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Dan Lu, Jun Liu, Yuan Chen, Fei Chen, Hui Yang
RATIONALE: Ganglioneuroblastoma is usually located in the adrenal gland, retroperitoneal ganglia, or posterior mediastinum, but rarely occurs in the parapharyngeal space. PATIENT CONCERNS: A 4-year-old girl presented with complaint of progressive inspiratory dyspnea and dysphagia, accompanying left-side Horner's syndrome. DIAGNOSE: Computed tomography (CT) scan revealed a giant mass with irregular low density in left oropharyngeal and posterior pharyngeal wall...
March 2018: Medicine (Baltimore)
Chun-Ho Park, Nozomi Shiwa, Kazunori Kimitsuki, Takehito Kakizaki, Daisaku Watanabe
This case report describes a congenital ganglioneuroblastoma in a 38-day-old male Japanese Black calf. The cervical multinodular mass was present at birth and grew rapidly. The cut surface was pale gray-to-yellow and had a gelatinous appearance. Hemorrhagic cysts of various sizes were observed in the nodule. Histologically, the mass contained clusters of neuroblastic cells, ganglionic cells, and Schwann-like cells. Immunohistochemically, the ganglionic cells showed strong positivity for neuron-specific enolase, neurofilament, synaptophysin, and chromogranin A, whereas the Schwann-like cells strongly expressed S-100, glial fibrillary acidic protein, and vimentin...
March 9, 2018: Journal of Veterinary Medical Science
Ferro Nguyen, Ivan S Alferiev, Peng Guan, David T Guerrero, Venkatadri Kolla, Ganesh S Moorthy, Michael Chorny, Garrett M Brodeur
PURPOSE: Currently, <50% of high-risk pediatric solid tumors like neuroblastoma (NB), can be cured, and many survivors experience serious or life-threatening toxicities, so more effective, less toxic therapy is needed. One approach is to target drugs to tumors using nanoparticles, which take advantage of the enhanced permeability of tumor vasculature. EXPERIMENTAL DESIGN: SN38, the active metabolite of irinotecan (CPT-11), is a potent therapeutic agent that is readily encapsulated in polymeric nanoparticles (NPs)...
March 7, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Karoline van de Loo, Stefan Balzer, Colin R MacKenzie, Thomas M Boemers, Monika Ortmann, Jörg Schaper, Arndt Borkhardt, Hans-Jürgen Laws, Michaela Kuhlen
BACKGROUND: Mycobacterium tuberculosis (M. tuberculosis) disease is a generally well-known problem among immunocompromised adults and children. In pediatric oncology, only few cases of M. tuberculosis disease are reported so far. CASE PRESENTATION: We report a case of concomitant lymphnode tuberculosis in a 4-year-old German boy with relapsed ganglioneuroblastoma. 18 months after the initial diagnosis, relapse with new paravertebral lesions and new lesions in the left lower lobe of the lung and in the perihilar lymphnodes suspicious of metastases of the ganglioneuroblastoma were detected...
March 5, 2018: BMC Infectious Diseases
Farhan A Mirza, Brian Synder, Vanessa D Smith, Raul A Vasquez
Pediatric cerebral ganglioneuroblastoma is an exceedingly rare tumor. We describe the case of a four-year-old male presenting with sudden mental status decline who was found to have a large intracranial lesion with intraventricular extension. Management of the case and pathological findings are discussed, along with a review of literature on this rare entity.
February 26, 2018: World Neurosurgery
Pei-Sen Yao, Guo-Rong Chen, Huang-Cheng Shang-Guan, Qing-Song Lin, Xing-Fu Wang, Shu-Fa Zheng, De-Zhi Kang
RATIONALE: Intracranial ganglioneuroblastoma represents a rare subtype of primitive neuroectodermal tumor. Here, we report a hippocampal ganglioneuroblastoma and a literature review of cerebral anglioneuroblastoma is carried out. PATIENT CONCERNS: We report a 16-year-old male patient presenting with absence seizure and high-infiltration hippocampal ganglioneuroblastoma. INTERVENTIONS: Magnetic resonance imaging (MRI) indicates a space-occupying lesion with a well-defined margin in the right temporal lobe and hippocampus...
