Tumors In Pediatric Population Research Articles

LMIC-03. BRAINSTEM RADIATION NECROSIS IN A PATIENT WITH RECURRENT MEDULLOBLASTOMA. SUCCESFULL TREATMENT OF A HIGHLY FEARED COMPLICATION

Abstract BACKGROUND Medulloblastoma is the most common malignant brain tumor in pediatric population. Standard treatment combines surgery, risk-adapted craniospinal irradiation and chemotherapy. Given the well-known cognitive sequelae in young children associated to radiation, strategies in infants try to avoid upfront radiation. A significant number of patients treated during infancy will ultimately relapse. Re-irradition in relapse setting has proven to be effective in disease control (survival depending on several factors) although there is a concern in exceeding radiation tolerance and risk of toxicity. METHODS Herein, we describe the case of male patient that presented at 1 year of age with a non-metastatic anaplastic medulloblastoma that was completely resected. He received 3 cycles of adjuvant chemotherapy (as per Head Start II), local relapse was detected in tumor bed during evaluation and was completely resected, afterwards he received myeloablative chemotherapy with stem cell rescue and posterior fossa radiation therapy (54 Gy). At 8 months off therapy patient developed spinal relapse in imaging with negative cytology, then treated with spinal irradiation 36Gy without adjuvant chemotherapy. He remained disease free during 12 months, then tumor recur at the right cerebellopontine angle, surgery was complete and there was no evidence of distant disease. Patient received adjuvant local radiation with 30.6Gy followed by chemotherapy (bi weekly bevacizumab + irinotecan). Cumulative maximal dose to brainstem was 84Gy. 2 months after radiation patient presented with extreme sleepiness and imaging findings compatible with brainstem radiation necrosis, symptoms resolved promptly after scheduled bevacizumab. Patient is currently 6-year-old and remains free of disease after 3 years off therapy. CONCLUSION Medulloblastoma treatment in infants is challenging because survival is not the only goal. Future clinical trials will help to better tailored treatment based on molecular biology. Re-irradiation in medulloblastoma has been described as well tolerated but clinician should be aware of its complications and management.

Extra-skeletal intracranial mesenchymal chondrosarcoma: systematic-literature review.

Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection. A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, "primary intracranial chondrosarcoma", "extraskeletal mesenchymal chondrosarcoma", "mesenchymal chondrosarcoma" and "pediatric". Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described. The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2years (range 2months to 18years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired. IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis. SafeMaximal resection followed by radiotherapy is the preferred treatment strategy.

Incidence and Management of Appendiceal Neuroendocrine Tumors in Pediatric Population: A Bicentric Experience with 6285 Appendectomies.

Neuroendocrine tumors (NETs) are rare tumors that arise from neuroendocrine cells and are the most common tumors of the appendix. NETs of the appendix usually cause no symptoms and often go unnoticed until they cause acute appendicitis or are discovered during an accidental appendectomy. As the trend towards the conservative treatment of acute appendicitis increases in the pediatric population, the question arises as to whether the majority of NETs go undetected and are only discovered at an advanced stage. The purpose of the proposed study is to review the incidence and outcomes of treatment for NETs of the appendix in children and include the data presented in the data pool for further review. From 1 January 2009 to 1 November 2023, a total of 6285 appendectomies were performed in two large pediatric centers in Croatia. After a retrospective review of the case records and histopathologic findings, a total of 31 children (0.49%) were diagnosed with NET of the appendix and included in the further analysis. The primary outcome of this study was the incidence and treatment outcome of pediatric patients diagnosed with NET of the appendix. Secondary outcomes included the patients' demographic, clinical, and laboratory data and the histopathologic characteristics of tumor species. The overall incidence of NETs of the appendix was stable over the study years, with minor fluctuations. The median age of patients was 14 (interquartile range-IQR: 12, 16) years, with a female predominance (64.5%). The majority of patients (96.8%) presented with acute abdominal pain and underwent appendectomy because acute appendicitis was suspected. Acute appendicitis was confirmed by histopathology in 18 (58%) cases. NETs of the appendix were not detected preoperatively in any of the patients. Among patients with confirmed acute appendicitis, most (n = 14; 77.8%) were found to have non-perforated acute appendicitis. In most children, the tumor was located at the tip of the appendix (n = 18; 58.1%), and the majority of tumors had a diameter of less than 1 cm (n = 21, 67.7%). The mitotic count (n = 25, 80.6%) and Ki-67 proliferation index (n = 23, 74.2%) were low in most patients, so most tumors were classified as NET G1 (n = 25, 80.6%), while NET G2 and NET G3 were found in four (12.9%) and two (6.5%) patients, respectively. All children were treated with appendectomy only. The median follow-up time was 54 (IQR: 24, 95) months. The incidence of appendiceal NET among pediatric patients is very low. NET occurs most frequently in adolescents, with a female predominance. Most tumors are less than 1 cm in diameter, located at the tip, and associated with non-perforated appendicitis. Appendectomy is the treatment of choice, and major surgery was not necessary in our cohort.

