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Malignant pheochromocytoma

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https://www.readbyqxmd.com/read/28643981/-adrenal-surgery-multidisciplinarity-is-mandatory
#1
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
https://www.readbyqxmd.com/read/28626127/a-case-of-retroperitoneal-paraganglioma
#2
Takao Amaike, Toshihisa Tamura, Atsuhiro Koga, Kazunori Shibao, Aiichiro Higure, Keiji Hirata
We report a surgical case of retroperitoneal paraganglioma. A paraganglioma is a catecholamine-producing tumor originating in the chromaffin cells of the sympathetic ganglion. It is a kind of pheochromocytoma which occurs on the outside of the adrenal gland. The patient was a 72 year old male with a history of hypertension and a pacemaker implantation. A mass in the ventral side of the right iliopsoas muscle was detected during a routine contrasting computed tomography (CT) examination for checking his pacemaker...
2017: Journal of UOEH
https://www.readbyqxmd.com/read/28605453/malignant-pheochromocytoma-and-paraganglioma-272-patients-over-55-years
#3
Oksana Hamidi, William F Young, Nicole M Iñiguez-Ariza, Nana Esi Kittah, Lucinda Gruber, Cristian Bancos, Shrikant Tamhane, Irina Bancos
Context: Malignant pheochromocytoma (PHEO) and paraganglioma (PGL) are rare and knowledge of the natural history is limited. Objective: We aimed to describe baseline characteristics and outcomes of patients with malignant PHEO and PGL (PPGL) and to identify predictors of shorter survival. Design: Retrospective review of patients with malignant PPGL evaluated from 1960 to 2016. Setting: Referral center. Patients: The group comprised 272 patients...
June 12, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28585682/the-usefulness-of-laparoscopic-adrenalectomy-in-the-treatment-of-adrenal-neoplasms-a-single-centre-experience
#4
Ryszard Pogorzelski, Sadegh Toutounchi, Ewa Krajewska, Patryk Fiszer, Agata Kącka, Mariusz Piotrowski, Małgorzata Szostek, Tomasz Wołoszko, Krzysztof Celejewski, Urszula Ambroziak, Tomasz Bednarczuk, Zbigniew Gałązka
INTRODUCTION: Adrenal neoplasms comprise about 10% of all tumours affecting this organ and constitute a significant, at first diagnostic and subsequently therapeutic, problem, especially since a relatively high proportion of neoplastic lesions are asymptomatic. The number of diagnosed metastases to adrenal glands is increasing. Surgical treatment involves both open surgery as well as laparoscopy. MATERIAL AND METHODS: There were 235 adrenalectomies performed at our centre due to various indications over the past four years...
June 6, 2017: Endokrynologia Polska
https://www.readbyqxmd.com/read/28585398/genetic-status-determines-18-f-fdg-uptake-in-pheochromocytoma-paraganglioma
#5
Ankita Tiwari, Nalini Shah, Vijaya Sarathi, Gaurav Malhotra, Ganesh Bakshi, Gagan Prakash, Kranti Khadilkar, Reshma Pandit, Anurag Lila, Tushar Bandgar
INTRODUCTION: Although few studies have demonstrated utility of (18) F- fluoro-2-deoxy-d-glucose positron emission tomography/computerised tomography ((18) F-FDG PET/CT) in benign pheochromocytoma/paragangliomas (PCC/PGLs), there limited data on factors predicting the FDG uptake in PCC/PGL. METHODS: The study was conducted at a tertiary health care centre. In addition to the routine investigations, all patients (n = 96) with PCC/PGL were evaluated with (18) F-FDGPET/CT and majority (n = 78) underwent (131) I-metaiodobenzyl guanidine ((131) I-MIBG) scintigraphy...
