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https://www.readbyqxmd.com/read/29067922/primary-adrenal-teratoma-an-unusual-tumor-challenges-in-diagnosis-and-surgical-management
#1
P Ramakant, C Rana, K R Singh, A Mishra
We report a rare case of a 25-year-old lady who presented with right hypochondriac pain associated with weakness for 3 months. Her hormonal evaluation was normal. Computed tomography scan revealed an adrenal mass for which a right open transperitoneal adrenalectomy was performed, and the lesion was diagnosed as mature teratoma on histopathological examination. Teratoma is an unusual neoplasm which arises from one or all the three germ layers. Extragonadal teratomas are rare in adults as compared to children and are mostly retroperitoneal in location, constituting only 4% of all primary teratomas...
October 23, 2017: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/29062578/immune-thrombocytopenia-in-a-child-with-neuroblastoma
#2
Hasan Tarkan Ikizoglu, Inci Ayan, Fatma Tokat, Tulay Tecimer, Gonca Topuzlu Tekant
Thrombocytopenia is a frequent finding in patients with solid tumors. It is usually caused by bone marrow infiltration or by myelosuppression due to anticancer therapy; however immune thrombocytopenia (ITP) associated with solid tumors is rare. Neuroblastoma is the most common extracranial solid tumor in children. Here we report the case of a two-year-nine-month-old patient with adrenal neuroblastoma who presented with ITP. Paraneoplastic ITP was considered in the differential diagnosis. Bone marrow infiltration and other causes of thrombocytopenia were excluded and the patient was treated with intravenous immunoglobulin and tumor resection...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/29058015/wnt-signaling-the-development-of-the-sympathoadrenal-paraganglionic-system-and-neuroblastoma
#3
REVIEW
Jürgen Becker, Jörg Wilting
Neuroblastoma (NB) is a tumor of the sympathoadrenal system arising in children under 15 years of age. In Germany, NB accounts for 7% of childhood cancer cases, but 11% of cancer deaths. It originates from highly migratory progenitor cells that leave the dorsal neural tube and contribute neurons and glial cells to sympathetic ganglia, and chromaffin and supportive cells to the adrenal medulla and paraganglia. Clinically, histologically and molecularly, NBs present as extremely heterogeneous, ranging from very good to very poor prognosis...
October 22, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28943972/small-non-coding-rna-abundance-in-adrenocortical-carcinoma-a-footprint-of-a-rare-cancer
#4
Srinivas V Koduru, Ashley N Leberfinger, Dino J Ravnic
BACKGROUND: Adrenocortical carcinoma (ACC) is a relatively rare, but aggressive type of cancer, which affects both children and adults. OBJECTIVE: Small non-coding RNAs (sncRNAs) play important roles and may serve as biomarkers for disease diagnosis, prognosis and treatment. METHODS: In our study, we sought to identify sncRNAs associated with malignant adrenal tumors. We obtained publicly available, small RNA sequencing data derived from 45 ACC and 30 benign tumors arising from the cortex of the adrenal gland, adrenocortical adenomas (ACA), and compared their sncRNA expression profiles...
2017: Journal of Genomics
https://www.readbyqxmd.com/read/28730220/verner-morrison-syndrome-literature-review
#5
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
https://www.readbyqxmd.com/read/28533356/loss-of-function-mutations-in-the-cables1-gene-are-a-novel-cause-of-cushing-s-disease
#6
Laura C Hernández-Ramírez, Ryhem Gam, Nuria Valdés, Maya B Lodish, Nathan Pankratz, Aurelio Balsalobre, Yves Gauthier, Fabio R Faucz, Giampaolo Trivellin, Prashant Chittiboina, John Lane, Denise M Kay, Aggeliki Dimopoulos, Stephan Gaillard, Mario Neou, Jérôme Bertherat, Guillaume Assié, Chiara Villa, James L Mills, Jacques Drouin, Constantine A Stratakis
The CABLES1 cell cycle regulator participates in the adrenal-pituitary negative feedback, and its expression is reduced in corticotropinomas, pituitary tumors with a largely unexplained genetic basis. We investigated the presence of CABLES1 mutations/copy number variations (CNVs) and their associated clinical, histopathological and molecular features in patients with Cushing's disease (CD). Samples from 146 pediatric (118 germline DNA only/28 germline and tumor DNA) and 35 adult (tumor DNA) CD patients were screened for CABLES1 mutations...
August 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28472487/11-oxygenated-androgens-are-biomarkers-of-adrenal-volume-and-testicular-adrenal-rest-tumors-in-21-hydroxylase-deficiency
#7
Adina F Turcu, Ashwini Mallappa, Meredith S Elman, Nilo A Avila, Jamie Marko, Hamsini Rao, Alexander Tsodikov, Richard J Auchus, Deborah P Merke
Context: Patients with 21-hydroxylase deficiency (21OHD) have long-term complications, resulting from poor disease control and/or glucocorticoid overtreatment. Lack of optimal biomarkers has made it challenging to tailor therapy and predict long-term outcomes. Objective: To identify biomarkers of disease control and long-term complications in 21OHD. Setting and Participants: Cross-sectional study of 114 patients (70 males), ages 2 to 67 years (median, 15 years), seen in a tertiary referral center...
