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Pheochromocytoma

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https://www.readbyqxmd.com/read/29146416/fulminant-adrenergic-myocarditis-complicated-by-pulmonary-edema-cardiogenic-shock-and-cardiac-arrest
#1
Pawel Rostoff, Bohdan Nessler, Patrycja Pikul, Karolina Golinska-Grzybala, Tomasz Miszalski-Jamka, Jadwiga Nessler
Adrenergic myocarditis is an uncommon presentation of pheochromocytoma and extremely rare cause of de novo acute heart failure (AHF). We present a case of a 31-year-old Caucasian woman with a history of hypertension and recurrent occipital headaches who was admitted to the emergency department due to severe de novo AHF presenting as pulmonary edema and cardiogenic shock. During the hospital admission the patient experienced asystolic cardiac arrest and was successfully resuscitated, intubated, and mechanically ventilated...
November 10, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29144820/pheochromocytoma-a-genetic-and-diagnostic-update
#2
Leilani B Mercado-Asis, Katherine I Wolf, Ivana Jochmanova, David Taïeb
OBJECTIVE: Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors derived from adrenal or extra-adrenal locations, respectively. Upon suspicion of PPGL, specific metabolomic, molecular, biochemical, imaging, and histopathological studies are performed to prove, localize, treat, and monitor disease progression. Recently, improved diagnostic tools allow physicians to accurately diagnose PPGL, even in patients presenting with small (less than 1 cm) or biochemically silent tumors, which previously delayed proper detection and treatment...
November 16, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/29144180/intraoperative-hemodynamic-instability-and-diagnosis-of-pheochromocytoma-during-excision-of-adrenal-incidentaloma-with-incomplete-workup-a-case-report
#3
Anthony Chang, George Silvay, Andrew Goldberg
Preoperative evaluation of incidentalomas for pheochromocytoma is imperative. This case report describes a scheduled adrenalectomy in an asymptomatic patient with what was eventually determined to be an incomplete biochemical workup. The intraoperative course was complicated by labile and rapid increases in blood pressure and heart rate, suggesting the missed diagnosis of pheochromocytoma. It is important for anesthesiologists to ensure adequate preoperative biochemical workup before excluding the possibility of coexisting pheochromocytoma...
November 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29142006/characterization-of-neuroendocrine-tumors-in-heterozygous-mutant-menx-rats-a-novel-model-of-invasive-medullary-thyroid-carcinoma
#4
Sara Molatore, Andrea Kügler, Martin Irmler, Tobias Wiedemann, Frauke Neff, Annette Feuchtinger, Johannes Beckers, Mercedes Robledo, Frederico Roncaroli, Natalia S Pellegata
Rats affected by the MENX syndrome spontaneously develop multiple neuroendocrine tumors (NETs) including adrenal, pituitary and thyroid gland neoplasms. MENX was initially reported to be inherited as a recessive trait and affected rats were found to be homozygous for the predisposing Cdkn1b mutation encoding p27. We here report that heterozygous MENX mutant rats (p27+/mut) develop the same spectrum of NETs seen in the homozygous (p27mut/mut) animals but with slower progression. Consequently, p27+/mut rats have a significantly shorter lifespan compared with their wild-type (p27+/+) littermates...
November 15, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29141379/-clinical-value-of-tumor-size-in-the-evaluation-of-adrenal-incidentalomas
#5
L L Li, L Zhao, J T Dou, G Q Yang, W J Gu, Z H Lü, J M Ba, Y M Mu, J M Lu, C Y Pan
Objective: To investigate the clinical value of tumor size in the evaluation of endocrinological and histological natures regarding adrenal incidentaloma (AI) patients. Methods: A total of 1 941 AI patients who were hospitalized in Department of Endocrinology, Chinese PLA General Hospital between January 1997 and December 2016 were retrospectively reviewed. The demographics of patients, imaging features, functional status and histological results were analyzed. Results: Of 1 941 patients, 984 (50.70%) were males, and 957 (49...