June 5, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28566531/sdhb-mutation-carriers-with-malignant-pheochromocytoma-respond-better-to-cvd
#6
Lauren Fishbein, Sivan Ben-Maimon, Stephen Keefe, Keith Cengel, Daniel A Pryma, Arturo Loaiza-Bonilla, Douglas L Fraker, Katherine L Nathanson, Debbie L Cohen
Pheochromocytomas and paragangliomas (PCC/PGL) are tumors in the adrenal medulla and extra adrenal sites, respectively. About a quarter are metastatic, often with a long latency period. Some PCC/PGL are defined as clinically aggressive given extensive local invasion into adjacent normal tissue. These tumors, like metastatic PCC/PGL, cannot be surgically cured and patients can have continued secretion of excessive catecholamines with all related sequelae. For patients with metastatic or aggressive tumors, treatment options are limited...
May 31, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28553607/characteristics-of-pediatric-pheochromocytoma-paraganglioma
#7
REVIEW
Vijaya Sarathi
The "rule of 10" used to describe pheochromocytoma/paragangliomas (PCC/PGLs) has been challenged. However, recent studies suggested that pediatric PCC/PGLs may follow a pattern. Hence, we reviewed the available literature to verify the same. We searched PubMed, Scopus, ProQuest, and Google Scholar for studies describing the genotype and/or phenotype characteristics of pediatric PCC/PGL cohorts published after 2000 in English language and those with sample size more than 35 were included in this review. Pediatric PCC/PGLs were malignant in 10%, synchronous bilateral in 20%, extra-adrenal in 30%, among which, 30% were extra-abdominal and familial in 40%...
May 2017: Indian Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28540670/open-adrenalectomy-in-the-era-of-laparoscopic-surgery-a-review
#8
REVIEW
Giovanni Taffurelli, Claudio Ricci, Riccardo Casadei, Saverio Selva, Francesco Minni
Open adrenalectomy still plays an important role in adrenal surgery. A review of the current literature has been carried out to discuss the main indication to open adrenalectomy with regards to the main adrenal pathologies. The authors underlined the role of open adrenalectomy on the basis of personal experience and a literature review. Indication to open adrenalectomy for adrenal cysts, myelolipomas, pheochromocytomas, metastases, adrenocortical carcinomas and tumour recurrences were analysed and discussed...
May 24, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28499761/cystadenoma-of-the-rete-testis-in-a-patient-with-von-hippel-lindau-disease
#9
Jacob Lewis, David Thiel, Gregory Broderick, Candice Bolan
Von-Hippel Lindau (VHL) disease is a rare, inherited multicystic disorder that is characterized by several benign and malignant neoplasms (1). Classically, the disease manifests itself in a broad spectrum including: renal cell carcinomas, intracranial and spinal hemangioblastomas, endolymphatic sac tumors, renal and pancreatic cysts, and pheochromocytomas. Another important, but commonly forgotten manifestation is the cystadenoma of the rete testis.
May 9, 2017: Urology
https://www.readbyqxmd.com/read/28497219/robot-assisted-adrenalectomy-indications-and-drawbacks
#10
C Nomine-Criqui, A Germain, A Ayav, L Bresler, L Brunaud
Adrenal tumors can vary from a benign adrenocortical adenoma with no hormonal secretion to a secretory adrenocortical malignancy (adrenocortical carcinoma) or a hormone-secreting tumor of the adrenal medulla (pheochromocytoma). Currently, laparoscopic adrenalectomy is regarded as the preferred surgical approach for the management of most adrenal surgical disorders, although there are no prospective randomized trials comparing this technique with open adrenalectomy. However, widespread adoption of robotic technology has positioned robotic adrenalectomy as an option in some medical centers...
May 12, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28491185/extra-adrenal-pheochromocytoma-at-the-organ-of-zuckerkandl-a-case-report-and-literature-review
#11
Tarana Gill, Kalie Adler, Alicia Schrader, Keyur Desai, Joshua Wermers, Nebiyu Beteselassie
Pheochromocytomas and paragangliomas are tumors that occur in characteristic locations and are commonly detected on imaging studies. A correct diagnosis is important because of differences in associated neoplasms, risk for malignancy, and need for genetic testing. In addition, associated complications, including death, can be avoided if appropriately recognized and treated. Here, we report a rare case of an extra-adrenal paraganglioma of the organ of Zuckerkandl.
