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Nicola Tufton, Kirun Gunganah, Shazia Hussain, Maralyn Druce, Robert Carpenter, Michael Ashby, William M Drake, Scott A Akker
We read with interest the article on whether patients with hormonally functioning phaeochromocytomas and paragangliomas receive proper adrenoreceptor blockade (1). Since the removal of intravenous phenoxybenzamine (Goldshield) from the UK formulae due to worldwide lack of availability, providing patients with adequate alpha adrenoreceptor blockade has become more challenging. We conducted a small retrospective audit in our department to investigate the efficacy of oral Phenoxybenzamine compared to intravenous Phenoxybenzamine as we had formed the clinical impression that that pre-operative preparation is currently less safe than it used to be...
October 19, 2016: Clinical Endocrinology
Eugenia Caballero Rodríguez, Beatriz Arencibia Pérez, Guillermo Hernández Hernández
Gangliocytic paraganglioma is an uncommon entity. The jejunum location is extremely rare: there are only five cases reported in medical literature. We present a case of gangliocytic paraganglioma.
October 19, 2016: Revista Española de Enfermedades Digestivas
Aruna Nambirajan, Mehar C Sharma, Madhu Rajeshwari, Aanchal Kakkar, Vaishali Suri, Chitra Sarkar
Ependymomas are gliomas that recapitulate normal ependymal cells. The epithelial membrane antigen (EMA) shows "dot-like" and "ring-like" staining patterns, highlighting "microlumens" or intracytoplasmic rosettes, a pathognomonic ultrastructural feature. NHERF1/EBP50, an adaptor protein localized at the apical plasma membrane of human epithelia, has been found to localize to these microlumens. We aimed to analyze the staining patterns of EMA and EBP50 in ependymomas and other tumors, and thereby compare their diagnostic utility...
October 7, 2016: Applied Immunohistochemistry & Molecular Morphology: AIMM
Tanveer Ahmad, Shankaragouda Patil, Ashwini Kumar Pasarad, Nandakumar Neralakere Maheshwarappa, Kolkebaile Sadanand Kishore
Paragangliomas, extra-adrenal pheochromocytomas, are rare catecholamine-secreting tumor. A 34-year-old lady admitted with diagnosis of ST elevation acute myocardial infarction with cardiogenic shock. Left ventricular function, severely depressed, returned to normal after initial stabilization. Coronary angiogram was normal. A para-aortic paraganglioma was diagnosed during the patient's work-up with biochemical studies, computed tomography of abdomen and functional radioisotopes imaging and was eventually surgically resected...
September 2016: Indian Heart Journal
Hiroyuki Hirai, Sanae Midorikawa, Shinichi Suzuki, Hironobu Sasano, Tsuyoshi Watanabe, Hiroaki Satoh
We herein present the findings of a 42-year-old woman with either adrenal pheochromocytoma or intraadrenal paraganglioma that simultaneously secreted somatostatin, thus mimicking insulin-dependent diabetes mellitus. Pheochromocytoma was clinically diagnosed based on scintigraphy, elevated catecholamine levels, and finally a histopathological analysis of resected specimens. The patient had diabetic ketosis, requiring 40 U insulin for treatment. Following laparoscopic adrenalectomy, insulin therapy was discontinued and the urinary c-peptide levels changed from 5...
2016: Internal Medicine
Yusuke Matsuda, Noriko Kimura, Takanobu Yoshimoto, Yoshihiro Sekiguchi, Junzo Tomoishi, Ichiro Kasahara, Yoshihito Hara, Yoshihiro Ogawa
Pheochromocytomas and paragangliomas, which exclusively produce dopamine, are very rare. Herein, we report for the first time a Japanese case of an exclusively dopamine-producing paraganglioma accompanied by detailed immunohistochemical analyses. A 70-year-old Japanese woman was referred to our hospital for functional examination of her left retroperitoneal mass. Her adrenal functions were normal, except for excessive dopamine secretion. After the tumorectomy, her dopamine level normalized. The histopathological diagnosis of the tumor was paraganglioma; this was confirmed by positive immunostaining of chromogranin A (CgA), tyrosine hydroxylase (TH), dopamine β-hydroxylase (DBH), and succinate dehydrogenase gene subunit B (SDHB)...
October 14, 2016: Endocrine Pathology
Ivana Jochmanova, Karel Pacak
Dysregulated metabolism is one of the key characteristics of cancer cells. The most prominent alterations are present during regulation of cell respiration, which leads to a switch from oxidative phosphorylation to aerobic glycolysis. This metabolic shift results in activation of numerous signaling and metabolic pathways supporting cell proliferation and survival. Recent progress in genetics and metabolomics has allowed us to take a closer look at the metabolic changes present in pheochromocytomas (PHEO) and paragangliomas (PGL)...
