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Adrenal tumor children

Roberto Iglesias Lopes, Jessica Ming, Martin A Koyle, Ronald Grant, Adriana Fonseca, Armando J Lorenzo
OBJECTIVE: To describe the experience and technique of zero-ischemia laparoscopic-assisted partial nephrectomy at The Hospital for Sick Children, as an alternative to the traditional open approach for nephron sparing surgery in selected children with Wilms' tumors (WT). MATERIALS AND METHODS: Patients with diagnosis of WT treated with neoadjuvant chemotherapy and who underwent laparoscopic-assisted nephron-sparing surgery at the Hospital for Sick Children from 2012-2016 were identified and charts reviewed retrospectively...
October 6, 2016: Urology
Nabeel I Uwaydah, Alex Jones, Mahmoud Elkaissi, Zhongxin Yu, Blake W Palmer
Neuroblastoma (NB) is the most common extra-cranial solid tumor in children and the most common malignancy in infants, with complete resection being curative in low-stage disease. The previous standard of treatment for many abdominal NBs involving the adrenal gland had been open surgery; however, there have been numerous descriptions of the safety and feasibility of a laparoscopic approach to resect adrenal masses in the pediatric population in benign and malignant disease, including improved cosmetic results, decreased length of stay, decreased surgical morbidity, and comparable oncological outcomes to open surgery...
September 2014: Journal of Robotic Surgery
Ayla Güven, Seher Polat
OBJECTIVE: Testicular adrenal rest tumors (TART) have been frequently shown in adolescents and adults with 21-hydroxylase deficiency. There have been no reports of TART in children with 3β-hydroxysteroid dehydrogenase deficiency (HSD3β. METHODS: Biopsy proven TART was found at 31/12 years old in older brother while TART determined at 22 moths old in younger by USG. Hormonal and anthropometric measurements were performed during glucocorticoid and fludrocortisone treatment...
July 29, 2016: Journal of Clinical Research in Pediatric Endocrinology
Xia Xu, Consolato Sergi
Adrenal tumors are quite rare in infancy and childhood with the exception of neuroblastoma. In fact, adrenocortical tumors (ACT) account for only 0.2% of all malignant cancers in children and adolescents. According to a multicenter registry investigation, the median interval between first endocrine symptoms and the diagnosis of ACT is 5months, and death is seen in 38% of patients, who suffer from tumor progression following the diagnosis in about 2½years. The prognosis of pediatric ACC is poor with a 5-year event-free survival of 54%...
September 2016: Contemporary Clinical Trials
Z Su, Y Y Li, H M Ma, J Zhang, M L Du
OBJECTIVE: To summarize the characterization of ovarian adrenal rest tumors (OART) in children and adolescent females with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD). METHOD: We have diagnosed 4 cases of CAH 21-OHD with OART in the recent 5 years in pediatric endocrinology unit of the first affiliated hospital of Sun Yat-Sen University. Clinical characterizations were summarized, including symptoms, growth patterns and bone age advancements, serum steroid concentrations, imaging results and the follow-up data...
June 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Shizhen Zhu, A Thomas Look
Neuroblastoma, an important developmental tumor arising in the peripheral sympathetic nervous system (PSNS), accounts for approximately 10 % of all cancer-related deaths in children. Recent genomic analyses have identified a spectrum of genetic alterations in this tumor. Amplification of the MYCN oncogene is found in 20 % of cases and is often accompanied by mutational activation of the ALK (anaplastic lymphoma kinase) gene, suggesting their cooperation in tumor initiation and spread. Understanding how complex genetic changes function together in oncogenesis has been a continuing and daunting task in cancer research...
2016: Advances in Experimental Medicine and Biology
Suk-Bae Moon
Neuroblastoma is the most common extracranial solid tumor in children. Mild hypertension is a frequent symptom, presumably an effect of catecholamines that tumors release. Reported herein is the rare occurrence of severe hypertension and subsequent heart failure attributable to adrenal gland neuroblastoma. A 3-year-old boy presented with anterior chest wall protrusion. Physical examination revealed severe hypertension, and left-sided cardiac failure was evident by echocardiography. Catecholamine metabolite (norepinephrine) levels were increased in serum (>2,000 pg/mL) and in urine (1,350...
