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Adrenal tumors

Evidio Domingo-Musibay, Paari Murugan, Alessio Giubellino, Sandeep Sharma, Daniel Steinberger, Jianling Yuan, Matthew A Hunt, Emil Lou, Jeffrey S Miller
BACKGROUND: Sebaceous carcinoma is an aggressive adnexal skin tumor with a predilection for the eyelids and sebaceous glands of the head and neck. CASE PRESENTATION: A 73 year-old man presented with confusion and was found to have widely disseminated sebaceous carcinoma with metastases to brain, lungs, liver, bowel, lymph nodes, and bone. Following initial treatment of the brain metastases with surgery he received post-operative radiosurgery. He then began systemic immunotherapy with pembrolizumab...
June 19, 2018: Journal for Immunotherapy of Cancer
Desmond A Brown, Nicholas L Deep, Colin L Driscoll, Michael J Link, Mark E Jentoft, David J Daniels
Epstein-Barr virus-associated smooth-muscle tumors are rare tumors seen in immunocompromised patients. Most cases occur in the context of AIDS and organ transplantation, and very rarely in the setting of congenital immunodeficiency, with only 5 case reports of the latter published so far in the literature. The authors report the case of a previously healthy 8-year-old girl with headaches and precocious puberty who was found to have a large skull base lesion. There was a synchronous left adrenal lesion. She underwent resection of the skull base lesion and a left adrenalectomy...
June 15, 2018: Journal of Neurosurgery. Pediatrics
Xisi Wang, Lijun Wang, Yan Su, Zhixia Yue, Tianyu Xing, Wen Zhao, Qian Zhao, Chao Duan, Cheng Huang, Dawei Zhang, Mei Jin, Xianfeng Cheng, Shenglan Chen, Yi Liu, Xiaoli Ma
To evaluate plasma cell-free DNA (cfDNA) as a promising biomarker for neuroblastoma (NB) tumor burden. Seventy-nine eligible patients with newly diagnosed NB were recruited from Beijing Children's Hospital between April 2016 and April 2017. Additionally, from September 2011 to June 2017, 79 patients with stable NB were evaluated with a median follow-up time of 21 months. Approximately 2 mL of peripheral blood was drawn upon enrollment, and plasma cfDNA levels were measured via quantitative polymerase chain reaction (qPCR)...
June 14, 2018: Cancer Medicine
Mario A Inchiosa
The principal finding from this study was the recognition that the α-adrenergic antagonist, phenoxybenzamine, possesses histone deacetylase inhibitory activity. Phenoxybenzamine is approved by the United States Food and Drug Administration for the treatment of hypertensive crises associated with tumors of the adrenal medulla, pheochromocytomas. It has several "off label" indications relative to its capacity to relax vascular smooth muscle and smooth muscle of the urogenital tract. The drug also has a long history of apparent efficacy in ameliorating, and perhaps reversing, the severe symptoms of neuropathic pain syndromes...
2018: PloS One
Kourosh Goudarzipour, Farzaneh Farahmandi, Ahmad Mohammadi, Reza Taherian
Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PNET) typically occurs in long or flat bones, soft tissues, or less often, solid organs. Ewing sarcoma/peripheral primitive neuroectodermal tumor arising from the adrenal gland is extremely rare, especially in children, and only limited cases are reported previously. Herein, we review a case of a 22-month-old girl who presented to our department with abdominal pain, bulging of the left flank, and a nonfunctioning adrenal lesion which was found to be an adrenal ES/PNET...
