 and December 31st, 2020, medical reports of patients diagnosed with solid tumors or lymphoma were checked to identify patients diagnosed with > 2 cm pleural effusion, and/or more than grade 1 ascites, and/or more than small pericardial effusion. Patients diagnosed with effusions and treated with IL-2 and DEX were identified as being in the effusion group. Meanwhile, patients with the same primary tumors and effusions but did not receive interleukin 2 and DEX injection were reviewed and classified as the control group. Forty patients with solid tumors and 66 patients with lymphoma were further diagnosed with pleural effusion, ascites, or pericardial effusion. A total of 85 patients received IL-2 and DEX injection while the remaining 21 did not. The Kaplan Meier analysis revealed a significant difference between the two groups, with p < 0.01 for event free survival (EFS) and p < 0.01 for overall survival (OS), both of which had p < 0.01. Hazard ratio was found to be 0.344 for OS and 0.352 for EFS. This retrospective study illustrates that thoracic, intraperitoneal, or pericardial intracavitary injection of DEX plus IL-2 can be an effective and safe treatment for pediatric cancers with pleural effusion, ascites, and pericardial effusion.

Purpose: This work aimed to report our experience with ultrasound-guided instillation for the treatment of lymphangiomas in children, so as to determine whether the combined use of bleomycin and dexamethasone achieved a higher response rate and a lower side effect rate.Methods: The medical records from patients with lymphangiomas between January 1st, 2013 and September 31st, 2020, were reviewed. Patients who received bleomycin combined with dexamethasone sclerotherapy were classified as the dexamethasone group, while those receiving bleomycin without dexamethasone were classified as the control group.Results: Altogether one hundred and twenty-seven patients were diagnosed with lymphangiomas. Among them, one hundred and five patients received bleomycin combined with dexamethasone injection, while the remaining twenty-two received bleomycin injection alone. The excellent rates were 89.52% [95% confidence interval (CI), 81.64–94.40%] in the dexamethasone group and 72.73% (95% CI, 52.51–92.94%) in the control group (p < 0.05). Additionally, the recurrence rates were 3.81% (95% CI, 1.22–10.03%) in the dexamethasone group and 13.64% (95% CI, 3.6–36.0%) in the control group (p > 0.05). After comparison between the two groups, the following risk factors were identified. These include >10 sacs at the initial stage of diagnosis, larger size after all injections, and response to the first injection.Conclusions: Although there was no significant difference in the recurrence rate between the two groups, this retrospective study demonstrated that the excellent response rates were dramatically improved between the two groups, suggesting that bleomycin combined with DEX was an effective and highly safe treatment for all types of pediatric lymphangiomas. Moreover, this study also identified three novel features as the significant risk factors for recurrence.

Purpose: Hepatoblastoma (HB) tumor rupture is currently considered as a high-risk factor in some risk stratification systems. This study aimed to investigate the value of HB tumor rupture in predicting the poor prognosis.Methods: The clinical data from children with high-risk HB or HB tumor rupture at our institution from October 2008 to 2017 were retrospectively reviewed and analyzed.Results: Together, 34 children with high-risk HB or HB tumor rupture were retrospected, including 25 in the high-risk group and nine in tumor rupture group. The 3-year overall survival (OS) rate in tumor rupture group was significantly higher than that of the high-risk group (100 vs. 64%, p = 0.0427). In tumor rupture group, seven (77.8%) of nine patients had a hemoglobin level ≤ 8 g/L and 3 of them (33.3%) had ≤ 6 g/L at the time of diagnosis. Peritoneal perfusion with interleukin-2 was implemented for each patient. At the end of the treatment, seven (77.8%) of nine patients achieved complete response (CR). No patient died at the last follow-up.Conclusions: HB tumor rupture might not be predictive of poor prognosis with the risk of peritoneal dissemination/relapse, in which peritoneal perfusion with interleukin-2 could play a role.