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Malignant pheochromocytoma

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https://www.readbyqxmd.com/read/28931294/-carney-triad
#1
L Fiala, I Kocáková, R Šimůnek, E Krejčí, I Babánková, R Šefr
Carney triad is a synchronous or metachronous association of gastric gastrointestinal stromal tumors (GIST), pulmonary chondroma and extra-adrenal paraganglioma. The majority of patients have only one or two components of the triad, all three tumors being found in only about 2% of the patients at the time of the first diagnosis. The most common combination is gastric and pulmonary tumors. We report a case of Carney triad which was diagnosed at Masaryk Memorial Cancer Institute. A 57-year-old female patient with a history of gastric resection for leiomyosarcoma at the age of 14 and with an unclear pulmonary lesion evident on chest X-ray since as early as 2003...
2017: Rozhledy V Chirurgii: Měsíčník Československé Chirurgické Společnosti
https://www.readbyqxmd.com/read/28902384/-multiple-endocrine-neoplasia
#2
Ludwig Schaaf, Friedhelm Raue
Multiple endocrine neoplasia type 1 and 2 are hereditary cancer syndromes. They are characterized by the occurrence of many benign and malignant tumor types, in MEN1 parathyroid tumors, pituitary tumors, and pancreas tumors, in MEN2 medullary thyroid carcinoma, pheochromocytoma, and parathyroid tumors. The autosomal dominant inherited tumor syndromes are caused by mutations in the MEN1 gene, a tumor suppressor gene, and mutations in the RET gene, an activated oncogene, in MEN2. The clinical expression of the different tumors can vary within and between families, with a good genotype-phenotype correlation in MEN2...
September 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28884434/clinical-predictors-of-malignancy-in-patients-with-pheochromocytoma-and-paraganglioma
#3
Mashaal Dhir, Wei Li, Melissa E Hogg, David L Bartlett, Sally E Carty, Kelly L McCoy, Sue M Challinor, Linwah Yip
BACKGROUND AND PURPOSE: Factors associated with malignancy in patients with pheochromocytoma (adrenal tumors, Pheo) and paraganglioma (extra-adrenal, PGL) are not well-defined and all patients require lifelong surveillance. The primary aim of our study was to determine genetic and clinical variables associated with malignancy in patients with Pheo/PGL. METHODS: Single institution retrospective review was performed of all patients who underwent surgery (1/95-1/15) for Pheo/PGL...
September 7, 2017: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/28875969/-gastroenterological-manifestations-of-von-hippel-lindau-disease
#4
EDITORIAL
Joanna Koniusz, Krzysztof Dąbkowski, Katarzyna Buczek, Aleksandra Gomółka, Teresa Starzyńska
Von Hippel-Lindau disease is rare autosomal dominant disorder that results from mutation of VHL gene. Typical manifestations of this syndrome include haemangioblastomas of retina, cerebellum and spinal cord, endolymphatic sac tumors, clear cell cancer and kidney cysts, pheochromocytoma, pancreatic cysts and neuroendocrine tumors. The differential diagnosis of pancreatic lesions in patients with von Hippel Lindau syndrome plays an important role. The pancreas in VHL disease is not only site of benign lesions (cysts, serous systic adenomas) but also of potentially malignant (neuroendocrine) and malignant tumors(metastases)...
August 21, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28807108/etiologies-and-management-of-cutaneous-flushing-malignant-causes
#5
REVIEW
Azeen Sadeghian, Hailey Rouhana, Brittany Oswald-Stumpf, Erin Boh
The second article in this 2-part continuing medical education series reviews the following malignant causes of flushing: mastocytosis, medullary thyroid carcinoma, pheochromocytoma, carcinoid tumors, gastroenteropancreatic neuroendocrine tumors, bronchogenic carcinoma, vasointestinal polypeptide secreting tumors, and renal cell carcinoma. The information provided will allow physicians to better distinguish patients who have worrisome presentations that require a more thorough investigation. Appropriate diagnostic workup and treatment options for these malignancies are reviewed...
