ganglioneuroblastoma children

BACKGROUND: Peripheral neuroblastic tumors arise from the sympathoadrenal lineage of the neural crest. They have been classified according to the International Neuroblastoma Pathology Committee (INPC) into Four categories according to International Neuroblastoma Pathology Committee (INPC): a) Neuroblastoma (NB) b) Ganglioneuroblastoma (GNB), nodular c) Ganglioneuroblastoma, intermixed, and d) Ganglioneuroma (GN). Because of the rarity of extra-adrenal peripheral neuroblastic tumors, limited information is available regarding the chemotherapy of NB and GNB...

AIM:  Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare autoimmune disorder. Approximately half of the cases are associated with neuroblastoma in children. This study's aim is to review management of our cases with OMAS-associated neuroblastoma for treatment approach as well as long-term follow-up. METHODS:  Age at onset of symptoms and tumor diagnosis, tumor location, histopathology, stage, chemotherapy, OMAS protocol, surgery, and follow-up period were evaluated retrospectively in six patients between 2007 and 2022...

PURPOSE: To examine the potential of whole-tumor radiomics analysis of T2-weighted imaging (T2WI) in differentiating neuroblastoma (NB) from ganglioneuroblastoma/ganglioneuroma (GNB/GN) in children. MATERIALS AND METHODS: This study included 102 children with peripheral neuroblastic tumors, comprising 47 NB patients and 55 GNB/GN patients, which were randomly divided into a training group (n = 72) and a test group (n = 30). Radiomics features were extracted from T2WI images, and feature dimensionality reduction was applied...

BACKGROUND: To assess the prevalence of oral and maxillofacial malignant neoplasias in children and adolescents diagnosed through biopsies sent to the Oral Pathology Laboratory at the University of Sao Paulo School of Dentistry. MATERIAL AND METHODS: A retrospective analysis of anatomopathological reports on patients between 1 and 18 years old issued by the oral and maxillofacial pathology laboratory between 1997 and 2021 was performed for demographic data, lesion site, type of biopsy, diagnostic hypothesis and final diagnosis...

BACKGROUND: Neuroblastic tumors (NBTs) are the most common extra-cranial solid tumors of childhood. Neurofibromatosis type 1 (NF1) is the most common neurocutaneous disorder with a predisposition to tumors. The co-occurrence of NBTs in the setting of NF1 has been occasionally reported, suggesting a non-casual association and likely configuring a spectrum of neural crest-derived disorders. AIM OF THE STUDY: To explore the occurrence of NBTs within NF1 and to report on its natural history, therapeutic strategies, and outcomes in an Italian cohort of children with NF1 and in the literature...

BACKGROUND: Neuroblastoma is the most common malignancy in children younger than seven years of age and is the most frequent extracranial solid tumor that occurs in childhood. While opsoclonus-myoclonus syndrome (OMS), a paraneoplastic neurologic syndrome, affects 2-3% of children with neuroblastoma, and the percentage of mediastinal localization of the tumor is 49%. The objective of this study was to identify and characterize features of the OMS syndrome and treatments of mediastinal and non-mediastinal neuroblastoma associated with OMS...

BACKGROUND: Biopsies for diagnosis before chemotherapy is common in children with malignant solid tumors. Wound healing is delayed by chemotherapy; however, the ideal interval between biopsy and chemotherapy remains unknown. We aimed to summarize the relationship between chemotherapy timing and postoperative surgical complications. PROCEDURE: We retrospectively reviewed patients with malignant solid tumors who underwent chemotherapy after surgical biopsy at our institution between January 2014 and August 2020...

Objective: To investigate the clinicopathological characteristics and long-term survival outcomes of pediatric adrenal malignancies. Method: This study retrospectively analyzed children with pathologically confirmed pediatric adrenal malignancies from Surveillance, Epidemiology, and End Results Database from 2000 to 2019. Kaplan-Meier curve was used to assess the overall survival (OS) and cancer-special survival (CSS), and the Log-Rank method was used to calculate statistical differences...

OBJECTIVES AND METHODS: Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare neuroinflammatory disorder. We aimed to retrospectively evaluate clinical and laboratory data and outcomes of 23 children diagnosed with OMAS in two children's hospitals between 2010 and 2021. RESULTS: There were 14 boys and 9 girls aged 4-113 months, median 24 months. Ten (43.5%) children had paraneoplastic causes: neuroblastoma/ganglioneuroblastoma (n = 9), acute lymphoblastic leukemia (n = 1)...

BACKGROUND: International standardized criteria for eligibility, evaluable disease sites, and disease response assessment in patients with refractory, progressive, or relapsed high-risk neuroblastoma enrolled in early-phase clinical trials are lacking. METHODS: A National Cancer Institute-sponsored Clinical Trials Planning Meeting was convened to develop an international consensus to refine the tumor site eligibility criteria and evaluation of disease response for early-phase clinical trials in children with high-risk neuroblastoma...

