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Introduction Ventriculo-peritoneal shunt (VPS) related ascites is a rare complication of pediatric low grade gliomas (pLGG). Physiopathology of this complication is not fully understood and there is paucity of data regarding the molecular profile of pLGG gliomas complicating with ascites and the optimal management of this unusual event. Methods International multi-institutional retrospective analysis of patients diagnosed with BRAF altered pLGG and ascites arising as a complication of VPS. Demographics, tumor characteristics, therapeutic approaches and outcomes were recorded. Results Nineteen patients were identified. Median age at diagnosis was 14 months (R: 2–144). Most patients (17; 89.4%) presented with lesions involving the optic pathway. Mean tumor standard volume was 34.8 cm 2 (R: 12.5–85.4). Pilocytic Astrocytoma was the most frequent histological diagnosis (14;7 3.7%). Eight (42.1%) tumors harbored BRAF V600-E mutation and seven (36.8%) KIAA1549 fusion. The onset of ascites was documented at a median time of 5 months following VPS insertion. Four (21%) patients were managed with paracentesis only, 7(36.8%) required both paracentesis and shunt diversion, 7(36.8%) required only a shunt diversion and 1 (5.2%) patient was managed conservatively. Chemotherapy regimen was changed in 10 patients following ascites. Eight patients received targeted therapy (4 dabrafenib/4 trametinib) and 5 were radiated. There were eleven survivors with a median OS of 69 months (R: 3–144). Conclusions Ascites is an early feature in the clinical course of young patients with midline BRAF altered pLGG, with high mortality rate observed in our cohort. The hypothesis of ascites as an adverse prognostic factor in pLGG warrants further prospective research.

Purpose: This study aims to report visual acuity outcomes for patients with optic pathway gliomas (OPG) treated with systemic chemotherapy and analyze the associated factors. Patients and Methods: A retrospective study of 29 children with OPG treated with chemotherapy at King Hussein Cancer Center (KHCC), Amman, Jordan, between May/2005 and August/2020. Details on patient demographics, tumor location, systemic chemotherapy, and progression of disease were extracted from medical records. Results: Fifty-four eyes of twenty-nine patients were included in this study with a follow-up range from 2 to 17 years. Sixteen patients (55%) had a history of neurofibromatosis-1 (NF1). Most of the eyes (31, 57%) had visual acuity ranges in the moderate or better group. The age group [greater than or equal to] 5 years at diagnosis, those with hydrocephalus, and patients with non-NF1 presented the worst visual acuity ranges from severe or worse; the p-value was 0.043, 0.0320, and 0.0054, respectively. Following treatment with systemic chemotherapy, visual acuity improved in 5 (17%) patients, remained the same in 23 (79%) patients, and only one patient (3%) had vision deterioration. Of the five patients who showed vision improvement, only one had radiological regression of the tumor. Parallel to this, three (10%) patients showed tumor progression in the final magnetic resonance image (MRI) findings without affecting the final vision. Conclusion: Children older than 5 years at diagnosis, in sporadic OPG, and those with hydrocephalus had the worst vision at presentation. Treatment with systemic chemotherapy prevented further deterioration of vision, and following treatment with systemic chemotherapy, most of the patients had the same vision; this stability indicates that vision at diagnosis is an important predictor for the final visual outcome. Keywords: glioma, neurofibromatosis, neuro-oncology, pediatric tumors, vision

Venous thromboembolism (VTE) in children is relatively rare, and more so among those with cancer. In this study, we report the characteristics and outcomes of children with cancer-associated thrombosis. We reviewed institutional databases for all children with cancer and a diagnosis of VTE at King Hussein Cancer Center in Jordan. Variables reviewed are patients' clinical characteristics, treatment for cancer, and anticoagulation therapy. Between January 2011 and December 2018, a total of 45 patients fulfilled the inclusion criteria, and the median age was 10.4 (0.8-17.9) years. The most common underlying diagnosis was acute lymphoblastic leukemia (n = 13, 29%). At the time of VTE, 29 (64.4%) patients were receiving chemotherapy, and eight (17.8%) had a central venous catheter (CVC). The majority of patients (n = 37, 82%) developed VTE within 30 days of hospitalization. Thrombosis mostly involved the extremities (n = 23, 51%) and sagittal vein (n = 12, 26.7%). All patients were treated with low-molecular-weight heparin (LMWH), complicated by bleeding in three (6.6%) patients. In contrast to adults, VTE in pediatric cancer patients is more associated with chemotherapy and recent hospitalization. LMWH is a safe and effective therapy for children with cancer who develop VTE.

