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Pheochromocytoma and extra-adrenal

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https://www.readbyqxmd.com/read/29144820/pheochromocytoma-a-genetic-and-diagnostic-update
#1
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/29121733/pheochromocytoma-as-a-reversible-cause-of-cardiomyopathy-analysis-and-review-of-the-literature
#2
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/29120654/-diagnosis-and-management-of-metastatic-pheochromocytoma-and%C3%A2-paraganglioma
#3
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/29113559/proceedings-of-the-2017-national-toxicology-program-satellite-symposium
#4
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/29021924/large-unilateral-adrenal-mass-with-surrounding-brown-fat-a-case-report
#5
Gabriel O Ologun, Zinal M Patel, Navpreet K Rana, Andrew Trecartin, Alice Shen, Douglas Trostle, David Bertsch
Pheochromocytomas are rare tumors derived from chromaffin cells located in the adrenal and extra adrenal tissues. Pheochromocytomas are diagnosed biochemically and localized using different imaging modalities. The definitive management is surgical resection. Brown adipose tissues are normally present during fetal development, with regression over time. Brown adipose tissues are thermogenic and usually located in the neck, mediastinum, and retroperitoneum. Here, we report a case of a unilateral pheochromocytoma surrounded by brown fat...
August 9, 2017: Curēus
https://www.readbyqxmd.com/read/28954983/a-phase-i-clinical-trial-for-131-i-meta-iodobenzylguanidine-therapy-in-patients-with-refractory-pheochromocytoma-and-paraganglioma-a-study-protocol
#6
Anri Inaki, Kenichi Yoshimura, Toshinori Murayama, Yasuhito Imai, Yoshikazu Kuribayashi, Tetsuya Higuchi, Megumi Jinguji, Tohru Shiga, Seigo Kinuya
Objective Pheochromocytoma and paraganglioma (PPGLs) are rare neuroendocrine tumors derived from the adrenal medulla or extra-adrenal paraganglioma from extra-adrenal chromaffin tissue. Although malignant PPGLs has miserable prognosis, the treatment strategy remains to be established. An internal radiation therapy using [(131)I]meta-iodobenzylguanidine ((131)I-mIBG) called MIBG therapy has been attempted as one of the systemic treatment of malignant PPGLs. The aim of this study is therefore to evaluate the safety and the efficacy of MIBG therapy for refractory PPGLs...
2017: Journal of Medical Investigation: JMI
https://www.readbyqxmd.com/read/28953019/pheochromocytoma-of-the-bladder
#7
Paige Williams, Larry Siref, Mike Feloney
Pheochromocytoma is a rare catecholamine-secreting tumor. Unless recognized and treated appropriately it can be lethal. Common symptoms include sustained or paroxysmal hypertension associated with headaches, sweating, or palpitations. This article describes a patient with an extra-adrenal pheochromocytoma who presented with symptoms associated with voiding.
October 2017: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/28944243/clinical-management-of-pheochromocytoma-and-paraganglioma-in-singapore-missed-opportunities-for-genetic-testing
#8
Winston Hong Wern Chew, Eliza Courtney, Kok Hing Lim, Shao Tzu Li, Yanni Chen, Min Han Tan, Alexander Chung, Joan Khoo, Amos Loh, Shui Yen Soh, Prasad Iyer, Lih Ming Loh, Joanne Ngeow
BACKGROUND: Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors of the adrenal glands and paraganglia, occurring sporadically or as a range of hereditary tumor syndromes. About 30% of PPGLs are attributed to germline mutations. Clinical presentation, including localization, malignant potential, and age of onset, varies depending on the genetic background. Genetic testing for PPGLs is not well studied in Southeast Asia. We reviewed clinical management of PPGLs in Singapore, highlighting current gaps in clinical practice...
September 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/28944143/laparoscopic-resection-of-ectopic-pheochromocytoma
#9
Hanhui Cai, Yuhua Zhang, Zhiming Hu
Ectopic pheochromocytoma (EP) is considered as pheochromocytoma located at extra-adrenal site. Surgical removal is believed to be the best choice for treatment of pheochromocytoma. We present a series EP resected by laparoscopic approach (LEP) and confirm its feasibility. We retrospectively reviewed clinical data of 4 patients underwent laparoscopic resection of LEP (periaortocaval EP, n = 1; retroperitoneal EP, n = 2; bladder EP, n = 1), which was collected and analyzed retrospectively in Zhejiang Provincial People's Hospital...
August 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28931294/-carney-triad
#10
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/28884434/clinical-predictors-of-malignancy-in-patients-with-pheochromocytoma-and-paraganglioma
#11
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...