Rapid-onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) and Congenital Central Hypoventilation Syndrome (CCHS) are ultra-rare distinct clinical disorders with overlapping symptoms including altered respiratory control and autonomic regulation. Although both disorders have been considered for decades to be on the same spectrum with necessity of artificial ventilation as life-support, recent acquisition of specific knowledge concerning the genetic basis of CCHS coupled with an elusive etiology for ROHHAD have definitely established that the two disorders are different...

BACKGROUND: Cervical neuroblastic tumors (NTs) are rare but less aggressive cancer with an above-average survival rate. Little has been published regarding the management and surgical outcomes of patients with cervical NTs based on pathology category. This study compared and identified the preoperative characteristics of cervical NTs in different pathology categories and evaluated the outcomes of patients undergoing surgical resection. MATERIALS AND METHODS: Upon the institutional review board's approval, a retrospective chart review was performed at Beijing Children's Hospital from April 2013 to August 2020...

Aims: The mediastinum is the second common site of origin of pediatric neuroblastic tumors. Primary thoracic neuroblastoma (NB) is considered to be a site with favorable outcome and is reported to have a better prognosis than the other sites of origin. This is an observational study on our experience in the management of children with primary thoracic neuroblastic tumors. Materials and Methods: A retrospective observational review of the medical records of all the children treated for primary thoracic neuroblastic tumors including NB, ganglioneuroblastoma, and ganglioneuroma over a period of 8 years from January 2011 to December 2018 at our Institute was performed...

Anti-Hu limbic encephalitis is a paraneoplastic syndrome in adults. In children, rare cases of anti-Hu limbic encephalitis were reported mostly without underlying tumors and clinical outcome are usually severe. Here, we describe a 4 year-old girl who developed cerebellar syndrome with abnormal behavior. The brain MRI showed several T2/FLAIR bilateral hyperintensities and auto-immune assessment showed positive anti-Hu antibodies. CT-scan revealed ganglioneuroblastoma which was surgically removed 3 months after onset...

Objectives: Spinal cord tumors are rare in children, mostly presented with unspecific symptoms that might pose a problem due to their possible malignancy and further complications. However, there are limited data on spinal cord lesions in Iran. This study aimed to present a series of 37 cases of primary spinal tumors treated at the same institution and briefly review their pathology, symptoms, and site of occurrence. Materials & Methods: In this study, 37 cases of spinal cord tumors and masses were selected within March 2007-2017, excluding spinal dysraphism...

AIMS: Risk stratification as per the International Neuroblastoma Risk Group (INRG) stratification is important for management of neuroblastoma. INRG incorporates various parameters including histological category as per the International Neuroblastoma Pathology Classification (INPC) and MYCN amplification, which were evaluated in fine needle aspiration biopsy (FNAB) samples of neuroblastoma patients to ascertain their impact in our population. METHODS: This was a retrospective study including 60 neuroblastoma cases diagnosed on FNAB, staged and stratified by INRG...

Peripheral neuroblastic tumours of neural crest origin are the most frequent solid neoplasms outside the CNS in children. Neuroblastoma/ganglioneuroblastoma/ganglioneuroma have a natural evolution of histological differentiation over time. Together with mitosis-karyorrhexis index and patient age (International Neuroblastoma Pathology Classification criteria), ganglion cell maturation determines grading and prognosis. Maturation presently is usually assessed only histologically. Immunocytochemical tissue markers defining neuroblast maturation in fetal CNS were here applied to peripheral neuroblastic tumours arising in the adrenal medulla or sympathetic chain...

Neuroblastoma (NB) is one of the most common extracranial malignant solid tumors in childhood, and over 90% of NBs are diagnosed in children under the age of 10 years old. For patients between 14 and 18 years old or older than 18 years, due to the rarity of NB, few studies have been performed in this population. Defined "adolescent cases" as individuals in 14-18 years old and "adult cases" as older than 18 years old, we reported five NB cases of adolescents and adults in our hospital. 137 cases presented a review of published literature on this topic...

BACKGROUND: Neuroblastoma is a common extracranial malignant tumor in children. Its main treatment modality is a combination of chemotherapy, radiotherapy, and surgery. Given the advances in chemotherapy regimens and the widespread use of bone marrow transplantation over the decades, there has been improvement in treatment efficacy, which has led to prolonged patient survival. Accordingly, long-term complications have become a growing concern among physicians and patients. This study aimed to analyze the survival rate of patients with neuroblastoma and the risk factors for developing second malignant neoplasms (SMNs)...

OBJECTIVE: To explore the value of CT-based radiomics in differential diagnosis of retroperitoneal neuroblastoma (NB) and ganglioneuroblastoma (GNB) in children. METHODS: A total of 172 children with NB and 48 children with GNB were assigned into the training set and testing set at the ratio of 7∶3 using a random stratified sampling method. Radiomics features were extracted and selected from non-enhanced and post-enhanced CT images. Based on the subset of optimal features, a multivariate regression model was used to establish the radiomics models for each phase and the combined radiomics models...