Abstract Background The effect of neuraminidase inhibitor (NAI) treatment on length of stay (LoS) in patients hospitalized with influenza is unclear. Methods We conducted a one-stage individual participant data (IPD) meta-analysis exploring the association between NAI treatment and LoS in patients hospitalized with 2009 influenza A(H1N1) virus (A[H1N1]pdm09) infection. Using mixed-effects negative binomial regression and adjusting for the propensity to receive NAI, antibiotic, and corticosteroid treatment, we calculated incidence rate ratios (IRRs) and 95% confidence intervals (CIs). Patients with a LoS of <1 day and those who died while hospitalized were excluded. Results We analyzed data on 18 309 patients from 70 clinical centers. After adjustment, NAI treatment initiated at hospitalization was associated with a 19% reduction in the LoS among patients with clinically suspected or laboratory-confirmed influenza A(H1N1)pdm09 infection (IRR, 0.81; 95% CI, .78–.85), compared with later or no initiation of NAI treatment. Similar statistically significant associations were seen in all clinical subgroups. NAI treatment (at any time), compared with no NAI treatment, and NAI treatment initiated <2 days after symptom onset, compared with later or no initiation of NAI treatment, showed mixed patterns of association with the LoS. Conclusions When patients hospitalized with influenza are treated with NAIs, treatment initiated on admission, regardless of time since symptom onset, is associated with a reduced LoS, compared with later or no initiation of treatment. We found that neuraminidase inhibitor (NAI) treatment initiated on hospital admission to patients with clinically diagnosed or laboratory-confirmed A(H1N1)pdm09 virus infection was associated with a reduction in hospital length of stay when compared to later or no NAI treatment.

Background and Objectives: Primary central nervous system (CNS) tumors are often associated with relatively poor outcomes. Data on the epidemiology and outcome of CNS tumors in Jordan are scarce. We aim to report the epidemiology and outcome of primary CNS tumors of patients managed at a comprehensive cancer care center in Jordan. Methods: We performed a retrospective chart review of all Jordanian patients with a primary CNS tumor diagnosis who were managed at the center between July 2003 and June 2019. We included all entities described in the 2021 CNS WHO classification system, in addition to pituitary neuroendocrine tumors (PitNETs). We used the Kaplan–Meier method to estimate the 1-year, 2-year, and 5-year overall survival (OS) rates for each entity. Results and Findings: We included 2094 cases. The numbers of pediatrics and adults were 652 (31.1%) and 1442 (68.9%), respectively. The three most common groups of tumors were “gliomas, glioneuronal tumors, and neuronal tumors” (n = 1200 [57.30%]), followed by meningiomas (n = 261 [12.5%]), embryonal tumors (n = 234 [11.2%]). The three most common tumor families were adult-type diffuse gliomas (n = 709 [33.8%]), medulloblastoma (n = 199 [9.5%]), and circumscribed astrocytic gliomas (n = 183 [8.7%]). The median survival for the entire cohort was 97 months (95CI; 81–112). Survival was significantly worse for males and adults compared to their respective counterparts. Among the most common tumor group, “gliomas, glioneuronal tumors, and neuronal tumors”, OS rates for adult-type diffuse gliomas were significantly lower than all other types. Overall, adult gliomas with IDH-mutations had a survival advantage over wildtype cases (IDH-mutant 1-year OS, 89% [82–97%] vs. IDH-wildtype 1-year OS, 60% [52–70%]; p < 0.001). Conclusions: We present a detailed analysis of the primary CNS tumors diagnosed in the largest cancer center in Jordan between 2003 and 2019. We compared the epidemiology and overall survival of these patients to worldwide estimates and found the epidemiology and outcome of these tumors comparable to worldwide trends.