November 2017: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/28799639/paraganglioma-of-the-renal-pelvis-a-case-report-and-review-of-literature
#12
Cheng Yi, Lin Han, Rui Yang, Junfeng Yu
PURPOSE: Paraganglioma, a kind of pheochromocytoma originating from embryonic neural crest, is a rare neuroendocrine neoplasm commonly located at extra-adrenal areas such as head, neck, and abdomen. There are a few reports on renal paragangliomas; fewer than 5 reported cases are renal pelvic paragangliomas, including our case. METHODS: Our patient, who had not experienced headache, hypertension, or palpitation, was founded to have a fixed mass in the left renal pelvis incidentally...
August 7, 2017: Tumori
https://www.readbyqxmd.com/read/28770279/-treatment-of-an-uncommon-case-of-a-cardiogenic-shock-simultaneous-use-of-a-va-ecmo-and-an-impella-cp%C3%A2
#13
H Haake, K Grün-Himmelmann, U Kania, F Trudzinski, P M Lepper, J Vom Dahl
We report the case of a 48-year old woman where probably the intramuscular administration of glucocorticoids by an orthopedist induced a pheochromocytoma crisis. The development of a cardiogenic shock with a cardiac arrest made the use of a venoarterial extra corporeal membrane oxygenation (VA-ECMO, Cardiohelp®, Maquet, Rastatt) necessary. To treat a pulmonary edema under VA-ECMO an Impella-CP® (Abiomed, Aachen) was implanted. A coronary angiography, endomyocardial biopsies and a computer tomography were performed...
August 2, 2017: Medizinische Klinik, Intensivmedizin und Notfallmedizin
https://www.readbyqxmd.com/read/28585398/genetic-status-determines-18-f-fdg-uptake-in-pheochromocytoma-paraganglioma
#14
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
#15
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
#16
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/28515046/kif1b-and-nf1-are-the-most-frequently-mutated-genes-in-paraganglioma-tumors
#17
Lucie Evenepoel, Raphaël Helaers, Laurent Vroonen, Selda Aydin, Marc Hamoir, Dominique Maiter, Miikka Vikkula, Alexandre Persu
Dear Editor, Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors arising from the adrenal medulla and extra-adrenal paraganglia, respectively. Forty percent are explained by germline mutations in known susceptibility genes. Furthermore, somatic mutations were identified in an additional 30% of PPGL, mostly in NF1, RET, VHL, MAX, and HRAS. However, screening is limited to a few studies mainly using Sanger sequencing (Burnichon, et al. 2012; Burnichon, et al. 2011; Crona, et al. 2013; Luchetti, et al...
May 17, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28503760/the-penetrance-of-paraganglioma-and-pheochromocytoma-in-sdhb-germline-mutation-carriers
#18
Johannes A Rijken, Nicolasine D Niemeijer, Marianne A Jonker, Karin Eijkelenkamp, Jeroen C Jansen, Anouk van Berkel, Henri J L M Timmers, Henricus P M Kunst, Peter H L T Bisschop, Michiel N Kerstens, Koen M A Dreijerink, Marieke F van Dooren, Anouk N A van der Horst-Schrivers, Frederik J Hes, C René Leemans, Eleonora P M Corssmit, Erik F Hensen
Germline mutations in SDHB predispose to hereditary paraganglioma syndrome type 4. The risk of developing paraganglioma (PGL) or pheochromocytoma (PHEO) in SDHB mutation carriers is subject of recent debate. In the present nationwide cohort study of SDHB mutation carriers identified by the clinical genetics centers of the Netherlands, we have calculated the penetrance of SDHB associated tumors using a novel maximum likelihood estimator. This estimator addresses ascertainment bias and missing data on pedigree size and structure...
May 14, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28491185/extra-adrenal-pheochromocytoma-at-the-organ-of-zuckerkandl-a-case-report-and-literature-review
#19
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/28481789/comparison-of-the-68ga-dotatata-pet-ct-fdg-pet-ct-and-mibg-spect-ct-in-the-evaluation-of-suspected-primary-pheochromocytomas-and-paragangliomas
#20
Hongli Jing, Fang Li, Ling Wang, Zhenghua Wang, Wei Li, Li Huo, Jingjing Zhang
Anatomical imaging modalities including CT and MRI are the mainstay of evaluation of primary pheochromocytoma or paraganglioma. However, nuclear medicine imaging is frequently necessary to determine the nature of the lesions. The purpose of this investigation is to assess which commonly used nuclear medicine modality might have a better diagnostic value in this clinical setting. METHODS: Eight patients who had been suspected of having either primary pheochromocytoma or primary paraganglioma and 1 patient with known pheochromocytoma were included in the analysis...
July 2017: Clinical Nuclear Medicine
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