keyword
https://read.qxmd.com/read/38491421/clinical-features-and-treatment-options-for-pediatric-adrenal-incidentalomas-a-retrospective-single-center-study
#1
JOURNAL ARTICLE
Xiaojiang Zhu, Saisai Liu, Yimin Yuan, Nannan Gu, Jintong Sha, Yunfei Guo, Yongji Deng
BACKGROUND: The aim of this study was to investigate the clinical features and treatment options for pediatric adrenal incidentalomas(AIs) to guide the diagnosis and treatment of these tumors. METHODS: The clinical data of AI patients admitted to our hospital between December 2016 and December 2022 were collected and retrospectively analyzed. All patients were divided into neonatal and nonneonatal groups according to their age at the time of the initial consultation...
March 16, 2024: BMC Pediatrics
https://read.qxmd.com/read/38486945/adrenal-ganglioneuroblastoma-with-metastasis-near-the-renal-hilum-in-an-adult-female-a-case-report-and-review-of-the-literature
#2
Xinzhang Zhang, Yiwen Zhang, Dan Peng, Xin Shi, Zhuorui Zhang, Junfeng Wang, Xue Zhang, Jinjun Leng, Wei Li
Ganglioneuroblastoma (GNB), predominantly observed in children, is an uncommon malignant tumor in adults, with established treatment protocols notably lacking. The present study details the case of a 20-year-old woman who presented with a left adrenal gland mass, identified during a physical examination. Additionally, an unidentified mass was noted near the renal hilum in the preoperative evaluation. Following thorough preoperative preparation, both the primary adrenal gland mass and the renal hilar mass were surgically removed...
April 2024: Oncology Letters
https://read.qxmd.com/read/38474067/high-titer-anti-zscan1-antibodies-in-a-toddler-clinically-diagnosed-with-apparent-rapid-onset-obesity-with-hypothalamic-dysfunction-hypoventilation-and-autonomic-dysregulation-syndrome
#3
Vlad Tocan, Akari Nakamura-Utsunomiya, Yuri Sonoda, Wakato Matsuoka, Soichi Mizuguchi, Yuichiro Muto, Takaaki Hijioka, Masao Nogami, Daiki Sasaoka, Fusa Nagamatsu, Utako Oba, Naonori Kawakubo, Hiroshi Hamada, Yuichi Mushimoto, Pin Fee Chong, Noriyuki Kaku, Yuhki Koga, Yasunari Sakai, Yoshinao Oda, Tatsuro Tajiri, Shouichi Ohga
Severe obesity in young children prompts for a differential diagnosis that includes syndromic conditions. Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) syndrome is a potentially fatal disorder characterized by rapid-onset obesity associated with hypoventilation, neural crest tumors, and endocrine and behavioral abnormalities. The etiology of ROHHAD syndrome remains to be established, but recent research has been focusing on autoimmunity. We report on a 2-year-old girl with rapid-onset obesity during the first year of life who progressed to hypoventilation and encephalitis in less than four months since the start of accelerated weight gain...
February 29, 2024: International Journal of Molecular Sciences
https://read.qxmd.com/read/38417946/benign-paraspinal-ganglioneuroma-with-paraneoplastic-opsoclonus-myoclonus-syndrome
#4
JOURNAL ARTICLE
Haritha Prabaharan, Suryakala Chandrasekaran, Niharika Shetty, Praveen Nayak K
Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare immune-mediated movement disorder occurring as a paraneoplastic manifestation of neuroblastic tumours (NTs), especially neuroblastoma in infancy. Ganglioneuroma (GN), the benign tumour in the spectrum, is rarely associated with OMAS. We report the case of a child in her second year of life presenting with acute onset of progressive paraplegia and OMAS. MRI showed diffuse and infiltrating left paraspinal mass from T3-T9 levels with differentials of neuroblastoma or ganglioneuroblastoma...
February 27, 2024: BMJ Case Reports
https://read.qxmd.com/read/38350091/coexistence-of-langerhans-cell-histiocytosis-and-ganglioneuroblastoma-revealed-by-18f-fdg-pet-ct-in-a-pediatric-patient
#5
JOURNAL ARTICLE
Yanfeng Xu, Xia Lu, Wei Wang, Jigang Yang
Coexistence of Langerhans cell histiocytosis and ganglioneuroblastoma is rare and seldom reported in the literature. A 3-year-old girl with Langerhans cell histiocytosis underwent 18F-FDG PET/CT imaging for staging, which demonstrated significant 18F-FDG accumulation in the mandibles. Unexpectedly, a mild hypermetabolic soft mass was detected in the upper retroperitoneum. Results of surgical pathology of the abdominal mass were consistent with ganglioneuroblastoma.