Review of surgical spproaches for posterior fossa tumors in pediatric population and illustrative cases

Introduction/Background: Posterior fossa tumors are common in the pediatric population, and various surgical approaches have been described to reach them based on their specific location. This paper aims to review the most common posterior fossa approaches in pediatric patients and present illustrative cases to provide insights into their successful application in specific clinical scenarios. Case report: Three cases are presented, each demonstrating a different approach and surgical outcome. Case 1 involved a telovelar approach for the resection of a medulloblastoma, resulting in complete removal without neurological deficits. Case 2 required a combination of telovelar and supracerebellar infratentorial approaches to achieve gross total resection of a pilocytic astrocytoma. In Case 3, an occipital transtentorial approach was utilized to access and completely resect an atypical teratoid rhabdoid tumor. Connclusion: The paper discusses the telovelar and transvermian approaches, the supracerebellar infratentorial approach, the transtentorial suboccipital approach, and intraventricular endoscopy as individual or combined approaches for posterior fossa tumors. The presented cases highlight the importance of tailoring the surgical approach to each patient and provide valuable insights for neurosurgeons managing similar clinical scenarios.

Frequency of Paediatric Oral and Maxillofacial Tumours: A Retrospective study

Background: Tumours in maxillofacial region can be classified in benign or malignant variety, they may also be of odontogenic and non-odontogenic origin. Surgical Intervention in childhood leads to many complications. The aim of this study was to determine the type and frequency of tumours in paediatric population in patients reported to Khyber College of Dentistry Peshawar. Methods. The admission record of total 80 child patients having maxillofacial tumours were reviewed over a period of 4 years and 5 months i.e., from January 2013 to May 2017 in the department of oral and maxillofacial surgery, Khyber College of Dentistry, Peshawar. Results: Out of 80 patients, 44 were males and 36 females. Mean age was 12.5±4.6. Most common age of presentation was 18 years. Most common tumour was vascular malformation followed by giant cell granuloma. Majority of the tumours were of benign nature. Conclusion: Vascular malformation and giant cell granuloma are the most common maxillofacial tumours in children in this region. Timely diagnosis is the key to avoid morbidity and mortality.

Frequency of Paediatric Oral and Maxillofacial Tumors:A retrospective Study

Background and objectives: Tumors in maxillofacial region can be classified in benign or malignant variety, they may also beof odontogenic and non-odontogenic origin. Surgical Intervention in childhood leads to many complications. The aim of thisstudy was to determine the type and frequency of tumors in pediatric population in patients reported to Khyber College ofDentistry Peshawar.Methods: The admission record of total 80 child patients having maxillofacial tumors were reviewed over a period of 4 yearsand 5 months i.e., from January 2013 to May 2017 in the department of oral and maxillofacial surgery, Khyber College ofDentistry, Peshawar.Results: Out of 80 patients, 44 were males and 36 females. Mean age was 12.5±4.6. Most common age of presentation was18 years. Most common tumor was vascular malformation followed by giant cell granuloma. Majority of the tumors were ofbenign nature.Conclusion: Vascular malformation and giant cell granuloma are the most common maxillofacial tumors in children in thisregion. Timely diagnosisis the key to avoid morbidity andmortality

Survival Outcomes of Pancreatic Tumors in Pediatric Population: Analysis of NCDB and SEER Databases

Survival Outcomes of Pancreatic Tumors in Pediatric Population: Analysis of NCDB and SEER Databases

Hepatobiliary mucinous cystadenoma in a two year old

Mucinous cystadenomas of hepatobiliary (MCAHb) are rare and generally benign intrahepatic cystic neoplasms, only few pediatric cases were found in the literature. These lesions are clinical presentation differ significantly so they can mimic other liver lesions. In this case report, we discuss a 2-year-old girl who presented with abdominal pain and distended abdomen, for which she had an abdominal ultrasound (US) and Computerized tomography (CT) were objectived cystic lesion lobe multi-loculated with regular borders in the left hepatic, the diagnostic was a hydatid cyst.The histopathological exam, after a laparotomy, revealed a biliary mucinous cystadenoma. Herein, we review the current literature on biliary mucinous cystic neoplasms in pediatric population. The diagnosis of MCAHb is made on several parameters radiological, biochemical, serological and histological.