May 2018: Iranian Journal of Kidney Diseases
Manisha H Shah, Whitney S Goldner, Thorvardur R Halfdanarson, Emily Bergsland, Jordan D Berlin, Daniel Halperin, Jennifer Chan, Matthew H Kulke, Al B Benson, Lawrence S Blaszkowsky, Jennifer Eads, Paul F Engstrom, Paul Fanta, Thomas Giordano, Jin He, Martin J Heslin, Gregory P Kalemkerian, Fouad Kandeel, Sajid A Khan, Wajih Zaheer Kidwai, Pamela L Kunz, Boris W Kuvshinoff, Christopher Lieu, Venu G Pillarisetty, Leonard Saltz, Julie Ann Sosa, Jonathan R Strosberg, Craig A Sussman, Nikolaos A Trikalinos, Nataliya A Uboha, Jonathan Whisenant, Terence Wong, James C Yao, Jennifer L Burns, Ndiya Ogba, Griselda Zuccarino-Catania
The NCCN Guidelines for Neuroendocrine and Adrenal Tumors provide recommendations for the management of adult patients with neuroendocrine tumors (NETs), adrenal gland tumors, pheochromocytomas, and paragangliomas. Management of NETs relies heavily on the site of the primary NET. These NCCN Guidelines Insights summarize the management options and the 2018 updates to the guidelines for locoregional advanced disease, and/or distant metastasis originating from gastrointestinal tract, bronchopulmonary, and thymus primary NETs...
June 2018: Journal of the National Comprehensive Cancer Network: JNCCN
Pierluigi Giampaolino, Luigi Della Corte, Carmen Formisano, Lara Cuomo, Simone Maurea, Valeria Romeo, Giuseppe Bifulco
Pheochromocytoma (PH) is a tumor that arises from chromaffin cells of the adrenal medulla. Though being this benign neoplasm very rare in pregnancies, lack of treatment nevertheless causes high mortality rates for both the mother and the fetus. Classic symptoms related to PH are hypertension, abdominal pain, diaphoresis, and headache; but it can be easily misdiagnosed as gestational hypertension or preeclampsia. Its appearance is sporadic, but there are some genetic disorders that favor its onset (e.g. MEN 2A and 2B)...
June 12, 2018: Gynecological Endocrinology
Tina T Thomas, Sahiti Chukkapalli, Raelene A Van Noord, Melanie Krook, Mark J Hoenerhoff, Jonathan R Dillman, Elizabeth R Lawlor, Valerie P Opipari, Erika A Newman
Preclinical testing of anticancer therapies relies on relevant xenograft models that mimic the innate tendencies of cancer. Advantages of standard subcutaneous flank models include procedural ease and the ability to monitor tumor progression and response without invasive imaging. Such models are often inconsistent in translational clinical trials and have limited biologically relevant characteristics with low proclivity to produce metastasis, as there is a lack of a native microenvironment. In comparison, orthotopic xenograft models at native tumor sites have been shown to mimic the tumor microenvironment and replicate important disease characteristics such as distant metastatic spread...
May 25, 2018: Journal of Visualized Experiments: JoVE
Maria S Tretiakova
Eosinophilic solid and cystic renal cell carcinoma (ESC-RCC) is a novel entity of rare tumors with rather unique morphology and immunohistochemical profile. Until recently these tumors were characterized by indolent behavior. Herein we describe a series of six primary and metastatic ESC-RCCs morphologically and immunophenotypically mimicking epithelioid angiomyolipoma (eAML). Retrospective review of unclassified RCCs with oncocytic phenotype yielded several candidate cases, 4 of which fulfilled diagnostic criteria after additional work-up...
June 6, 2018: Human Pathology
Wei Yao, Kuiran Dong, Kai Li, Shan Zheng, Xianmin Xiao
OBJECTIVE: To investigate and compare long-term outcomes in children undergoing laparoscopic or open adrenalectomy for local adrenal neuroblastoma. METHODS: A retrospective review was conducted of 37 children with local adrenal neuroblastoma treated between January 2005 and December 2013 in our hospital. These patients met inclusion criteria for having adrenal neuroblastoma and undergoing operative resection. All patients were successfully followed up until December 2017...