October 15, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
Didier Bessis, Michèle Bigorre, Nausicaa Malissen, Guillaume Captier, Christine Chiaverini, Claire Abasq, Sébastien Barbarot, Olivia Boccara, Emmanuelle Bourrat, Hassan El Fertit, Catherine Eschard, Thomas Hubiche, Jean-Philippe Lacour, Nicolas Leboucq, Emmanuel Mahé, Stéphanie Mallet, Myriam Marque, Ludovic Martin, Juliette Mazereeuw-Hautier, Nathalie Milla, Alice Phan, Patrice Plantin, Marie-Christine Picot, Eve Puzenat, Valérie Rigau, Pierre Vabres, Sylvie Fraitag, Franck Boralevi
BACKGROUND: Hair collar sign (HCS) and hair tuft of the scalp (HTS) are cutaneous signs of an underlying neuroectodermal defect, but most available data are based on case reports. OBJECTIVE: We sought to define the clinical spectrum of HCS and HTS, clarify the risk for underlying neurovascular anomalies, and provide imaging recommendations. METHODS: A 10-year multicenter retrospective and prospective analysis of clinical, radiologic, and histopathologic features of HCS and HTS in pediatric patients was performed...
October 11, 2016: Journal of the American Academy of Dermatology
Letizia Canu, Silvia Pradella, Elena Rapizzi, Rossella Fucci, Andrea Valeri, Vittorio Briganti, Valentino Giachè, Gabriele Parenti, Tonino Ercolino, Massimo Mannelli
Metastatic pheochromocytomas (PHEOs) and paragangliomas (sPGLs) are rare neural crest-derived tumors with a poor prognosis. About 50% of them are due to germ-line mutations of the SDHB gene. At present, there is no cure for these tumors. Their therapy is palliative and represented by different options among which antiangiogenic drugs, like sunitinib, have been hypothesized to be effective especially in malignant SDHB mutated tumors. We report the effects of sunitinib therapy in a SDHB mutation carrier affected by a malignant sPGL...
October 10, 2016: Archives of Endocrinology and Metabolism
Khavatkopp C, Angadi N
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Sarah Sarojini M, Ganesh H K
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
James F Powers, Karel Pacak, Arthur S Tischler
A major impediment to the development of effective treatments for metastatic or unresectable pheochromocytomas and paragangliomas has been the absence of valid models for pre-clinical testing. Attempts to establish cell lines or xenografts from human pheochromocytomas and paragangliomas have previously been unsuccessful. NOD-scid gamma (NSG) mice are a recently developed strain lacking functional B-cells, T-cells, and NK cells. We report here that xenografts of primary human paragangliomas will take in NSG mice while maintaining their architectural and immunophenotypic characteristics as expressed in the patients...
October 6, 2016: Endocrine Pathology
Mutsushi Yamasaki, Yoshiyasu Sato, Takeo Nomura, Fuminori Sato, Shinya Uchino, Hiromitsu Mimata
Multiple endocrine neoplasia type 2 (MEN2) is an autosomal-dominant cancer syndrome with major components of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN2B is the most aggressive and rarest of the MEN2 variants. Pheochromocytoma in MEN2 is virtually always located in the adrenal medulla, but MEN2-associated extra-adrenal pheochromocytomas (paraganglioma) are rare. A 59-year-old man who has been diagnosed with MEN2B consulted our hospital for surgical treatment of a 10-mm left adrenal mass and a 30-mm retroperitoneal mass...
October 5, 2016: Asian Journal of Endoscopic Surgery
Isabelle Bourdeau, Solange Grunenwald, Nelly Burnichon, Emmanuel Khalifa, Nadine Dumas, Marie-Claire Binet, Serge Nolet, Anne-Paule Gimenez-Roqueplo
BACKGROUND: More than 40% of patients with paragangliomas (PGL) harbor a germline mutation of the known PGL susceptibility genes mainly in the SDHB or SDHD genes. OBJECTIVE: To characterize the genetic background of the French Canadian (FC) patients with PGL and provide new clinical and paraclinical insights on SDHC-related PGL. METHODS: Genetic testing has been offered to FC patients affected with PGL followed at the adrenal genetics clinic at Centre Hospitalier de l'Université de Montréal (CHUM)...