Purpose Medulloepithelioma is a rare brain tumor that has been classified as embryonal tumor with multilayered rosettes (ETMR) if it harbors C19MC amplification. In rare instances, it shows evidence of heterologous differentiation. Methods We report a case of a 10-year-old female who presented with headache, squint, and minimal left sided weakness of 1 week duration. Results Microscopy revealed a small round blue cell tumor with focal glandular and tubular differentiation. In one focus, well-developed osteoid was identified. The tumor labeled with LIN28A immunostain. Conclusions Unusual features can be encountered in medulloepithelioma which should be in the differential diagnosis of CNS embryonal tumors. Full description of the case with review of the literature and comparison between cases with and without heterologous elements is presented.

Background Management of craniopharyngioma in children is challenging, and their quality of life can be significantly affected. Series describing this from low-middle income countries (LMIC) are few. Patients and methods The study provides a retrospective chart review of pediatric patients <18 years old, diagnosed with craniopharyngioma between 2003 and 2014, and treated at King Hussein Cancer Center, Jordan. Results Twenty-four patients (12 males) were identified. Median age at diagnosis was 7.4 years (0.9–16.4 years). Commonest symptoms were visual impairment and headache (71%). Review of seventeen preoperative MRIs showed hypothalamic involvement in 88% and hydrocephalus in 76%. Thirteen patients (54%) had multiple surgical interventions. Five patients (21%) had initial gross total resection. Eleven patients (46%) received radiotherapy and six (25%) intra-cystic interferon. Five years’ survival was 87 ± 7% with a median follow-up of 4.5 years (0.3–12.3 years). Four patients (17%) died; one after post-operative cerebral infarction and three secondary to hypothalamic damage. At their last evaluation, all but one patient required multiple hormonal supplements. Ten patients (42%) had best eye visual acuity (VA) >20/40, and four (16%) were legally blind. Eleven patients (46%) were overweight/obese; one had gastric bypass surgery. Seven patients had hyperlipidemia, and eight developed fatty liver infiltration. Eleven patients (65%) were attending schools and one at college. Nine of the living patients (53%) expressed difficulty to engage in the community. Conclusions Management of pediatric craniopharyngioma is particularly complex and demanding in LMIC. Multidisciplinary care is integral to optimize the care and minimize the morbidities. A management outline for LMIC is proposed.

Synchronous primary malignant brain tumors are rare. We present a 5-year-old boy with synchronous glioblastoma and medulloblastoma. Both tumor samples had positive p53 stain and loss of PMS2 and MLH1 stains. The child had multiple café au lait spots and a significant family history of cancer. After subtotal resection of both tumors, he received craniospinal radiation with concomitant temozolomide followed by chemotherapy, alternating cycles of cisplatin/lomustine/vincristine with temozolomide. Then, he started maintenance treatment with cis-retinoic acid (100 mg/m 2 /day for 21 days). He remained asymptomatic for 34 months despite a follow-up brain MRI consistent with glioblastoma relapse 9 months before his death. Cis-retinoic acid may have contributed to prolong survival in this child with a probable biallelic mismatch repair syndrome.

Purpose To describe a medication error of intrathecal Cytarabine overdose that was managed conservatively with no apparent toxicities. Summary An 11-year-old girl was diagnosed with bone marrow relapsed precursor B-cell acute lymphoblastic leukemia. According to her chemotherapy protocol, she was started on triple intrathecal chemotherapy consisting of Methotrexate, Cytarabine and Hydrocortisone on day 1 of the protocol. After the intrathecal therapy being administered to the patient, the pharmacist who checked the medication realized that the wrong formulation of Cytarabine was used to prepare the intrathecal therapy; this error resulted in five times overdose of Cytarabine. The patient was then managed conservatively without cerebrospinal fluid exchange. Our patient remained clinically and neurologically stable without apparent toxicities and was discharged safely from hospital. Conclusion Supportive care without the need for invasive procedures such as cerebrospinal fluid exchange may be adequate for managing intrathecal Cytarabine overdose.