June 7, 2018: Pediatric Surgery International
Mariana Fuertes, Julieta Tkatch, Josefina Rosmino, Leandro Nieto, Mirtha Adriana Guitelman, Eduardo Arzt
Cushing's disease (CD) is an endocrine disorder originated by a corticotroph tumor. It is linked with high mortality and morbidity due to chronic hypercortisolism. Treatment goals are to control cortisol excess and achieve long-term remission, therefore, reducing both complications and patient's mortality. First-line of treatment for CD is pituitary's surgery. However, 30% of patients who undergo surgery experience recurrence in long-term follow-up. Persistent or recurrent CD demands second-line treatments, such as pituitary radiotherapy, adrenal surgery, and/or pharmacological therapy...
2018: Frontiers in Endocrinology
John J Manov, Alexander Diaz-Bode, Russ Kuker
A 66-year old woman had a left breast mass. Biopsy showed invasive ductal carcinoma. A PET/CT scan demonstrated hypermetabolism in the left breast and atypical heterogeneously increased uptake throughout the skeleton as well as a minimally FDG-avid right adrenal myelolipoma. PET/CT 4 months later after 6 cycles of neoadjuvant chemotherapy demonstrated increased size and FDG avidity of this adrenal mass concerning for metastasis and uniformly increased skeletal FDG avidity. Biopsy demonstrated adrenal myelolipoma...
June 5, 2018: Clinical Nuclear Medicine
Anna Babinska, Mariusz Kaszubowski, Krzysztof Sworczak
Due to the fact that overweight or obesity is accompanied by hormonally active adrenal tumors: Cushing Syndrome-(CS) and Subclinical Cushing Syndrome (SCS), it is of high interest the correlation between different adipokines and cytokines secreted by adipose tissue, with metabolic disorders and hormonal activity in this group. Even in non-functioning adrenal incidentalomas (NFAI) elevated risk for cardiovascular disease and metabolic syndrome was demonstrated. The aim of the study was to investigate plasma adiponectin, leptin, resistin, tumor necrosis factor α (TNFα), interleukin 6 (IL6) and monocyte chemoattractant protein 1 (MCP1) levels in patients with NFAIs and healthy subjects...
June 5, 2018: Endocrine Journal
Rémy Liechti, Lana Fourie, Stefan Fischli, Jürg Metzger
Lymphangiomas (LAs) are rare benign tumors of the lymphatic vessels. In total, 95% of all reported LAs are located in the head, neck and the mediastinum. LAs of the adrenal gland are very rare and currently, only ~54 cases have been reported in literature. We present a case of a big left-sided adrenal LA. Abdominal imaging revealed a big cystic lesion in the left upper abdomen of unknown origin. For diagnostic and therapeutic reasons we performed explorative midline laparotomy. The left adrenal gland was found to be the origin of the cystic tumor...
May 2018: Journal of Surgical Case Reports
Apeksha Chaturvedi, Philip J Katzman, Arie Franco
We report the imaging appearances of a case of pathologically proven, neonatal neuroblastoma 4S with diffuse hepatic metastatic involvement at presentation. Patient had an abnormal appearing liver both by ultrasound and MR. There was no evidence for associated adrenal tumor by imaging. Lack of an associated adrenal mass led to initial misinterpretation of diffuse hepatic accumulation of MIBG seen with radionuclide scintigraphy. To the best our knowledge, this is the first report of metastatic neonatal 4S neuroblastoma without an adrenal (or extra-adrenal) primary identified either on pre- or post-natal imaging...
March 2018: Journal of Radiology Case Reports
Amadou Magagi, Harissou Adamou, Ibrahim Amadou Magagi, Maazou Halidou, Oumarou Habou, Hassane Moussa Diongolé, Maman Sani Rabiou, Mahaman Boukari Baoua
This study decribes the treatment of a 37-year old female patient, with a history of poorly treated arterial hypertension (AH), referred to surgical consultation with right lower back pain, vertigo, headache, sweating and palpitations. During hospitalization, blood pressure (BP) ranged between 130/80 mm Hg and 190/120 mm Hg. The remainder of the clinical examination was normal. Thoracoabdominal scanner showed adrenal mass measuring 55x45x65 mm compressing the inferior vena cava and the right renal vein. The assay of urinary vanilmandelic acid (VMA) was 11...