September 2017: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/28801285/-clinical-analysis-of-939-patients-with-adrenal-lesions-detected-by-abdominal-computed-tomography
#6
Cun-Xia Fan, Jia-Jun Zhang, Ying-Ying Cai, Chun-Yan Wu, Shao-Zhou Zou, Yi-Kai Xu, Yao-Ming Xue, Mei-Ping Guan
OBJECTIVE: To investigate the prevalence, etiology and clinical characteristics of adrenal lesions detected by abdominal computed tomography (CT). METHODS: This retrospective study was conducted in patients with adrenal lesions detected by abdominal CT examinations in Nanfang Hospital between July, 2014 and June, 2015. The clinical data of the patients were collected for analysis of the demographics, comorbidities, imaging characteristics, biochemical profiles, clinical diagnosis and intervention...
August 20, 2017: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/28760236/adrenal-imaging
#7
REVIEW
Mishal Mendiratta-Lala, Anca Avram, Adina F Turcu, N Reed Dunnick
Cross-sectional imaging can make a specific diagnosis in lesions, such as myelolipomas, cysts, and hemorrhage, and is often sufficient to distinguish benign from malignant adrenal processes. CT and MRI are useful studies to identify pheochromocytomas and cortisol-secreting or androgen-secreting tumors. In patients with primary aldosteronism, adrenal venous sampling remains the most accurate localizing study and should be performed in all patients older than 35. Radiolabeled isotope studies serve as second-line diagnostic tests for malignant adrenal tumors, primary or metastatic, as well as for pheochromocytoma...
September 2017: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/28759096/pheochromocytoma-induced-cardiomyopathy-mimicking-acute-coronary-syndrome
#8
Viliane Vilcant
Pheochromocytoma is a rare catecholamine-secreting tumor with a prevalence of 0.1% to 0.6% in hypertensive patients. The classic triad of symptoms is headache, palpitations, and diaphoresis, but clinical presentation varies greatly. Pheochromocytoma can also mimic acute coronary syndrome and heart failure. With surgical resection, appropriate preoperative medical therapy, and 10% malignancy rate, prognosis is usually good. In the present case, a patient presented to the emergency department with symptoms suggesting a non-ST-segment elevation myocardial infarction and was transferred to a tertiary medical center for a cardiac catheterization...
August 1, 2017: Journal of the American Osteopathic Association
https://www.readbyqxmd.com/read/28753846/the-landscape-of-whole-genome-alterations-and-pathologic-features-in-genitourinary-malignancies-an-analysis-of-the-cancer-genome-atlas
#9
Mark W Ball, Michael A Gorin, Charles G Drake, Hans J Hammers, Mohamad E Allaf
BACKGROUND: The accumulation of somatic genetic alterations drives carcinogenesis. Little is known, however, about how the level of genetic alteration across an entire cancer genome affects tumor grade, stage or survival. OBJECTIVE: To investigate the influence of somatic mutation count (MC) and copy number variation (CNV) on pathologic and oncologic outcomes in patients with genitourinary malignancies in The Cancer Genome Atlas (TCGA). DESIGN, SETTING, AND PARTICIPANTS: TCGA data sets for adrenocortical carcinoma (ACC), bladder urothelial carcinoma (BLCA), chromophobe renal cell carcinoma (RCC; KICH), clear cell RCC (KIRC), papillary RCC (KIRP), pheochromocytoma and paraganglioma (PCPG), prostate adenocarcinoma (PRAD), and testis germ cell tumor (TGCT) were accessed via cBioportal...
February 8, 2017: European Urology Focus
https://www.readbyqxmd.com/read/28752085/review-of-pediatric-pheochromocytoma-and-paraganglioma
#10
REVIEW
Reshma Bholah, Timothy Edward Bunchman
Pheochromocytoma (PCC) and paraganglioma (PGL) are rare chromaffin cell tumors which secrete catecholamines and form part of the family of neuroendocrine tumors. Although a rare cause of secondary hypertension in pediatrics, the presentation of hypertension in these patients is characteristic, and treatment is definitive. The gold standard for diagnosis is via measurement of plasma free metanephrines, with imaging studies performed for localization, identification of metastatic lesions and for surgical resection...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28748315/interferon-alpha-treatment-for-disease-control-in-metastatic-pheochromocytoma-paraganglioma-patients
#11
Julien Hadoux, Marie Terroir, Sophie Leboulleux, Frederic Deschamps, Abir Al Ghuzlan, Ségolène Hescot, Lambros Tselikas, Isabelle Borget, Caroline Caramella, Desirée Déandréis, Diane Goere, Thierry De Baere, Martin Schlumberger, Eric Baudin
Interferon-alpha (IFN-alpha) is recommended in neuroendocrine tumors (NET). Malignant pheochromocytoma and paragangliomas (MPPGLs) constitute a rare subgroup of NET with few treatment options. IFN-alpha efficacy in patients with MPPGLs was evaluated in a single-center retrospective study. Progression-free survival (PFS) was the primary endpoint according to RECIST 1.1 and/or PERCIST 1.0, and response rate, safety, and symptomatic efficacy were secondary endpoints. Fourteen patients received peginterferon alfa-2a (90 to 180 μg/week) or interferon alfa-2b (1...