2016: International Medical Case Reports Journal
Jing He, Wei Zhong, Jixiao Zeng, Jinhong Zhu, Ruizhong Zhang, Fenghua Wang, Tianyou Yang, Yan Zou, Huimin Xia
Neuroblastoma is one of the most commonly diagnosed extracranial solid tumors in infancy; however, the etiology of neuroblastoma remains largely unknown. Previous genome-wide association study (GWAS) indicated that several common genetic variations (rs110419 A > G, rs4758051 G > A, rs10840002 A > G and rs204938 A > G) in the LIM domain only 1 (LMO1) gene were associated with neuroblastoma susceptibility. The aim of this study was to evaluate the correlation between the four GWAS-identified LMO1 gene polymorphisms and neuroblastoma risk in a Southern Chinese population...
April 19, 2016: Oncotarget
Zeynep Atay, Ediz Yesilkaya, Senay Savas Erdeve, Serap Turan, Leyla Akin, Erdal Eren, Esra Doger, Zehra Aycan, Zehra Yavas Abali, Aysşehan Akinci, Zeynep Siklar, Samim Ozen, Cengiz Kara, Meltem Tayfun, Erkan Sari, Filiz Tutunculer, Gulcan Seymen Karabulut, Gulay Karaguzel, Semra Cetinkaya, Halil Saglam, Aysun Bideci, Selim Kurtoglu, Tulay Guran, Abdullah Bereket
AIM: The causes of gonadotropin-independent precocious puberty are diverse, and often have overlapping clinical and biochemical features. With the exception of congenital adrenal hyperplasia (CAH), disorders that cause gonadotropin-independent precocious puberty (GIPP) are uncommon. The literature is devoid of any large-scale studies on the etiologic distribution of GIPP. The aim of this study was to determine the frequency of each etiology in a cohort of patients with GIPP (excluding those with CAH), and to evaluate the clinical and laboratory features of these patients...
May 2016: Journal of Clinical Endocrinology and Metabolism
Paula Keskitalo, Terhi Remes-Pakarinen, Paula Vähäsalo, Jaakko Niinimäki, Liisa Kröger
Chronic nonbacterial osteomyelitis is an autoinflammatory disease occurring mainly in children and adolescents, typically involving recurrent or persistent osteitic foci. The symptom is bone pain, possibly accompanied by soft tissue tenderness. Some patients exhibit symptoms of systemic inflammation. The. precise etiology of the disease is not known, but an imbalance of inflammatory and anti-inflammatory cytokines is presumed to play a role in the development of the disease. While an anti-inflammatory analgesic is in most cases sufficient to calm down the osteitis, the use of corticosteroids, anti- TNF-a inhibitors or bisphosphonates is required in some cases...
2016: Duodecim; Lääketieteellinen Aikakauskirja
J Hubertus, A Pohl, I Schmid, D von Schweinitz
INTRODUCTION: Tumors of the adrenal gland are rare in children younger than 24 months of age. While neuroblastomas are most important in this age group, adrenal hemorrhage and other tumors are sometimes difficult to distinguish. Harvesting biopsies is mandatory in these young patients to obtain information on molecular markers, namely, MYCN and 1p deletion. PATIENTS: Between 03/2012 and 10/2013, 11 patients younger than 24 months of age with suspicious adrenal tumors were operated on laparoscopically...
April 2016: Klinische Pädiatrie
Takahiro Tsutsumimoto, Paul Williams, Toshiyuki Yoneda
Neuroblastoma (NB), which arises from embryonic neural crest cells, is the most common extra-cranial solid tumor of childhood. Approximately half of NB patients manifest bone metastasis accompanied with bone pain, fractures and bone marrow failure, leading to disturbed quality of life and poor survival. To study the mechanism of bone metastasis of NB, we established an animal model in which intracardiac inoculation of the SK-N-AS human NB cells in nude mice developed osteolytic bone metastases with increased osteoclastogenesis...
November 2014: Journal of Bone Oncology
Jenna L Riis, Douglas A Granger, Cynthia S Minkovitz, Karen Bandeen-Roche, Janet A DiPietro, Sara B Johnson
RATIONALE: Neuroendocrine-immune regulation is essential for maintaining health. Early-life adversity may cause dysregulation in the neuroendocrine-immune network through repeated activation of the stress response, thereby increasing disease risk. OBJECTIVE: This paper examined the extent to which maternal psychological well-being moderates neuroendocrine-immune relations in children. METHODS: We used data from a laboratory-based study of mothers and their five-year old children (n = 125 mother-child pairs) conducted from 2011 to 2013 in Baltimore, Maryland...