2018: Pan African Medical Journal
Marie-Claire Wagle, Daniel Kirouac, Christiaan Klijn, Bonnie Liu, Shilpi Mahajan, Melissa Junttila, John Moffat, Mark Merchant, Ling Huw, Matthew Wongchenko, Kwame Okrah, Shrividhya Srinivasan, Zineb Mounir, Teiko Sumiyoshi, Peter M Haverty, Robert L Yauch, Yibing Yan, Omar Kabbarah, Garret Hampton, Lukas Amler, Saroja Ramanujan, Mark R Lackner, Shih-Min A Huang
KRAS - and BRAF -mutant tumors are often dependent on MAPK signaling for proliferation and survival and thus sensitive to MAPK pathway inhibitors. However, clinical studies have shown that MEK inhibitors are not uniformly effective in these cancers indicating that mutational status of these oncogenes does not accurately capture MAPK pathway activity. A number of transcripts are regulated by this pathway and are recurrently identified in genome-based MAPK transcriptional signatures. To test whether the transcriptional output of only 10 of these targets could quantify MAPK pathway activity with potential predictive or prognostic clinical utility, we created a MAPK Pathway Activity Score (MPAS) derived from aggregated gene expression...
2018: NPJ Precision Oncology
Paula Marincola Smith, Colleen M Kiernan, Thuy B Tran, Lauren M Postlewait, Shishir K Maithel, Jason Prescott, Timothy Pawlik, Tracy S Wang, Jason Glenn, Ioannis Hatzaras, Rivka Shenoy, John Phay, Lawrence A Shirley, Ryan C Fields, Linda Jin, Sharon Weber, Ahmed Salem, Jason Sicklick, Shady Gad, Adam Yopp, John Mansour, Quan-Yang Duh, Natalie Seiser, Konstantinos Votanopoulos, Edward A Levine, George Poultsides, Carmen C Solórzano
BACKGROUND: Adrenocortical carcinoma (ACC) is a rare and aggressive cancer. This report describes factors and outcomes associated with resection of extra-adrenal organs en bloc during index adrenalectomy. METHODS: Patients who underwent ACC resection for non-metastatic disease from 1993 to 2014 at 13 participating institutions of the US-ACC Group were included in the study. Factors associated with en bloc resection were assessed by uni- and multivariate analysis...
June 4, 2018: Annals of Surgical Oncology
Jing He, Yan Zou, Xiaodan Liu, Jinhong Zhu, Jiao Zhang, Ruizhong Zhang, Tianyou Yang, Huimin Xia
Neuroblastoma is a commonly occurring extracranial pediatric solid tumor without defined etiology. Polymorphisms in pre-miRNAs have been demonstrated to associate with the risk of several cancers. So far, no such polymorphism has been investigated in neuroblastoma. With this in mind, we performed a two-center case-control study to assess the association of genetic variants in pre-miRNAs and neuroblastoma susceptibility in Chinese children, including 393 cases and 812 controls. We found that miR-34b/c rs4938723 T > C polymorphism was significantly associated with decreased neuroblastoma risk (TC versus TT: adjusted odds ratio [OR] = 0...
June 1, 2018: Molecular Therapy. Nucleic Acids
C C H J Kuijpers, L E L Hendriks, J L Derks, A-M C Dingemans, A S R van Lindert, M M van den Heuvel, R A Damhuis, S M Willems
OBJECTIVES: Biological predisposition for specific metastatic organs might differ between molecular subgroups of lung cancer. We aimed to assess the association between molecular status and metastatic organs at diagnosis in a nationwide stage IV non-squamous non-small cell lung cancer ((ns)-NSCLC) cohort. METHODS: All ns-NSCLC from 2013 that were stage IV at diagnosis were identified from the Netherlands Cancer Registry, which records information on metastatic organs at diagnosis...
July 2018: Lung Cancer: Journal of the International Association for the Study of Lung Cancer
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