July 26, 2017: Hormones & Cancer
https://www.readbyqxmd.com/read/28705916/biodistribution-and-dosimetry-of-18-f-meta-fluorobenzyl-guanidine-mfbg-a-first-in-human-pet-ct-imaging-study-of-patients-with-neuroendocrine-malignancies
#12
Neeta Pandit-Taskar, Pat B Zanzonico, Kevin D Staton, Jorge A Carrasquillo, Diane Reidy-Lagunes, Serge K Lyashchenko, Eva Burnazi, Hanwen Zhang, Jason S Lewis, Ronald Blasberg, Steven M Larson, Wolfgang Andreas Weber, Shakeel Modak
Introduction: Iodine-123-meta-iodobenzylguanidine ((123)I-MIBG) imaging is currently a mainstay in the evaluation of many neuroendocrine tumors, especially neuroblastoma. (123)I-MIBG imaging has several limitations that can be overcome by the use of a PET agent. (18)F-MFBG is a positron emission tomography (PET) analog of MIBG that may allow for single-day, high-resolution quantitative imaging. We conducted a first-in-human study of (18)F-MFBG PET imaging to evaluate the safety, feasibility, pharmacokinetics, and dosimetry of (18)F-MFBG in neuroendocrine tumors (NETs)...
July 13, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28702269/marker-negative-pheochromocytoma-associated-with-inferior-vena-cava-thrombosis
#13
S Poudyal, M Pradhan, S Chapagain, B R Luitel, P R Chalise, U K Sharma, P R Gyawali
Pheochromocytoma associated with inferior vena cava (IVC) thrombosis is very rare. A 27-year-old female presented with right flank pain and hypertensive urgency. Contrast-enhanced CT abdomen and gadolinium-contrast MRI abdomen revealed right adrenal mass suspicious of malignancy with invasion and compression to the right IVC wall along with IVC thrombus extending from the level of renal veins to the level of confluence with hepatic veins. Her routine laboratory investigations including 24-hour urine fractionated metanephrines, vanillylmandelic acid, and cortisol were normal...
2017: Case Reports in Urology
https://www.readbyqxmd.com/read/28688648/nationwide-review-of-hormonally-active-adrenal-tumors-highlights-high-morbidity-in-pheochromocytoma
#14
Punam P Parikh, Gustavo A Rubio, Josefina C Farra, John I Lew
BACKGROUND: Adrenal adenomas are benign tumors often discovered incidentally, and >70% are hormonally inactive. The remaining subset may produce excess aldosterone, cortisol, or catecholamine. Perioperative outcomes after adrenalectomy for such "hormonally active" tumors remain unclear. This study examines in-hospital outcomes after unilateral adrenalectomy for hormonally active tumors. METHODS: A retrospective review was performed using the Nationwide Inpatient Sample (2006-2011) to identify patients undergoing unilateral adrenalectomy for hormonally active or inactive tumors...
July 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28687023/adrenalectomy-for-incidentaloma-lessons-learned-from-a-single-centre-series-of-274-patients
#15
Sébastien Gaujoux, Adeline Aimé, Guillaume Assié, Roberto Ciuni, Stéphane Bonnet, Florence Tenenbaum, Jérome Bertherat, Bertrand Dousset
BACKGROUND: Adrenal incidentalomas are increasingly diagnosed and include a wide spectrum of lesions from benign adenomas to secreting or malignant lesions. The aim of the present study is to report a large single-institution experience of patients undergoing surgery for adrenal incidentaloma with particular attention to their diagnosis and post-operative course and the evolution of surgical practice over time. METHODS: From 1993 to 2013, 274 patients underwent adrenalectomy for incidentaloma...