February 2, 2024: Clinical Nuclear Medicine
https://read.qxmd.com/read/38190263/a-geospatial-assessment-of-industrial-releases-and-pediatric-neuroblastic-tumours-at-diagnosis-a-retrospective-case-series
#6
JOURNAL ARTICLE
Domenica Tambasco, Meredith Franklin, Shelley A Harris, Pauline Tibout, Andrea S Doria
Environmental risk factors associated with malignancy of pediatric neuroblastic tumours are not well-known and few studies have examined the relationship between industrial emissions and neuroblastic tumour diagnosis. A retrospective case series of 310 patients was evaluated at a tertiary hospital in Toronto, Canada between January 2008, and December 2018. Data from the National Pollutant Release Inventory (NPRI) were used to estimate exposure for a dozen chemicals with known or suspected carcinogenicity or embryotoxicity...
January 8, 2024: Archives of Environmental & Occupational Health
https://read.qxmd.com/read/38140886/intracranial-leptomeningeal-cns-ganglioneuroblastoma-first-report-and-review-of-the-literature
#7
JOURNAL ARTICLE
Daniel De Frutos Marcos, Mónica Rivero-Garvía, Javier Marquez-Rivas, Maria Jose Mayorga-Buiza, Ainhoa Casajús Ortega, Laura Ciércoles Ramírez
BACKGROUND: CNS ganglioneuroblastoma in an extremely rare embryonal tumour, specifically in the pediatric population. Bad prognosis is documented due to aggressiveness and absence of protocolized treatment at the moment. CLINICAL DESCRIPTION: We present the case of a 5-year-old boy who presented with sudden loss of consciousness. CT scan was performed showing a large posterior fossa lesion with several intraventricular focal lesions, suggesting metastases, the largest one located inside the III ventricle...
December 23, 2023: British Journal of Neurosurgery
https://read.qxmd.com/read/38028045/a-needle-in-the-haystack-an-unusual-case-presentation-of-ganglioneuroblastoma-at-a-tertiary-care-center-in-coastal-karnataka
#8
Shalini Radhakrishnan, Ranjitha Rao, Harsha Prasada Lashkari, Hema Kini, Jyoti Ramnath Kini, Vatsala Basavaraju Kudurugundi, Vanishree Ashok, Chaithra Venkataramana Gowthuvalli
KEY CLINICAL MESSAGE: This case report highlights the importance of recognizing and accurately diagnosing ganglioneuroblastoma, an uncommon variant of neuroblastic tumors in children. Ganglioneuroblastomas have diverse clinical and morphological presentations, and histopathological examination is paramount in guiding treatment decisions, especially in cases with ambiguous symptoms. Early detection is crucial, as the prognosis varies significantly based on the subtype and the presence of metastatic disease...
November 2023: Clinical Case Reports
https://read.qxmd.com/read/38001727/thoracoscopy-for-pediatric-thoracic-neurogenic-tumors-a-european-multi-center-study
#9
JOURNAL ARTICLE
Jean François Lecompte, Sabine Sarnacki, Sabine Irtan, Christian Piolat, Aurélien Scalabre, Isabelle Talon, Julien Rod, Nicoleta Panait, Gregory Rodesch, Ana Lourdes Luis Huertas, Olivier Abbo, Martine Demarche, Edouard Habonimana, Quentin Ballouhey, Dominique Valteau-Couanet, Florent Guérin
OBJECTIVES: To assess the efficacy of thoracoscopy and the outcome for children with thoracic neurogenic tumors. METHODS: We performed a retrospective review of 15 European centers between 2000 and 2020 with patients who underwent thoracoscopy for a neurogenic mediastinal tumor. We assessed preoperative data, complications, and outcomes. Results were expressed with the median and range values. RESULTS: We identified 119 patients with a median age of 4 years old (3 months-17 years)...
November 18, 2023: Cancers
https://read.qxmd.com/read/37991019/n6-methyladenosine-mediated-overexpression-of-long-noncoding-rna-adamts9-as2-triggers-neuroblastoma-differentiation-via-regulating-lin28b-let-7-mycn-signaling
#10
JOURNAL ARTICLE
Yun Liu, Jun Zhang, Fang Cao, Xiaobao Dong, Jie Li, Yanna Cao, Zhanglin Li, Yan Guo, Jie Yan, Yuanyuan Liu, Qiang Zhao
Neuroblastomas have shed light on the differentiation disorder that is associated with spontaneous regression or differentiation in the same tumor at the same time. Long noncoding RNAs (lncRNAs) actively participate in a broad spectrum of biological processes. However, the detailed molecular mechanisms underlying lncRNA regulation of differentiation in neuroblastomas remain largely unknown. Here, we sequenced clinical samples of ganglioneuroma, ganglioneuroblastoma, and neuroblastoma. We compared transcription profiles of neuroblastoma cells, ganglion cells, and intermediate state cells; verified the profiles in a retinoic acid-induced cell differentiation model and clinical samples; and screened out the lncRNA ADAMTS9 antisense RNA 2 (ADAMTS9-AS2), which contributed to neuroblastoma differentiation...