December 2017: Medicine (Baltimore)
Natasha Alexander, Katie Sullivan, Furqan Shaikh, Meredith S Irwin
BACKGROUND: Ganglioneuromas (GNs) usually demonstrate favorable histological and clinical features. Surgery is often performed due to clinical symptoms and/or theoretical concerns that GN may transform into neuroblastoma (NB); however, several studies have identified significant GN-surgical morbidities. OBJECTIVES: We compared the natural history, biological and clinical features of GN and ganglioneuroblastoma-intermixed (GNB-I) managed by surgery or observation to inform management and surveillance...
January 25, 2018: Pediatric Blood & Cancer
Michael Sirignano, Jonathan R Dillman, Brian D Weiss, Charles T Quinn, Bin Zhang, Weizhe Su, Andrew T Trout
BACKGROUND: Reticuloendothelial system MRI signal hypointensity is common in pediatric oncology patients with solid abdominal tumors. OBJECTIVE: To assess changes in liver, spleen and bone marrow T2-weighted MRI signal intensity over time and their relationship to blood transfusion history in children with solid abdominal tumors. MATERIALS AND METHODS: In this retrospective study we measured liver, spleen and bone marrow signal intensity on axial T2-weighted MR images obtained December 2009 through February 2016 in children with hepatoblastoma, neuroblastoma, ganglioneuroblastoma and Wilms tumor...
December 19, 2017: Pediatric Radiology
Signe Risum, Ulrich Knigge, Seppo W Langer
Survival of stage 4 ganglioneuroblastoma (GNB) patients is poor; no reports exist of patients surviving up to 5 years (1, 2). We report the clinical and therapeutic course of a patient with stage 4 GNB surviving beyond expectations due to a multimodal treatment approach incorporating new technologies in cancer diagnostic and treatment.
December 2017: Clinical Case Reports
Wendy G Mitchell, Franz Blaes
Cancer and autoimmunity come together in paraneoplastic syndromes (PNS), which reflect the remote, not direct, effects of cancer. In the pediatric population, a variety of PNS have been described, but the most common of these rare disorders are instigated by neuroblastic tumors, such as neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The main pediatric-onset neurological PNS are ROHHAD syndrome, anti-ANNA1 (anti-Hu), and opsoclonus-myoclonus syndrome. They manifest distinctive neurological features, which aid the diagnosis, though under-recognition still poses serious challenges and risks...
August 2017: Seminars in Pediatric Neurology
Min Zhang, Zhiteng Jiang, Sheng Chen, Zhixiang Wu, Kai Chen, Yeming Wu
Neuroblastoma (NB) is a highly malignant solid tumor in children. The cysteine endopeptidase legumain is expressed in adult solid tumors, but its expression in NB has not been examined. In this study, we assayed legumain expression in two NB cell lines and in microarrays of tumor tissues collected from 46 children with undifferentiated NB, differentiated NB and ganglioneuroblastoma. Correlation analyses showed that legumain was expressed in all NB cell lines tested, and that expression correlated with the degree of tumor differentiation...
October 10, 2017: Chemical Biology & Drug Design
Mikako Warren, Ryosuke Matsuno, Henry Tran, Hiroyuki Shimada
BACKGROUND: This study evaluated the utility of Phox2b in pediatric tumors. Previously tyrosine hydroxylase (TH) was the most widely utilized sympathoadrenal marker specific for neural crest tumors with neuronal/neuroendocrine differentiation. However, its sensitivity is insufficient. Recently Phox2b has emerged as another specific marker for this entity. METHODS: Phox2b IHC was performed on 159 pediatric tumors including (1) 65 neural crest tumors with neuronal differentiation [peripheral neuroblastic tumors (pNT)]: 15 neuroblastoma undifferentiated (NB-UD), 10 NB poorly differentiated (NB-PD), 10 NB differentiating (NB-D), 10 ganglioneuroblastoma intermixed (GNBi), 10 GNB nodular (GNBn), and 10 ganglioneuroma (GN); (2) 23 neural crest tumors with neuroendocrine differentiation [pheochromocytoma/paraganglioma (PCC/PG)]; (3) 27 other neural crest tumors including one composite rhabdomyosarcoma/neuroblastoma; and (4) 44 non-neural crest tumors...
October 7, 2017: Histopathology
Scott Deeney, Camille Stewart, Amanda L Treece, Jennifer O Black, Mark A Lovell, Timothy Garrington, Frederick Karrer, Jennifer Bruny
PURPOSE: The best method for diagnosing pediatric nonnephroblastoma solid intraabdominal tumors is unknown. We hypothesized that core needle biopsy (CNB) is noninferior to open wedge biopsy (OWB) for pathologic diagnosis. METHODS: We prospectively enrolled children aged 1day to 17years with radiographic evidence of nonnephroblastoma solid intraabdominal tumors scheduled for OWB from 5/2013 to 12/2015 at a single institution. Four 16-gauge CNBs were obtained, followed by OWB...