November 14, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/29136674/familial-hyperparathyroidism-disorders-of-growth-and-secretion-in-hormone-secretory-tissue
#6
Stephen J Marx, Delmar Muniz Lourenco
Six syndromes of familial hyperparathyroidism are compared: 1) Familial hypocalciuric hypercalcemia (FHH) expresses primary hyperparathyroidism (PHPT) beginning at birth with lifelong hypercalcemia. There is nonsuppressed PTH secretion from outwardly normal parathyroid glands. It reflects germline heterozygous mutation in CASR, GNA11, or AP2S1. 2) Neonatal severe primary hyperparathyroidism is severest of the six syndromes. It requires urgent total parathyroidectomy in infancy. It usually reflects biallelic inactivation of the CASR...
November 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/29130501/tert-promoter-hypermethylation-is-associated-with-metastatic-disease-in-abdominal-paraganglioma
#7
Fredrika Svahn, C Christofer Juhlin, Johan O Paulsson, Omid Fotouhi, Jan Zedenius, Catharina Larsson, Adam Stenman
Telomere maintenance, a hallmark of cancer for cell immortalization, is commonly achieved by telomerase activation through induction of the telomerase reverse transcriptase (TERT) gene. Pheochromocytomas (PCC) and abdominal paragangliomas (PGL) (together PPGL) are endocrine tumours for which TERT promoter mutations and telomerase activation have been previously reported [1]. Only 10-15% of PPGL metastasize, however in the absence of metastases, the identification of malignant disease is a diagnostic dilemma...
November 11, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/29127554/is-there-any-role-for-minimally-invasive-surgery-in-net
#8
REVIEW
M Thomaschewski, H Neeff, T Keck, H P H Neumann, T Strate, E von Dobschuetz
Neuroendocrine tumors (NET) represent the variability of almost benign lesions either secreting hormones occurring as a single lesion up to malignant lesions with metastatic potential. Treatment of NET is usually performed by surgical resection. Due to the rarity of NET, surgical treatment is mainly based on the experience and recommendations of experts and less on the basis of prospective randomized studies. In addition, the development and establishment of new surgical procedures is made more difficult by their rarity...
November 11, 2017: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/29126554/preoperative-genetic-testing-in-pheochromocytomas-and-paragangliomas-influences-the-surgical-approach-and-the-extent-of-adrenal-surgery
#9
Pavel Nockel, Mustapha El Lakis, Apostolos Gaitanidis, Lily Yang, Roxanne Merkel, Dhaval Patel, Naris Nilubol, Tamara Prodanov, Karel Pacak, Electron Kebebew
BACKGROUND: Our knowledge of the susceptibility genes for pheochromocytomas/paragangliomas has increased; however, data on its impact on surgical decision-making has not been described. The aim of this study was to determine the effect of routine preoperative genetic testing on the operative intervention in patients with pheochromocytomas/paragangliomas. METHODS: One-hundred-eight patients diagnosed with pheochromocytomas/paragangliomas who underwent 118 operations had preoperative genetic testing for 9 known pheochromocytoma/paraganglioma susceptibility genes...
November 7, 2017: Surgery
https://www.readbyqxmd.com/read/29124493/genotype-phenotype-correlation-in-asian-indian-von-hippel-lindau-vhl-syndrome-patients-with-pheochromocytoma-paraganglioma
#10
Nilesh Lomte, Sanjeet Kumar, Vijaya Sarathi, Reshma Pandit, Manjunath Goroshi, Swati Jadhav, Anurag R Lila, Tushar Bandgar, Nalini S Shah
The data in genotype-phenotype correlation in Indian von Hippel-Lindau (VHL) patients is limited. We have retrospectively studied 31 genetically proven VHL patients with pheochromocytoma/paraganglioma (PCC/PGL) from families and have reviewed the World literature on PCC/PGL in patients with large VHL deletions. Three patients had large deletions and 28 patients had other mutations [missense mutations in 25, 3 bp deletion in 2 and single bp duplication in one]. Unilateral PCC were significantly more common in patients with large VHL deletions whereas multiple PCC (bilateral PCC or PCC + sympathetic PGL) were significantly more common in those with other mutations...