August 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28469338/pheochromocytoma-in-a-child-without-hypertension-a-contribution-to-the-rule-of-10s
#8
Prasanta Kumar Tripathy, Kaumudee Pattnaik, Manjushree Nayak, Hiranya Kishor Mohanty
Pheochromocytoma (PCC) is a neuroendocrine tumor originating from chromaffin tissue in adrenal medulla. Its diagnosis and treatment are well defined in adults, but experience in children is limited. Children constitute only 10% of reported cases, the average age at presentation being 11 years. The most common presentation is sustained hypertension, which is absent in only 10% of children. We managed a 14-month-old female child with PCC, but she was not hypertensive. We report two unusual features, in this case, an extremely young age at presentation and a childhood case of nonhypertensive PCC contributing for "rule of 10s...
January 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28465480/clinical-and-biological-features-of-neuroblastic-tumors-a-comparison-of-neuroblastoma-and-ganglioneuroblastoma
#9
Wen-Guang He, Yu Yan, Wen Tang, Rong Cai, Gang Ren
Neuroblastoma (NB), ganglioneuroblastoma intermixed (GNBi) and ganglioneuroblastoma nodular (GNBn) are neuroblastic tumors that present with a wide range of symptoms and variable prognoses. We retrospectively reviewed the pretreatment clinical (age, sex and tumor stage) and biological (MYCN amplification; and levels of lactate dehydrogenase, ferritin and neuron-specific enolase) characteristics of 279 patients who were diagnosed with pathologically confirmed NB and GNB from January 2005 to December 2015. The median age at diagnosis increased with grade of differentiation (NB: 28...
June 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/28450075/congenital-adrenal-hyperplasia
#10
REVIEW
Selma Feldman Witchel
The congenital adrenal hyperplasias comprise a family of autosomal recessive disorders that disrupt adrenal steroidogenesis. The most common form is due to 21-hydroxylase deficiency associated with mutations in the 21-hydroxylase gene, which is located at chromosome 6p21. The clinical features associated with each disorder of adrenal steroidogenesis represent a clinical spectrum that reflect the consequences of the specific mutations. Treatment goals include normal linear growth velocity and "on-time" puberty in affected children...
October 2017: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/28445278/analysis-of-treatment-of-large-abdominal-malignancies-in-children-complicated-with-abdominal-compartment-syndrome-report-of-six-cases
#11
Zeng Le-Xiang, Wu Yao-Hao, Li Na, Qiu Rong-Lin, Zhou Jia-Jia, Jiang Wen-Li, Zhang Jie, Deng Xiao-Geng
To explore effective treatment of large abdominal malignancies in children complicated with abdominal compartment syndrome (ACS).Six children with large abdominal malignancies complicated with ACS were admitted to our department from January 2013 to January 2016, and the changes in their breathing, heart rate, oxygen saturation, abdominal circumference, bladder pressure, and urine output, as well as the treatment measures and outcomes, were retrospectively analyzed.The 6 children included 1 child with bilateral nephroblastoma, 1 child with abdominal alveolar rhabdomyosarcoma, 1 child with right ovarian malignant teratoma complicated with abdominal glioma, 1 child with abdominal malignant teratoma, 1 child with right nephroblastoma, and 1 child with left adrenal gland neuroblastoma...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28433999/circadian-plasma-cortisol-measurements-reflect-severity-of-hypercortisolemia-in-children-with-different-etiologies-of-endogenous-cushing-syndrome
#12
Amit Tirosh, Maya B Lodish, Charalampos Lyssikatos, Elena Belyavskaya, Georgios Z Papadakis, Constantine A Stratakis
BACKGROUND: The utility of circadian cortisol variation in estimating the degree of hypercortisolemia in different forms of endogenous Cushing syndrome (CS) has not been evaluated in children yet. METHODS: A retrospective cohort study, including children who underwent surgery due to CS (n = 115), was divided into children with a pituitary adenoma (Cushing disease) (n = 88), primary adrenal CS (n = 21), or ectopic adrenocorticotropin- or corticotropin-releasing hormone (ACTH-/CRH)-secreting tumors (n = 6)...
2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28383813/mibg-avidity-correlates-with-clinical-features-tumor-biology-and-outcomes-in-neuroblastoma-a-report-from-the-children-s-oncology-group
#13
Steven G DuBois, Rajen Mody, Arlene Naranjo, Collin Van Ryn, Douglas Russ, Derek Oldridge, Susan Kreissman, David L Baker, Marguerite Parisi, Barry L Shulkin, Harrison Bai, Sharon J Diskin, Vandana Batra, John M Maris, Julie R Park, Katherine K Matthay, Gregory Yanik
BACKGROUND: Prior studies suggest that neuroblastomas that do not accumulate metaiodobenzylguanidine (MIBG) on diagnostic imaging (MIBG non-avid) may have more favorable features compared with MIBG avid tumors. We compared clinical features, biologic features, and clinical outcomes between patients with MIBG nonavid and MIBG avid neuroblastoma. PROCEDURE: Patients had metastatic high- or intermediate-risk neuroblastoma and were treated on Children's Oncology Group protocols A3973 or A3961...