Complications of Endoscopic Skull Base Surgery for Sellar and Parasellar Tumors in Pediatric Population; Neurosurgical Perspectives.

BackgroundAdvances in surgical techniques based on in-depth anatomical knowledge of the skull base have broadened the indications for endoscopic skull base surgery (ESS) with the advantage of wide and direct surgical exposure while minimizing invasiveness. However, the low incidence of the indicated diseases and narrow surgical corridors in children have limited the popularization of ESS. In addition, surgical complications and preventive interventions are not yet well known. Therefore, we retrospectively investigated the complications and prevention methods of ESS in children with a comprehensive review.MethodsWe retrospectively analyzed the medical records of pediatric patients who underwent ESS for sellar and parasellar tumors at Seoul National University Children’s Hospital from July 2010 to December 2020. Visual and endocrine status, extent of resection, complications, and recurrences were investigated depending on the pathology of the tumor. In addition, a comprehensive literature review regarding the complications of pediatric ESS was performed.ResultsA total of 98 patients were enrolled. The median age of the patients was 12 years, and 52 patients were male. Preoperative visual disturbance was found in 53 patients, anterior pituitary function deficit in 69, and diabetes insipidus in 32. Gross total resection was attempted in 67 patients and achieved in 62 (93%). Biopsy and cyst fenestration were the goals of surgery in 26 patients, and all were achieved as planned. Regarding outcomes, visual disturbance worsened in two patients (2%), endocrine status was aggravated in 34 (35%) patients, and new-onset diabetes insipidus occurred in 27 (41%) patients. The overall surgical complication rate (other than aggravation of visual or endocrine status) was 17%. Postoperative meningitis (12%) was the most common complication, followed by cerebrospinal fluid leakage (2%), vasospasm, hemorrhage and infarction. By pathological diagnosis, craniopharyngioma had the highest complication rate of 29%. All but one patient with postoperative hemorrhage showed no permanent deficits.ConclusionESS in children is feasible and relatively safe. More attention and different postoperative management protocols are required in children to avoid complications, especially in craniopharyngiomas. However, the complications can be mostly managed conservatively without permanent neurologic deficits.

A 17-year experience on bilaterality of ovarian germ cell tumors in pediatric population and its clinical implications: A single-center study

BackgroundSince the incidence of ovarian germ cell tumor (GCT) in pediatric population is low, there is little information regarding this tumor. A synchronous and metachronous bilateral mass is not infrequently observed in GCT patients. It is important to prevent early ovarian failure and preserve fertility. MethodsWe retrospectively reviewed the medical records of 112 patients less than 18 years surgically treated for ovarian GCT between 2008 and 2021 at the Department of Pediatric surgery of the Seoul National University Hospital (SNUH). Among these, 13 patients (11.6%) had bilateral masses. In metachronous masses, the recurrence period and therapeutic approach were evaluated. When ovarian failure was a concern, fertility preservation was attempted. ResultsMean age was 8.1 ± 4.0 years. Patients with a bilateral mass were older (p = 0.004). Follow-up was 39.6 ± 40.6 months. Five patients had a synchronous mass, and 8 patients experienced metachronous recurrence. Two patients with a synchronous mass had a metachronous neoplasm (n = 1) and recurrence (n = 1). Patients with metachronous recurrence started menstruation except for two patients who had not reached puberty. The mean recurrence interval was 45.9 ± 38.6 months. Ovum banking was requested for 6 patients and performed in 2. ConclusionsDuring our 17 years of experience, bilaterality was observed in 11.6% of GCT patients. Metachronous mass occurs after 5 years. A longer follow-up plan is needed for early detection. Our multidisciplinary approach to preserve fertility seemed to be successful with close cooperation with gynecologists.