December 2017: Journal of Pediatric Surgery
James Lonie, Rachael Boles, Jason Boldery
No abstract text is available yet for this article.
September 18, 2017: ANZ Journal of Surgery
Simon Rajendran, Debra Jensen, W Glenn McCluggage
The development of a somatic neoplasm within an ovarian dermoid cyst (mature cystic teratoma) is a rare, but well described, phenomenon which occurs in approximately 1% of all cases. Any of the tissue components of a dermoid cyst has the potential to undergo neoplastic transformation with carcinoid tumors and squamous cell carcinomas being among the most common neoplasms. We report a case of a ganglioneuroblastoma arising within an ovarian dermoid cyst, an association which, as far as we are aware, has not been described previously...
September 29, 2017: International Journal of Gynecological Pathology
Austėja Juškaitė, Indrė Tamulienė, Jelena Rascon
BACKGROUND: Neuroblastoma (NB) is the most common extracranial solid tumour in children. This is a very rare disease with heterogeneous biology varying from complete spontaneous regression to a highly aggressive tumour responsible for 15% of malignancy-related death in early childhood. Analyses of survival rates in Europe have shown a considerable difference between Northern/Western and Eastern European countries. Treatment results of NB in Lithuania have never been analyzed. AIM: To assess the survival rate of children with NB according to initial spread of the disease, age at diagnosis, the MYCN amplification, risk group, and treatment period...
2017: Acta Medica Lituanica
Xiaofei Zhao, Hongming Zhuang
A series of I-MIBG scanning was performed in a 33-year-old woman with a history of ganglioneuroblastoma and a known left renal cyst. The initial studies did not reveal any MIBG activity in the renal cyst. However, increased MIBG activity was noted in the same renal cyst in the subsequent follow-up scans.
August 12, 2017: Clinical Nuclear Medicine
Sounak Gupta, Jun Zhang, Lori A Erickson
Ganglioneuromas represent the most well-differentiated spectrum of neoplasia arising from the sympathetic nervous system, while neuroblastomas represent the most poorly differentiated counterpart, and ganglioneuroblastomas represent intermediate stages of differentiation. Small series of cases have documented the co-occurrence of ganglioneuroma with a pheochromocytoma (Pheo)/paraganglioma (PGL) component. We report the clinicopathologic features of eight such cases, diagnosed between 2003 and 2015 with a mean follow-up of 22 months (1-47), which were evaluated for syndrome associations, SDHB expression, and clinical outcome...
September 2017: Endocrine Pathology
Shilpa Rao, R T Rajeswarie, T Chickabasaviah Yasha, Bevinahalli N Nandeesh, Arimappamagan Arivazhagan, Vani Santosh
INTRODUCTION: CNS embryonal tumors comprise a group of highly malignant neoplasms with a wide spectrum of histomorphological entities that includes Medulloblastoma (MB), Atypical Teratoid/Rhabdoid Tumor (AT/RT), Neuroblastoma (NB), Ganglioneuroblastoma (GNB), Embryonal Tumor with Multilayered Rosettes (ETMR), and the embryonal tumor-Not Otherwise Specified (NOS). The entity ETMR includes previously described histopathologic patterns-Embryonal Tumor with Abundant Neuropil and True Rosettes (ETANTR), Ependymoblastoma (EBL), and Medulloepithelioma (MEPL)...
November 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Oana Andreea Belei, Elena Rodica Heredea, Estera Boeriu, Tamara Marcela Marcovici, Simona Cerbu, Otilia Mărginean, Emil Radu Iacob, Daniela Iacob, Andrei Gheorghe Marius Motoc, Eugen Sorin Boia
Chronic diarrhea in infants is a common condition for addressability to pediatric gastroenterologists. The causes are multiple and the delay in reaching the final diagnosis can lead to complications in the general condition of the child. The purpose of this review is to present the bio-clinical and histogenetic particularities of a rare clinical entity, characterized by tumoral causes of chronic diarrhea. VIPomas are neuroendocrine tumors that autonomously secrete vasoactive intestinal peptide (VIP). Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by VIP-producing tumors only rarely occurs in adult patients with non-pancreatic disease...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
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