July 7, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28685225/phase-ii-trial-of-pazopanib-in-advanced-progressive-malignant-pheochromocytoma-and-paraganglioma
#16
Sina Jasim, Vera J Suman, Camilo Jimenez, Pamela Harris, Kostandinos Sideras, Jill K Burton, Francis Paul Worden, Richard J Auchus, Keith C Bible
INTRODUCTION: Pheochromocytomas and paragangliomas (Pheo/PGL) are rare, vascular, sometimes malignant endocrine tumors. Case reports indicate the activity of vascular endothelium growth factor receptor-targeted kinase inhibitors in these cancers. OBJECTIVES: To assess the antitumor activity and tolerability of pazopanib in progressive malignant Pheo/PGL. PATIENTS AND METHODS: This multicenter Phase II trial (MC107C) enrolled individuals  ≥18 years old with disease progression ≤ 6 months prior to registration, Eastern Cooperative Oncology Group PS 0-2, and measurable disease (response evaluation criteria in solid tumors 1...
August 2017: Endocrine
https://www.readbyqxmd.com/read/28670912/mandibular-lytic-lesion-in-familial-paraganglioma-syndrome-type-i-a-clinical-conundrum
#17
Parul Sinha, Sonia N Yuen, Rebecca D Chernock, Bruce H Haughey
OBJECTIVE: The entity of primary mandibular paraganglioma (PGL) is not well accepted within the head and neck. Mandibular PGLs hitherto reported in literature are malignant metastatic lesions, mostly from a pheochromocytoma. METHODS: We report a case of mandibular lytic lesion in a young female with multifocal PGLs but no family history of PGLs. We also performed a literature search to identify published cases of mandibular PGL. RESULTS: Lack of established criteria for malignancy in a PGL made diagnosis and treatment challenging...
August 2017: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/28665830/undiagnosed-pheochromocytoma-simulating-malignant-hyperthermia
#18
Nisha S Ramani, Robert Stoppacher, Ajaykumar C Morani, Charles Catanese
INTRODUCTION: Pheochromocytomas are rare catecholamine-producing neuroendocrine tumors. They are surgically curable but can be lethal if remain undiagnosed. We report a patient earlier diagnosed with malignant hyperthermia but later found to have pheochromocytoma on autopsy. CASE REPORT: After a preprocedural pain block for elective right shoulder arthroscopy, a 53-year-old hypertensive white man developed chest pain. In the operating room, he had increased blood pressure...
September 2017: American Journal of Forensic Medicine and Pathology
https://www.readbyqxmd.com/read/28650885/aggressive-imaging-features-in-a-malignant-pheochromocytoma-with-a-novel-mutation-of-the-sdhb-gene
#19
Soon-Ah Park, Dae Ho Lee, Hun Soo Kim
We describe a 15-year-old boy with a huge bilateral adrenal pheochromocytoma that had a de novo germline mutation in the succinate dehydrogenase subunit B (SDHB) gene. F-FDG PET/CT revealed bilateral metabolically active large masses in the adrenal glands and the activated brown adipose tissues. The I-MIBG scintigraphic findings revealed only a mild accumulation of MIBG in the right adrenal mass, but a high uptake in the left adrenal mass. Thus, F-FDG PET/CT imaging may be more effective than I-MIBG scintigraphy for the evaluation of pheochromocytomas that are associated with highly malignant characteristics resulting from mutations of the SDHB gene...
September 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28643981/-adrenal-surgery-multidisciplinarity-is-mandatory
#20
Marie-Laure Matthey Gié, Marie Nicod Lalonde, Elena Gonzalez Rodriguez, Nicolas Demartines, Maurice Matter
Adrenal tumours are rare and their management is challenging. Every patient presenting with adrenal mass or symptoms of hormones hypersecretion should be investigated. The two important questions to be answered are : 1. Is the tumour secreting ? Is the tumour malignant or not ? A complete endocrine work-up and a nativ CT-scan may usually answer these two questions but have to be interpreted by specialists in a multidisciplinary team setting. The decisions about managements of adrenal pathologies do follow international guidelines which are regularly updated...
June 14, 2017: Revue Médicale Suisse
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