June 2017: Radiology case reports
https://www.readbyqxmd.com/read/28477311/update-on-adrenal-tumours-in-2017-world-health-organization-who-of-endocrine-tumours
#12
REVIEW
Alfred King-Yin Lam
The fourth edition of the World Health Organization (WHO) classification of endocrine tumours contains substantial new findings for the adrenal tumours. The tumours are presented in two chapters labelled as "Tumours of the adrenal cortex" and "Tumours of the adrenal medulla and extra-adrenal paraganglia." Tumours of the adrenal cortex are classified as cortical carcinoma, cortical adenoma, sex cord stromal tumours, adenomatoid tumour, mesenchymal and stromal tumours (myelolipoma and schwannoma), haematological tumours, and secondary tumours...
May 6, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28477304/routine-genetic-screening-with-a-multi-gene-panel-in-patients-with-pheochromocytomas
#13
Emilia Sbardella, Treena Cranston, Andrea M Isidori, Brian Shine, Aparna Pal, Bahram Jafar-Mohammadi, Greg Sadler, Radu Mihai, Ashley B Grossman
PURPOSE: Several new gene mutations have been reported in recent years to be associated with a risk of familial pheochromocytoma. However, it is unclear as to whether extensive genetic testing is required in all patients. METHODS: The clinical data of consecutive patients operated for pheochromocytoma over a decade in a tertiary referral center were reviewed. Genetic screening was performed using a 10-gene panel: RET, VHL, SDHB, SDHD, SDHA, SDHC, SDHAF2, MAX, TMEM127 and FH...
May 5, 2017: Endocrine
https://www.readbyqxmd.com/read/28469506/genotype-phenotype-correlation-in-patients-with-germline-mutations-of-vhl-ret-sdhb-and-sdhd-genes-thai-experience
#14
Chutintorn Sriphrapradang, Kitjapong Choopun, Atchara Tunteeratum, Thanyachai Sura
Mutations in the VHL, RET, SDHB, and SDHD genes are responsible for von Hippel-Lindau (VHL) disease, multiple endocrine neoplasia type 2 (MEN2), and familial paraganglioma, respectively. However, genotype-phenotype correlation data are lacking in Southeast Asia. A retrospective medical chart review was performed on patients referred to the genetics service. We found 35 patients diagnosed with clinical syndromes (16 VHL, 9 MEN2, 9 paragangliomas, and 1 neurofibromatosis type 1). In patients with VHL, 5 known VHL mutations were identified: p...
2017: Clinical Medicine Insights. Endocrinology and Diabetes
https://www.readbyqxmd.com/read/28458869/an-unusual-cause-of-back-pain-a-case-of-large-nonfunctioning-retroperitoneal-paraganglioma-presented-as-a-large-cystic-lesion-a-case-report-and-review-of-literature
#15
Siu Yan Amy Kok, Chung Ying Leung, Ki Yau Chow
Pheochromocytoma arising from outside the adrenal glands is also called paraganglioma. When it occurs below the diaphragm, in the organ of Zuckerkandl or retroperitoneum, it is also called extra-adrenal pheochromocytoma. Paragangliomas are rare tumors which arise from neuroendocrine cells and extra-adrenal paragangliomas (EAPs) account for only 10-15% if all paragangliomas and may present incidentally as a symptomless mass. Typical triad of sweating, headache and fluctuating hypertension if not present makes preoperative diagnosis difficult...