Parotid Tumors in Pediatric Population: 10 Years of Experience

Parotid Tumors in Pediatric Population: 10 Years of Experience

Intramedullary Spinal Cystic Lesions Mimicking Cavernoma with Spontaneous Myelum Hemorrhage in Children: A Case Report

Background: Intramedullary spinal masses is a rare yet devastating and challenging. One of the biggest difficulty is to reveal the mass type and feature, thus determine the definitive treatment. Despite its difficulties, many controversies persist regarding diagnosis and management. 
 Case Presentation: We report a case of 6 years old female came with gradual right limb weakness for 1 week before admission. It preceded by neck stiffness and for 2 weeks ago. Radiological examination revealed intradural intramedullary mass suggesting a cavernoma at VC1-C2 and VTh12-L1 level. The histopathological results shows unspecified hematoma.
 Conclusions: Intramedullary tumors in pediatric population is rare and can mimic any other mass lesion. MRI is the mainstay diagnostic tool of this patient. Complete surgical resection is the main goal of treatment, but the histopathologic features is the most important predictor of the functional outcome.

Values of the apparent diffusion coefficient in the differential diagnosis of tumors of the posterior fossa in the pediatric population of Colombia

Introduction: Posterior fossa tumors are common in the pediatric population and require adequate characterization by means of structural magnetic resonance imaging (MRI) and advanced MRI techniques to achieve an appropriate therapeutic approach. Objectives: To determine the usefulness of apparent diffusion coefficient (ADC) values for the differential diagnosis of posterior fossa tumors in the pediatric population treated at a reference hospital in Bogotá D.C., Colombia. Materials and methods: Diagnostic accuracy study carried out in 28 pediatric patients diagnosed with posterior fossa tumor between 2017 and 2019 at the Fundación Hospital de la Misericordia, a quaternary care institution. ADC values were measured and compared with histopathological diagnosis as gold standard, obtaining sensitivity, specificity, and positive and negative predictive values. Differences between medians were determined using the Kruskall-Wallis test. The p value between the quantitative ADC value and the gold standard was calculated using Pearson's chi-squared test, with a significance level of p<0.05. Results: The mean age of the participants was 83.9 months (SD=57 months), and 64.28% of them were boys. Medulloblastoma was the most frequent tumor (39.29%). For the diagnosis of medulloblastoma, an ADC value of 0.6210x10-³mm²/s was established, obtaining a sensitivity and specificity of 81.82% and 76.47%, respectively. For the diagnosis of pilocytic astrocytoma, an ADC of 1.03x10-³mm²/s was determined, with a sensitivity of 66.67% and a specificity of 89.40%. Conclusions: ADC value is useful to classify and differentiate posterior fossa tumors in the Colombian pediatric population, showing an inversely proportional relationship with the tumor grade.

Wilms or non-Wilms tumors? Imaging features of renal tumors in pediatrics

Introduction: Identifying the imaging features of renal tumors in pediatric population allows reaching more accurate diagnoses and implementing more appropriate treatments. Objective: To describe the imaging findings of renal tumors in children and to assess the association between imaging findings and histological diagnosis of Wilms tumors versus Non-Wilms tumors, and between imaging features and intraoperative rupture of Wilms tumors, as well as the level of agreement between radiological and histological diagnosis (Wilms vs. Non-Wilms tumor). Materials and methods: Cross-sectional study conducted in 47 children with a pathological diagnosis of kidney tumor and treated between 2012 and 2018 in a pediatric hospital in Bogotá D.C., Colombia. The patients’ medical records, as well as their ultrasound, tomography and magnetic resonance studies were reviewed. Two univariate logistic regression analyses were performed to assess the association between imaging findings and histopathological diagnosis and between imaging features and intraoperative rupture of Wilms tumors, calculating the respective Odds Ratio (OR) with a 95% confidence interval. In addition, the level of agreement between radiological and histological diagnosis was determined using the Cohen’s kappa coefficient. Results: A significant association was found between histological diagnosis of Wilms tumor and the presence of necrosis, tumor enhancement, pseudocapsule, rupture signs, tumor volume and tumor size (OR: 21.6, 15.17, 14.57, 8.21, 7.93, and 4.37, respectively; p<0.05). An association between having Wilms tumors and a lower frequency of metastases was also found (OR: 0.19; p<0.05). The kappa coefficient between radiological diagnosis of Wilms/non-Wilms tumors and histological diagnosis was 0.78 (CI95%: 0.59-0.96; p<0.05). Additionally, Wilms tumors volume was significantly associated with the occurrence of rupture (OR: 3.08; p<0.05). Conclusions: There are imaging findings such as necrosis, tumor enhancement and tumor volume that can help predict the histological diagnosis of Wilms tumors, as well as perioperative rupture. In addition, a moderate to very good concordance between radiological diagnosis of Wilms/non-Wilms tumors and histological findings was found.