February 2016: Social Science & Medicine
Bree R Eaton, Natia Esiashvili, Sungjin Kim, Briana Patterson, Elizabeth A Weyman, Lauren T Thornton, Claire Mazewski, Tobey J MacDonald, David Ebb, Shannon M MacDonald, Nancy J Tarbell, Torunn I Yock
BACKGROUND: Endocrine dysfunction is a common sequela of craniospinal irradiation (CSI). Dosimetric data suggest that proton radiotherapy (PRT) may reduce radiation-associated endocrine dysfunction but clinical data are limited. METHODS: Seventy-seven children were treated with chemotherapy and proton (n = 40) or photon (n = 37) radiation between 2000 and 2009 with ≥3 years of endocrine screening. The incidence of multiple endocrinopathies among the proton and photon cohorts is compared...
June 2016: Neuro-oncology
Asma Deeb, Hana Al Suwaidi, Salima Attia, Ahlam Al Ameri
UNLABELLED: Combined17α-hydroxylase/17,20-lyase deficiency is a rare cause of congenital adrenal hyperplasia and hypogonadism. Hypertension and hypokalemia are essential presenting features. We report an Arab family with four affected XX siblings. The eldest presented with abdominal pain and was diagnosed with a retroperitoneal malignant mixed germ cell tumour. She was hypertensive and hypogonadal. One sibling presented with headache due to hypertension while the other two siblings were diagnosed with hypertension on a routine school check...
2015: Endocrinology, Diabetes & Metabolism Case Reports
C Esposito, I Giurin, M Iaquinto, M Escolino, M C Salerno, G De Filippo, A Savanelli, A Settimi, B Cigliano
Minimally invasive approach to the adrenal gland was first reported in 1992. Since then, the experience with the laparoscopic technique for adrenal disease in children has been limited. We report our experience with minimally invasive adrenal surgery in children. Two young girls (2 and 4 years old) with a left adrenal mass were operated using minimally invasive surgery (MIS) in our Unit. Ultrasonography and MRI showed in the oldest a 2 x 3 cm adrenal mass, while in the youngest a 5.5 x 5 cm adrenal tumor was found...
December 2015: Minerva Pediatrica
Hana Turkova, Tamara Prodanov, Marek Maly, Victoria Martucci, Karen Adams, Jiri Widimsky, Clara C Chen, Alexander Ling, Electron Kebebew, Constantine A Stratakis, Tito Fojo, Karel Pacak
OBJECTIVE: Overall about 10 to 20% of pheochromocytomas/paragangliomas (PHEOs/PGLs) are metastatic, with higher metastatic potential observed in succinate dehydrogenase subunit B/fumarate hydratase (SDHB/FH)-related tumors. Due to the improved availability of biochemical and genetic testing and the frequent use of anatomical/functional imaging, there is currently a higher detection rate of metastatic PHEO/PGL. METHODS: A retrospective analysis of 132 patients (27 children, 105 adults) with metastatic PHEO/PGL diagnosed and treated from 2000 to 2014 was conducted...
March 2016: Endocrine Practice
A E Solovyov, V V Morgun, A P Paholchuk
Neuroblastoma the most common malignant tumor of childhood, which is often localized in the retroperitoneal space, mainly in the adrenal glands, paravertebral retroperitoneal space, at rare in the posterior mediastinum, in the neck, presacral area. First symptoms of neuroblastoma are nonspecific, mimic various diseases. In the following clinical manifestations depend on the localization of the tumor, stage presence and location of metastases. In the diagnosis of neuroblastoma using ultrasonography and computed tomography...
June 2015: Klinichna Khirurhiia
Min Kyung Yu, Mo Kyung Jung, Ki Eun Kim, Ah Reum Kwon, Hyun Wook Chae, Duk Hee Kim, Ho-Seong Kim
PURPOSE: In male patients with congenital adrenal hyperplasia (CAH), the presence of testicular adrenal rest tumors (TARTs) have been reported, however their prevalence and clinical manifestations are not well known. Untreated TARTs may lead to testicular structural damage and infertility. This study was conducted to investigate the prevalence of TARTs in male patients with CAH, and characterize the manifestations to identify contributing factors to TART. METHODS: Among 102 CAH patients aged 0-30 years, 24 male patients have been regularly followed up in our outpatient clinic at Severance Children's Hospital from January 2000 to December 2014...
September 2015: Annals of Pediatric Endocrinology & Metabolism
Linna Zhang, Kathleen Scorsone, Sarah E Woodfield, Peter E Zage
PURPOSE: Children with high-risk neuroblastoma have poor survival rates, and novel therapies are needed. We hypothesized that cabozantinib would be effective against neuroblastoma tumor cells and tumors in preclinical models via inhibition of receptor tyrosine kinase signaling pathways. METHODS: We determined neuroblastoma cell viability after treatment with cabozantinib alone and in combination with 13-cis-retinoic acid, topotecan, and temozolomide using MTT assays...
November 2015: Cancer Chemotherapy and Pharmacology
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