November 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28367616/management-of-neuroblastoma-icmr-consensus-document
#14
REVIEW
Deepak Bansal, Sidharth Totadri, Girish Chinnaswamy, Sandeep Agarwala, Tushar Vora, Brijesh Arora, Maya Prasad, Gauri Kapoor, Venkatraman Radhakrishnan, Siddharth Laskar, Tanvir Kaur, G K Rath, Sameer Bakhshi
Neuroblastoma (NBL) is the most common extra-cranial solid tumor in childhood. High-risk NBL is considered challenging and has one of the least favourable outcomes amongst pediatric cancers. Primary tumor can arise anywhere along the sympathetic chain. Advanced disease at presentation is common. Diagnosis is established by tumor biopsy and elevated urinary catecholamines. Staging is performed using bone marrow and mIBG scan (FDG-PET/bone scan if mIBG unavailable or non-avid). Age, stage, histopathological grading, MYCN amplification and 11q aberration are important prognostic factors utilized in risk stratification...
June 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28347316/trends-of-testis-sparing-surgery-for-pediatric-testicular-tumors-in-south-china
#15
Yun-Lin Ye, Qiu-Ming He, Fu-Fug Zheng, Sheng-Jie Guo, Fang-Jian Zhou, Zi-Ke Qin
BACKGROUND: Testis-sparing surgery is not popular in South China. This study aimed to investigate this procedure for pediatric testicular tumors. METHODS: Children with testicular benign tumors were retrospectively analyzed from January 2001 to June 2015 in the Sun Yat-sen University Cancer Center (SYSUCC) and the First Affiliated Hospital (SYSU-1st). Follow-up was performed until June 2016, and the proportions of TSS in the two hospitals during the different periods were compared...
March 27, 2017: BMC Surgery
https://www.readbyqxmd.com/read/28326217/a-rare-adrenal-mass-in-a-3-month-old-a-case-report-and-literature-review
#16
Ashish Garg, Elza Pollak-Christian, Navneetha Unnikrishnan
A three-month-old female infant presented with abdominal distention for 2 months. A large palpable mass in right upper quadrant was noted on physical exam. Abdominal ultrasound revealed a large heterogeneous mass with multiple cystic components. Mass was surgically excised and pathology was consistent with mature adrenal teratoma. Teratoma is a germ cell tumor mainly found in gonadal tissues. Occurrence of adrenal gland teratoma in children is very rare with less than 10 pediatric case reports in English literature...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28324046/characteristics-of-pediatric-vs-adult-pheochromocytomas-and-paragangliomas
#17
MULTICENTER STUDY
Christina Pamporaki, Barbora Hamplova, Mirko Peitzsch, Aleksander Prejbisz, Felix Beuschlein, Henri J L M Timmers, Martin Fassnacht, Barbara Klink, Maya Lodish, Constantine A Stratakis, Angela Huebner, Stephanie Fliedner, Mercedes Robledo, Richard O Sinnott, Andrzej Januszewicz, Karel Pacak, Graeme Eisenhofer
Context: Pheochromocytomas and paragangliomas (PPGLs) in children are often hereditary and may present with different characteristics compared with adults. Hereditary PPGLs can be separated into cluster 1 and cluster 2 tumors due to mutations impacting hypoxia and kinase receptor signaling pathways, respectively. Objective: To identify differences in presentation of PPGLs between children and adults. Design: A retrospective cross-sectional clinical study...
April 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28289431/management-issues-of-congenital-adrenal-hyperplasia-during-the-transition-from-pediatric-to-adult-care
#18
REVIEW
Jin-Ho Choi, Han-Wook Yoo
Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers...
February 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28246490/cystic-adrenal-lesions-focus-on-pediatric-population-a-review
#19
REVIEW
Mara Carsote, Adina Ghemigian, Dana Terzea, Ancuta Augustina Gheorghisan-Galateanu, Ana Valea
BACKGROUND AND AIM: The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults. GENERAL DATA: Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression...
2017: Clujul Medical (1957)
https://www.readbyqxmd.com/read/28091891/adrenal-teratoma-a-case-series-and-review-of-the-literature
#20
Eric J Kuo, Anthony E Sisk, Zhiming Yang, Jiaoti Huang, Michael W Yeh, Masha J Livhits
Adrenal teratomas are rare neoplasms and there is limited data on their surgical outcomes and long-term prognosis. We aimed to review our institutional experience and compare this to the existing literature on adrenal teratomas in adults and children. An institutional pathology database was searched for cases of adrenal teratoma (June 1956-July 2016). Clinical and imaging data were abstracted from the medical records and pathology slides were obtained for review. In addition, a PubMed search for "adrenal teratoma" from June 1952 to July 2016 was performed to identify reports of primary adrenal teratoma in patients of all ages...
June 2017: Endocrine Pathology
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