Secretory carcinoma of salivary gland - A systematic review of pediatric case reports and case series.

Aim:Mammary analog secretory carcinoma (MASC) is a new pathological entity of salivary gland origin recognized as Secretory Carcinoma (SC) in the WHO 2017 classification. Pediatric cases of MASC were reviewed systematically from 2010 to 2019.Materials and Methods:Databases were searched from 2010 to 2019 for pediatric case reports and case series, excluding retrospective studies. A total of 12 manuscripts were reviewed for clinical, histological and immunohistochemical findings.Results:A total of 13 pediatric cases (11 case reports and 1 case series of 2 cases) of MASC in pediatric patients were found. The youngest reported age was 5 years. The common site was parotid gland usually presenting as a slowly growing firm, painless mass.Conclusion:MASC should be considered in the differential diagnosis of salivary gland tumors in pediatric population, especially from parotid gland. Extended research on such recent entities with more inputs from new cases reported in literature may outstretch the possibilities of therapeutic fusion inhibition in future.

SURGICAL AND SEIZURE TREATMENT OUTCOMES IN ADULT DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMORS: A CASE SERIES.

SUMMARYDysembryoplastic neuroepithelial tumors (DNETs) are benign neoplasms classified in the category of glioneuronal tumors. The estimated incidence of DNETs is 0.03 per 100,000 person per year with the age peak in a range between 10 and 14 years, and decreasing dramatically with increasing age. They are seldom diagnosed in persons above 20 years of age, being a cause of tumor-related intractable epilepsy that begins in childhood or adolescence. They have been proven to be the second most common type of epileptogenic tumors in pediatric population. These rare tumors cause chronic drug-resistant partial complex seizures with or without secondary generalization. Herein, we provide institutional case series of six adult patients with temporal lobe DNET presenting with complex partial seizures. Lesionectomy was performed with tumor resection in toto in three patients. In another three, partial resection was performed, whereas tumor remnant was left intact to avoid possible basal ganglia damage. All patients were seizure free postoperatively. Lesionectomy alone in temporal lobe epilepsy was associated with less favorable outcome than anterior temporal lobectomy. Total tumor removal is considered a major prognostic factor in most studies.

COVID -19 in children with cancer: a review

The World Health Organization (WHO) has announced that COVID -19 is a public health emergency requiring international concern. Children patients with cancer are highly vulnerable to this virus during SARS-CoV- 2 outbreak. These children have varying degrees of immunosuppression due to primary illness and also anti-tumor therapy. Although children in comparison to adults have a milder course of COVID -19 infection, pediatric patients with malignancies had a higher risk for severe clinical events due to COVID-19 in comparison to children without cancer. Most neoplasms in pediatric population have an aggressive manner and therefore require prolonged periods of multi agent chemotherapy. In adults with cancer who have stable disease, postponement of therapy is possible, but in children delay in onset of treatment is not a suitable option. One of the most effective strategies in order to prevent COVID -19 is minimizing the risk of exposure and also extreme isolation. Moreover, in the majority of children with cancer hospital admission is required. At present, there is no specific antiviral treatment for SARS -CoV - 2, hence the increased surveillance and preventive strategies in order to reduce the risk of exposure should be considered. There are many unanswered questions in management of children with cancer who were infected with COVID -19. In this setting, treatment decisions need to be individualized on a case-by-case basis and in order to deliver a suitable treatment, patient stratification is required. Pediatric patients with cancer should receive their anti-cancer treatment in the setting of vigorous screening for SARS -CoV - 2. Further studies are needed to understand the course of COVID -19 infection in children with cancer.