November 9, 2017: Familial Cancer
https://www.readbyqxmd.com/read/29122903/giant-cystic-pheochromocytoma
#11
Kris Miguel Cajipe, Gabriela Gonzalez, Dharam Kaushik
A 56-year-old healthy woman presents with 2-year history of symptoms classic for pheochromocytoma. Evaluation revealed one of the largest cystic pheochromocytomas reported but without any metastatic disease. After achieving medical management of her symptoms, surgical removal was performed successfully and without any complications intraoperatively. Pathology of the mass confirmed the diagnosis. The patient had complete resolution of her symptoms thereafter.
November 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29122324/hemodynamic-instability-during-surgery-for-pheochromocytoma-comparing-the-transperitoneal-and-retroperitoneal-approach-in-a-multicenter-analysis-of-341-patients
#12
Wessel M C M Vorselaars, Emily L Postma, Eric Mirallie, Julien Thiery, Mattan Lustgarten, Jesse D Pasternak, Rocco Bellantone, Marco Raffaelli, Thomas Fahey, Menno R Vriens, Laurent Bresler, Laurent Brunaud, Rasa Zarnegar
BACKGROUND: Intraoperative hemodynamic instability is a major challenge during adrenalectomy for pheochromocytoma. Typically, pheochromocytoma is performed laparoscopically either through the retroperitoneal or transperitoneal approach. We aimed to determine if the operative approach affects intraoperative hemodynamic instability during surgery for pheochromocytoma in a large multicenter multicenter cohort. METHODS: Retrospective, multicenter analysis of consecutive patients with pheochromocytoma who underwent total unilateral laparoscopic adrenalectomy without conversion were included...
November 6, 2017: Surgery
https://www.readbyqxmd.com/read/29121735/excluding-the-pheochromocytoma
#13
EDITORIAL
Nicola Tufton, Scott A Akker
No abstract text is available yet for this article.
December 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29121733/pheochromocytoma-as-a-reversible-cause-of-cardiomyopathy-analysis-and-review-of-the-literature
#14
Rong Zhang, Deepashree Gupta, Stewart G Albert
CONTEXT: Pheochromocytoma and paraganglioma are rare neuroendocrine tumors which overproduce catecholamines and arise from the adrenal gland or extra-adrenal chromaffin cells of the sympathetic and parasympathetic ganglia (1). Excessive catecholamine-induced stimulation of cardiac myocytes leads to damage which manifests in several forms ranging from Takotsubo to dilated cardiomyopathy. Diagnosis of pheochromocytoma-related cardiomyopathies is often delayed due to the atypical presentation associated with many cases...
December 15, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/29120941/effects-of-gynosaponin-tn-2-on-l-dopa-induced-cytotoxicity-in-pc12-cells
#15
Keon Sung Shin, Hyun Jin Park, Keun Hong Park, Kyung Sook Lee, Sung Woo Jeong, Bang Yeon Hwang, Chong Kil Lee, Myung Koo Lee
Gynosaponins have pharmacological effects on 3,4-L-dihydroxyphenylalanine (L-DOPA)-related or dopamine-related neurological diseases; however, the neuroprotective functions of single compound of gynosaponins remain undefined. This study investigated the cytotoxic effects of gynosaponin TN-2 on L-DOPA in pheochromocytoma 12 cells. Gynosaponin TN-2, at 0.5-3 μM, did not exhibit cytotoxicity and protected against L-DOPA (100 and 200 μM)-induced cell death. Gynosaponin TN-2 (0.5 and 1.0 μM) inhibited the L-DOPA (100 and 200 μM)-induced sustained extracellular signal-regulated protein kinases 1 and 2 phosphorylation...
November 8, 2017: Neuroreport
https://www.readbyqxmd.com/read/29120654/-diagnosis-and-management-of-metastatic-pheochromocytoma-and%C3%A2-paraganglioma
#16
Ivana Jochmanová, Ivica Lazúrová
Pheochromocytoma (PHEO) and paraganglioma (PGL) are rare catecholamine-producing neuroendocrine tumors arising from adrenal medulla or extra-adrenal sympathetic and parasympathetic ganglia. Most of the PHEOs/PGLs are benign tumors, but metastatic disease is common, especially in patients with particular genetic background. Although PHEOs/PGLs were described more than a century ago, diagnosis and therapy of metastatic disease are still challenging. Advances in understanding molecular and metabolic changes associated with tumorigenesis lead us to identification of the background of these tumors...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29117221/validation-of-pathological-grading-systems-for-predicting-metastatic-potential-in-pheochromocytoma-and-paraganglioma
#17
Jung-Min Koh, Seong Hee Ahn, Hyeonmok Kim, Beom-Jun Kim, Tae-Yon Sung, Young Hoon Kim, Suck Joon Hong, Dong Eun Song, Seung Hun Lee
PURPOSE: The Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP) was proposed for predicting the metastatic potential of pheochromocytoma and paraganglioma to overcome the limitations of the Pheochromocytoma of the Adrenal Scaled Score (PASS). However, to date, no study validating the GAPP has been conducted, and previous studies did not include mutations in the succinate dehydrogenase type B (SDHB) gene in the score calculation. In this retrospective cohort study, we validated the prediction ability of GAPP and assessed whether it would be improved by inclusion of the loss of SDHB immunohistochemical staining...