October 4, 2016: Journal of Clinical Endocrinology and Metabolism
B G Challis, R T Casey, H L Simpson, M Gurnell
Phaeochromocytomas and paragangliomas (PPGLs) are catecholamine secreting neuroendocrine tumours that predispose to haemodynamic instability. Currently, surgery is the only available curative treatment, but carries potential risks including hypertensive and hypotensive crises, cardiac arrhythmias, myocardial infarction and stroke, due to tumoral release of catecholamines during anaesthetic induction and tumour manipulation. The mortality associated with surgical resection of PPGL has significantly improved from 20-45% in the early 20(th) century(1) to 0-2...
October 3, 2016: Clinical Endocrinology
Fan Chen, Xiangyu Wang, Yang Wang, Hui Meng, Xinguo Hou, Yaofeng Zhu, Wei Gao, Xuewen Jiang, Shouzhen Chen, Zhaocun Zhang, Zhichuan Zou, Tianyi He, Yue Yang, Kejia Zhu, Yong Wang, Yaxiao Liu, Jianfeng Cui, Benkang Shi, Gang Yin
Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing's syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH)...
September 2016: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
G C Ginesu, M Barmina, P Paliogiannis, M Trombetta, M L Cossu, C F Feo, F Addis, A Porcu
BACKGROUND: Paragangliomas are rare neoplasms that originate from the neural crest. They are malignant in approximately 10% of cases, with a 50% survival rate at 5 years from diagnosis. In most cases, manifestations of malignancy (such as metastasis) are lacking, and paragangliomas are considered benign lesions. Pancreatic paragangliomas are extremely rare, with only 31 cases described in the scientific literature to date. CASE SUMMARY: Here we describe a case of a 55-year-old Caucasian male patient referred to our institution in September 2013 for lumbar pain lasting five months...
September 21, 2016: International Journal of Surgery Case Reports
Sheikh Asad, Jens Peters-Willke, Louise Nott
Paragangliomas (or extra-adrenal paragangliomas) are rare neuroendocrine tumors that arise from the extra-adrenal autonomic paraganglia, small organs consisting mainly of neuroendocrine cells that are derived from the embryonic neural crest and have the ability to secrete catecholamines. This is in contrast to pheochromocytomas, where tumors occur in the adrenal gland. Malignant paraganglioma is a very rare presentation. We present a case report of a 56-year-old woman with acute foot drop with no previous significant background history...
December 2015: J Spine Surg
Luis Jaime Castro-Vega, Soto Romuald Kiando, Nelly Burnichon, Alexandre Buffet, Laurence Amar, Christophe Simian, Amandine Berdelou, Pilar Galan, Martin Schlumberger, Nabila Bouatia-Naji, Judith Favier, Brigitte Bressac-de Paillerets, Anne-Paule Gimenez-Roqueplo
CONTEXT: The microphthalmia-associated transcription factor (MITF) regulates the survival, proliferation and differentiation of neural crest-derived lineages. Recent studies reported an increased risk of melanoma in individuals carrying the rare variant MITF, p.E318K (rs149617956). Whether this variant plays a role in other neural-crest derived tumors is unknown. OBJECTIVE: In the present study, we aimed at determining the prevalence of the MITF, p.E318K variant in a well characterized French cohort of pheochromocytomas/paragangliomas (PCC/PGL)...
September 28, 2016: Journal of Clinical Endocrinology and Metabolism
Roland Darr, Joan Nambuba, Jaydira Del Rivero, Ingo Janssen, Maria Merino, Milena Todorovic, Bela Balint, Ivana Jochmanova, Josef T Prchal, Ronald Lechan, Arthur S Tischler, Vera Popovic, Dragana Miljic, Karen T Adams, F Ryan Prall, Alexander Ling, Meredith R Golomb, Michael Ferguson, Naris Nilubol, Clara Chen, Emily Chew, David Taieb, Constantine A Stratakis, Antonio Fojo, Chunzhang Yang, Electron Kebebew, Zhengping Zhuang, Karel Pacak
The syndrome of paraganglioma (PGL), somatostatinoma (SOM), and early childhood polycythemia in patients with somatic mutations in the hypoxia-inducible factor 2 alpha (HIF2A) gene is described in only a few patients worldwide. The present study provides detailed information about the clinical aspects and course of 7 patients with this syndrome and brings these experiences into perspective with the pertinent literature. Six females and one male presented at a median age of 28 years (range 11-46). Two were found to have HIF2A somatic mosaicism...
September 27, 2016: Endocrine-related Cancer
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