MON-LB016 Tyrosine Kinase Inhibitor Induced Hypothyroidism in Pediatric and Young Adult Population: An Institutional Review

Tyrosine Kinase Inhibitor Induced Hypothyroidism in Pediatric and Young Adult Population: An institutional reviewBackground:Tyrosine kinase inhibitors (TKIs) are a class of molecular targeted therapies approved for the treatment of several hematological and solid tumors in pediatric population. Thyroid dysfunction, most commonly primary hypothyroidism, is a well described adverse effect in adults. There is no available data in the pediatric population regarding the risk of thyroid dysfunction with the use of TKIs.Objective:To document the incidence of hypothyroidism in the pediatric and young adult patients on TKI therapy.Methods:A retrospective chart review including patients’ ≤ 21 years of age who had been treated with at least 1 of 10 predetermined TKIs for malignancy was performed. Demographics, TKI use and duration, thyroid hormone labs, and history of head/neck radiation were collected. We excluded patients with pre-existing thyroid disease prior to start of TKI therapy. Thyroid dysfunction was defined as TSH >5mcIU/mL during TKI therapy. Results:A total of 152 patients who were treated with TKIs for malignancy were identified. The mean age was 12.4 years (SD 6.5). About 20% of patients had therapy with multiple TKI drugs. A total of 24 patients were noted to have TSH elevation >5mcIU/ml of which 19 had a TSH >10 mcIU/mL or low free T4. Fourteen patients were started on levothyroxine. Average duration of TKI therapy prior to development of thyroid dysfunction was 6.7 months but over half developed hypothyroidism within 3 months of initiation of TKI therapy. Cabozantinib and pazopanib were responsible for 70% of TKI associated cases of thyroid dysfunction.Conclusion:This is the first report of incidence of primary hypothyroidism in pediatric and young adult patients treated with TKIs. Thyroid dysfunction can develop in the first few months of therapy and often is clinically significant. Early recognition and treatment of this complication will be important for patient care especially as use of these class of drugs increase.

Telovelar approach for microsurgical resection of an unusually located choroid plexus papilloma in the luschka foramen of an infant – Case report and review of literature

BackgroundChoroid plexus tumors are rare intraventricular tumors. Most of these tumors are benign choroid plexus papillomas. Most common location of these tumors in pediatric population is lateral ventricle although posterior fossa located tumors are also reported. Tumors located within luschka foramen extending to cerebellopontine angle are extremely rare in this age. So, there are little data in the literature regarding optimal management and surgical approach of these tumors within this challenging location. Case descriptionWe present an extremely rare case of choroid plexus papilloma of luschka foramen in an 8 months old boy extended laterally to cerebellopontine angle and pontomedullary junction. Tumor was resected successfully through telovelar approach without posing any injury to cerebellum, brainstem or cranial nerves. We also performed a review of limited literature available around different aspects of these tumors. ConclusionChoroid plexus papillomas of luschka foramen in pediatric population, although rare, can be effectively resected through telovelar approach without posing any neurological sequels to patient.

Long Noncoding RNA NHEG1 Drives β-Catenin Transactivation and Neuroblastoma Progression through Interacting with DDX5.

Recent studies suggest that long noncoding RNAs (lncRNAs) play essential roles in tumor progression. However, the functional roles and underlying mechanisms of lncRNAs in neuroblastoma (NB), the most common malignant solid tumor in pediatric population, still remain elusive. Herein, through integrating analysis of a public RNA sequencing dataset, neuroblastoma highly expressed 1 (NHEG1) was identified as a risk-associated lncRNA, contributing to an unfavorable outcome of NB. Depletion of NHEG1 led to facilitated differentiation and decreased growth and aggressiveness of NB cells. Mechanistically, NHEG1 bound to and stabilized DEAD-box helicase 5 (DDX5) protein through repressing proteasome-mediated degradation, resulting in β-catenin transactivation that altered target gene expression associated with NB progression. We further determined a lymphoid enhancer binding factor 1 (LEF1)/transcription factor 7-like 2 (TCF7L2)/NHEG1/DDX5/β-catenin axis with a positive feedback loop and demonstrated that NHEG1 harbored oncogenic properties via its interplay with DDX5. Administration of small interfering RNAs against NHEG1 or DDX5 reduced tumor growth and prolonged survival of nude mice bearing xenografts. High NHEG1 or DDX5 expression was associated with poor survival of NB patients. These results indicate that lncRNA NHEG1 exhibits oncogenic activity that affects NB progression via stabilizing the DDX5 protein, which might serve as a potential therapeutic target for NB.