2017: PloS One
https://www.readbyqxmd.com/read/29113559/proceedings-of-the-2017-national-toxicology-program-satellite-symposium
#18
Susan A Elmore, Famke Aeffner, Dinesh S Bangari, Torrie A Crabbs, Stacey Fossey, Shayne C Gad, Wanda M Haschek, Jessica S Hoane, Kyathanahalli Janardhan, Ramesh C Kovi, Gail Pearse, Lyn M Wancket, Erin M Quist
The 2017 annual National Toxicology Program Satellite Symposium, entitled "Pathology Potpourri," was held in Montreal, Quebec, Canada at the Society of Toxicologic Pathology's 36th annual meeting. The goal of this symposium was to present and discuss challenging diagnostic pathology and/or nomenclature issues. This article presents summaries of the speakers' talks along with select images that were used by the audience for voting and discussion. Various lesions and other topics covered during the symposium included renal papillary degeneration in perinatally exposed animals, an atriocaval mesothelioma, an unusual presentation of an alveolar-bronchiolar carcinoma, a paraganglioma of the organ of Zuckerkandl (also called an extra-adrenal pheochromocytoma), the use of human muscle samples to illustrate the challenges of manual scoring of fluorescent staining, intertubular spermatocytic seminomas, medical device pathology assessment and discussion of the approval process, collagen-induced arthritis, incisor denticles, ameloblast degeneration and poorly mineralized enamel matrix, connective tissue paragangliomas, microcystin-LR toxicity, perivascular mast cells in the forebrain thalamus unrelated to treatment, and 2 cases that provided a review of the International Harmonization of Nomenclature and Diagnostic Criteria (INHAND) bone nomenclature and recommended application of the terminology in routine nonclinical toxicity studies...
January 1, 2017: Toxicologic Pathology
https://www.readbyqxmd.com/read/29108378/preoperative-risk-factors-for-massive-blood-loss-in-adrenalectomy-for-pheochromocytoma
#19
Hongju Liu, Bin Li, Xuerong Yu, Yuguang Huang
Background: This retrospective analysis of patients who underwent adrenalectomy for pheochromocytoma aimed to determine preoperative risk factors for intraoperative massive blood loss. Preoperative identification of patients at high-risk of massive blood loss may be helpful in anesthesia management and preoperative preparation. Materials and Methods: The study involved data of 268 patients who had undergone pheochromocytoma surgery at the Peking Union Medical College Hospital between January 1, 2013 and October 31, 2016...
October 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29101001/icariside-ii-a-pde5-inhibitor-from-epimedium-brevicornum-promotes-neuron-like-pheochromocytoma-pc12-cell-proliferation-via-activating-no-cgmp-pkg-pathway
#20
Jianmei Gao, Yingshu Xu, Ming Lei, Jingshan Shi, Qihai Gong
Icariside II (ICS II), a phosphodiesterase 5 inhibitor (PDE 5-I), is a major ingredient of Epimedium brevicornum, with wide spectrum of neuroprotective properties. However, little is known about the potential beneficial effect of ICS II on neuronal cell proliferation, and its possible underlying mechanism remains still unclear. We hypothesized that the beneficial effect of ICS II on neuron-like highly differentiated rat pheochromocytoma (PC12) cell proliferation is correlated with the nitric oxide (NO) signaling pathway and its upstream of PI3K/AKT pathway...
October 31, 2017: Neurochemistry International
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