November 22, 2023: JCI Insight
https://read.qxmd.com/read/37929737/late-relapse-in-neuroblastoma-case-report-and-review-of-the-literature
#11
Rejin Kebudi, Begum Koc, Banu Oflaz Sozmen
BACKGROUND: Neuroblastoma is the most common extra-cranial solid tumor in children. The survival rate of relapsed/refractory neuroblastoma is dismal. Late recurrence may occur rarely. CASE PRESENTATION: We have, herein, presented a case with stage IV neuroblastoma who relapsed after 11 years and had a subsequent relapse after 15 years from the initial diagnosis, and reviewed cases with late relapsed (after >5 years) neuroblastoma in the literature. The case presented with recurrent disease at the T7 vertebra after 11 years from the initial diagnosis...
October 27, 2023: Current Pediatric Reviews
https://read.qxmd.com/read/37895355/chronic-diarrhea-caused-by-vasoactive-intestinal-peptide-secreting-tumor
#12
Oana Belei, Diana-Georgiana Basaca, Elena Rodica Heredea, Emil Radu Iacob, Laura Olariu, Roxana Folescu, Andrei Gheorghe Marius Motoc, Anda-Maria Nanu, Otilia Mărginean
VIPomas are a type of neuroendocrine tumor that independently produces vasoactive intestinal peptide (VIP). VIPomas causing watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome are not frequently observed in adult patients without pancreatic ailments. However, in children, the occurrence of a VIPoma originating in the pancreas is exceedingly uncommon. Instead, WDHA syndrome is more commonly associated with neurogenic tumors that secrete VIP, often located in the retroperitoneum or mediastinum. Among infants, chronic diarrhea is a prevalent issue that often necessitates the attention of pediatric gastroenterologists...
September 27, 2023: Life
https://read.qxmd.com/read/37885116/norepinephrine-transporter-and-vesicular-monoamine-transporter-2-tumor-expression-as-a-predictor-of-response-to-131-i-mibg-in-patients-with-relapsed-refractory-neuroblastoma
#13
JOURNAL ARTICLE
Vandana Batra, Ajami Gikandi, Bruce Pawel, Daniel Martinez, M Meaghan Granger, Araz Marachelian, Julie R Park, John M Maris, Kieuhoa T Vo, Katherine K Matthay, Steven G DuBois
BACKGROUND: Prior studies suggest that norepinephrine transporter (NET) and vesicular monoamine transporter 2 (VMAT2) mediate meta-iodobenzylguanidine (MIBG) uptake and retention in neuroblastoma tumors. We evaluated the relationship between NET and VMAT2 tumor expression and clinical response to 131 I-MIBG therapy in patients with neuroblastoma. METHODS: Immunohistochemistry (IHC) was used to evaluate NET and VMAT2 protein expression levels on archival tumor samples (obtained at diagnosis or relapse) from patients with relapsed or refractory neuroblastoma treated with 131 I-MIBG...
January 2024: Pediatric Blood & Cancer
https://read.qxmd.com/read/37862564/laparoscopic-transperitoneal-adrenalectomy-for-adrenal-tumors-in-children-technical-considerations-and-surgical-experience
#14
JOURNAL ARTICLE
Ankur Mandelia, Sabaretnam Mayilvaganan, Prathibha B Naik, Pujana Kanneganti
Aims: This study aimed to review our surgical experience of laparoscopic adrenalectomy (LA) for adrenal masses in the pediatric age group. Materials and Methods: The electronic medical records of all patients younger than 18 years of age who underwent LA between 2016 and 2023 were retrospectively reviewed. Children with adrenal tumors localized to the site of origin without evidence of encasement of major vasculature or involvement of adjacent organs were considered for LA. Patients with diagnosis of malignant adrenal tumor on preoperative work-up underwent open adrenalectomy...
October 20, 2023: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://read.qxmd.com/read/37843625/musashi-2-msi2-promotes-neuroblastoma-tumorigenesis-through-targeting-myc-mediated-glucose-6-phosphate-dehydrogenase-g6pd-transcriptional-activation
#15
JOURNAL ARTICLE
Ping Jiang, Ting Zhang, Bin Wu, Xiaoqing Li, Mingpeng Fu, Banglao Xu
Neuroblastoma (NB) is the deadliest pediatric solid tumor due to its rapid proliferation. Aberrant expression of MYCN is deemed as the most remarkable feature for the predictive hallmark of NB progression and recurrence. However, the phenomenon that only detection of MYCN in the nearly 20% of NB patients hints that there should be other vital oncogenes in the progression of NB. Here, we firstly show that MSI2 mRNA is augmented by analyzing public GEO datasets in the malignant stage according to International Neuroblastoma Staging System (INSS) stages...