April 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28458565/nvp-auy922-a-novel-hsp90-inhibitor-inhibits-the-progression-of-malignant-pheochromocytoma-in-vitro-and-in-vivo
#16
Jianpo Lian, Dengqiang Lin, Xing Xie, Yunze Xu, Lieyu Xu, Li Meng, Yu Zhu
PURPOSE: Malignant pheochromocytoma (PCC) is a rare tumor with a very poor prognosis and no effective treatments. The aim of this study was to assess the efficacy of a novel second-generation synthetic heat-shock protein 90 (HSP90) inhibitor, NVP-AUY922, to treat malignant PCC in vitro and in vivo. MATERIALS AND METHODS: Cell Counting Kit-8 (CCK-8) and Transwell assays were used to assess the effects of NVP-AUY922 on the proliferation and migration of the PCC cell line PC12...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28449504/malignant-pheochromocytoma-in-the-anterior-mediastinum-with-sternal-invasion-a-case-report
#17
Mingzhi Song, Kebin Sun, Tian Xia, Lei Zhou, Yangyang Li, Zhe Sun, Yuchi Zhang, Xianbin Zhang, Ran Sun, Bo Chen, Qingwei Tan
Pheochromocytomas are catecholamine-producing neuroendocrine tumors that usually occur in the adrenal medulla or sympathetic paraganglia. Anterior mediastinum involvement with pheochromocytoma is rare and may not present with typical symptoms. Its clinical manifestation may be unclear and a high index of suspicion is required for accurate diagnosis. We report a rare case of pheochromocytoma of the anterior mediastinum in a 51-year-old female. A painful hard mass on the sternum was the only clinical manifestation...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28429074/ultrastructure-of-pheochromocytoma-undescribed-morphologic-features
#18
A Beiras Fernandez, A Kornberger, M Fraga, C F Vahl, A Beiras
We examined samples of human pheochromocytoma from 11 patients aged 30-70 years including one case of malignant pheochromocytoma with a view to identifying previously unreported ultrastructural details.We identified two types of nuclear inclusions consisting of irregularly shaped singular or multiple granulofibrillar formations with a typical concentric halo, on the one hand, and accumulations of egg-shaped structures consisting of granules and microfilaments, on the other. In some of the tumor cells, membrane-covered inclusions containing parallel laminar elements arranged in a paracrystalline, periodic fashion, or mega-mitrochondriae characterized by increased electrodensity of their matrix, and fibrillary material in the spaces between the cristae were present...
April 20, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28413794/comparative-evaluation-of-iodine-131-metaiodobenzylguanidine-and-18-fluorodeoxyglucose-positron-emission-tomography-in-assessing-neural-crest-tumors-will-they-play-a-complementary-role
#19
Soumyakanti Kundu, Purushottam Kand, Sandip Basu
BACKGROUND: 18-Fluorodeoxyglucose positron emission tomography (FDG-PET) has established a role in the evaluation of several malignancies. However, its precise clinical role in the neural crest cell tumors continues to evolve. PURPOSE: The purpose of this study was to compare iodine-131 metaiodobenzylguanidine ((131)I-MIBG) and FDG-PET of head to head in patients with neural crest tumors both qualitatively and semiquantitatively and to determine their clinical utility in disease status evaluation and further management...
January 2017: South Asian Journal of Cancer
https://www.readbyqxmd.com/read/28396811/giant-cystic-pheochromocytoma-with-low-risk-of-malignancy-a-case-report-and-literature-review
#20
Ravi Maharaj, Sangeeta Parbhu, Wesley Ramcharan, Shanta Baijoo, Wesley Greaves, Dave Harnanan, Wayne A Warner
Giant pheochromocytomas are rare silent entities that do not present with the classical symptoms commonly seen in catecholamine-secreting tumors. In many cases they are accidentally discovered. The algorithm to diagnose a pheochromocytoma consists of biochemical evaluation and imaging of a retroperitoneal mass. The female patient in this case report presented with a palpable abdominal mass and was cured with surgical resection. She suffered no recurrence or complications on follow-up. The left retroperitoneal mass measured 27 × 18 × 12 cm and weighed 3,315 grams...
2017: Case Reports in Oncological Medicine
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