October 16, 2023: Medical Oncology
https://read.qxmd.com/read/37819531/-peripheral-neuroblastic-tumors-in-childhood
#16
REVIEW
Marit Bernhardt, Christian Vokuhl
Peripheral neuroblastic tumors represent the fourth-largest group of malignant tumors in childhood. The majority of these tumors are neuroblastomas, which can be classified into undifferentiated, poorly differentiated, and differentiating subtypes. In addition, peripheral neuroblastic tumors include ganglioneuroblastoma, a composite tumor composed of Schwannian cell stroma and neuroblasts as well as benign ganglioneuroma. In this overview, histopathological diagnostic criteria and grading systems, as well as common molecular alterations that are of prognostic and therapeutic significance, are discussed...
October 11, 2023: Pathologie (Heidelb)
https://read.qxmd.com/read/37794991/great-mimicker-a-case-of-occult-melanoma-displaying-ganglioneuroblastic-differentiation
#17
Paulina Skrzypkowska, Michał Kunc, Wojciech Biernat
Melanomas are known for their diverse morphological features, presenting a diagnostic challenge for pathologists. Uncommon variations of melanoma can exhibit distinct cytological and histomorphological characteristics, including ganglioneuroblastic differentiation. However, this phenomenon is extremely rare, with only a few documented cases. Here, we present a unique case of an occult metastatic melanoma with ganglioneuroblastic differentiation developing in a 76-year-old male. The diagnosis was based on histopathology, immunophenotyping, and molecular testing, which revealed SOX10 positivity and an NRAS mutation...
2023: Contemporary Oncology Współczesna Onkologia
https://read.qxmd.com/read/37790931/single-cell-transcriptome-sequencing-reveals-tumor-heterogeneity-in-family-neuroblastoma
#18
JOURNAL ARTICLE
Yunlong Zhang, Yue Ma, Qingqing Liu, Yifei Du, Liang Peng, Jianwu Zhou, Zhenzhen Zhao, Changchun Li, Shan Wang
Neuroblastoma(NB) is the most common extracranial solid tumor in childhood, and it is now believed that some patients with NB have an underlying genetic susceptibility, which may be one of the reasons for the multiplicity of NB patients within a family line. Even within the same family, the samples show great variation and can present as ganglioneuroblastoma or even benign ganglioneuroma. The genomics of NB is still unclear and more in-depth studies are needed to reveal its key components. We first performed single-cell RNA sequencing(sc-RNAseq) analysis on clinical specimens of two family neuroblastoma(FNB) and four sporadic NB cases...
2023: Frontiers in Immunology
https://read.qxmd.com/read/37763032/nonspecific-gastrointestinal-symptoms-as-the-first-sign-of-ganglioneuroblastoma-intermixed-case-report-and-literature-review
#19
REVIEW
Łukasz Lipiński, Justyna Lipińska, Maria Kowalczuk, Izabela Kopeć, Magdalena Maria Woźniak, Joanna Mitek-Palusińska, Małgorzata Mitura-Lesiuk
Nonspecific gastrointestinal symptoms remain a problem for pediatricians because, out of a thousand trivial cases, there are rare diseases that require in-depth diagnostics and extensive knowledge to identify them. These complaints may be caused by a neoplastic process. We present the case of a 5-year-old boy whose diagnostic pathway lasted about 3 months. He was admitted to hospital due to severe abdominal pain. Physical examination revealed a bloated, hard, and painful abdomen. In the standing X-ray, the features of intestinal obstruction were visualized...
September 21, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/37693287/the-prognostic-and-predictive-value-of-plasma-d-dimer-in-children-with-neuroblastoma-a-7-year-retrospective-analysis-at-a-single-institution
#20
JOURNAL ARTICLE
Zhen-Ni Wang, Yao Zhang, Jian Sun, Zhen-Zhen Zhao, Shan Wang, Chao Yang
PURPOSE: Elevated plasma D-dimer level is a poor prognostic factor for many solid tumors. However, limited research has been conducted on D-dimer in children with neuroblastoma (NB), and its clinical significance remains unclear. The present study investigated the clinical and prognostic significance of D-dimer in pediatric NB patients. METHODS: A retrospective analysis of all newly admitted NB patients was conducted from January 2014 to December 2020. Baseline clinicopathological features, preoperative laboratory parameters, and follow-up information were collected...
September 2023: Annals of Surgical Treatment and Research
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