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Malignant pheochromocytoma

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https://www.readbyqxmd.com/read/28081522/adrenal-giant-cystic-pheochromocytoma-treated-by-posterior-retroperitoneoscopic-adrenalectomy
#1
Carlos E Costa Almeida, Marta Silva, Luis Carvalho, Carlos Manuel Costa Almeida
INTRODUCTION: Giant cystic pheochromocytoma (>10cm) is rare with only a few cases described in the literature. Preoperative diagnosis is very difficult because clinical, biochemical and radiologic finds are usually not consistent with a pheochromocytoma. Open surgery is traditionally the gold standard. CASE PRESENTATION: A 51 year-old male patient resorted to surgery department with an adrenal cystic incidentaloma. He was asymptomatic, mild hypertension easily controlled, with increased plasma fractionated metanephrines...
December 27, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28063406/contemporary-imaging-of-incidentally-discovered-adrenal-masses
#2
REVIEW
Daniel J Wale, Ka Kit Wong, Benjamin L Viglianti, Domenico Rubello, Milton D Gross
Adrenal lesions are routinely encountered incidentally in clinical practice. Although most of these lesions are benign, malignancy needs to be excluded. Therefore, the initial clinical workup is to exclude aggressive characteristics suggesting malignancy and to identify characteristics predictive of the most common benign lesion, an adrenal adenoma. Predicting a benign adenoma using a variety of imaging modalities has been widely studied using unenhanced computed tomography (CT), contrast enhanced CT, and magnetic resonance (MR) imaging...
January 4, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28058556/surgical-treatment-of-malignant-pheochromocytoma-and-paraganglioma-retrospective-case-series
#3
Veljko Strajina, Benzon M Dy, David R Farley, Melanie L Richards, Travis J McKenzie, Keith C Bible, Florencia G Que, David M Nagorney, William F Young, Geoffrey B Thompson
INTRODUCTION: Pheochromocytoma and paraganglioma (PPGL) are rare neoplasms; about 10% are malignant. Literature regarding possible benefit from resection is extremely limited. METHODS: A 20 year review of all patients undergoing surgery for malignant PPGL at the Mayo Clinic Rochester Campus between 1994 and June 2014 was performed. RESULTS: We identified 34 patients undergoing surgery for malignant PPGL. Median follow up was 6 and 5 years survival was 90% (median 11 years)...
January 5, 2017: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/28006088/case-of-parotid-mucoepidermoid-carcinoma-expanding-the-spectrum-of-von-hippel-lindau-related-neoplasms
#4
Michael H Berger, Darcy A Kerr, Artur E Rangel Filho, Zoukaa B Sargi
BACKGROUND: von Hippel-Lindau (VHL)-related tumors occurring outside the spectrum of VHL-defining tumors are rare, and mucoepidermoid carcinoma (MEC) in the setting of VHL disease has not been described. METHODS AND RESULTS: We describe a patient with confirmed VHL mutation who presented with a parotid mass and a history of 2 central nervous system (CNS) hemangioblastomas and 1 pheochromocytoma. Fine-needle aspiration (FNA) of the mass suggested a benign Warthin tumor...
December 22, 2016: Head & Neck
https://www.readbyqxmd.com/read/27992508/disentangling-of-malignancy-from-benign-pheochromocytomas-paragangliomas
#5
Kyong Young Kim, Jung Hee Kim, A Ram Hong, Moon-Woo Seong, Kyu Eun Lee, Su-Jin Kim, Sang Wan Kim, Chan Soo Shin, Seong Yeon Kim
OBJECTIVE: Many malignant tumors initially appear benign but subsequently exhibit extensive metastases. Early identification of malignant pheochromocytomas and paragangliomas (PPGLs) before metastasis is important for improved prognosis. However, there are no robust prognostic indices of recurrence and malignancy. The aim of this study was to identify the clinical and histopathological factors that predict malignant PPGLs. DESIGN: Retrospective follow-up study. METHODS: In this study, we included 223 patients with pathologically confirmed PPGLs who were treated between 2000 and 2015 at the Seoul National University Hospital in South Korea...
2016: PloS One
https://www.readbyqxmd.com/read/27988912/clinical-characteristics-of-adrenal-tumors-in-children-a-retrospective-review-of-a-15-year-single-center-experience
#6
Xiaokun Lin, Dazhou Wu, Congde Chen, Na Zheng
OBJECTIVE: Adrenal tumors are rare in children. The aim of this study is to review and analyze clinical data on the diagnosis and management of adrenal tumors in children. METHODS: Between 2001 and 2015, 48 pediatric patients (<14 years old) admitted to our institute with adrenal tumors were reviewed. Clinical features, imaging studies, surgical approaches, as well as pathological diagnoses were recorded. RESULTS: The series comprised 28 males and 20 females...
December 17, 2016: International Urology and Nephrology
https://www.readbyqxmd.com/read/27913608/the-clinical-utility-of-circulating-neuroendocrine-gene-transcript-analysis-in-well-differentiated-paragangliomas-and-pheochromocytomas
#7
M Pęczkowska, J Cwikla, M Kidd, A Lewczuk, A Kolasinska-Ćwikła, D Niec, I Michałowska, A Prejbisz, A Januszewicz, J Chiarelli, L Bodei, I Modlin
CONTEXT: Paragangliomas and pheochromocytomas (PPGLs) exhibit variable malignancy, which is difficult to determine by histopathology, amine measurements or tissue genetic analyses. OBJECTIVE: To evaluate whether a 51-neuroendocrine gene blood analysis has clinical utility as a diagnostic and prognostic marker. DESIGN: Prospective cohort study. Well-differentiated PPGLs (n = 32), metastatic (n = 4); SDHx mutation (n = 25); 12 biochemically active, Lanreotide treated (n = 4)...
February 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27894345/ultrasonographic-features-of-adrenal-gland-lesions-in-dogs-can-aid-in-diagnosis
#8
Elena Pagani, Massimiliano Tursi, Chiara Lorenzi, Alberto Tarducci, Barbara Bruno, Enrico Corrado Borgogno Mondino, Renato Zanatta
BACKGROUND: Ultrasonography to visualize adrenal gland lesions and evaluate incidentally discovered adrenal masses in dogs has become more reliable with advances in imaging techniques. However, correlations between sonographic and histopathological changes have been elusive. The goal of our study was to investigate which ultrasound features of adrenal gland abnormalities could aid in discriminating between benign and malignant lesions. To this end, we compared diagnosis based on ultrasound appearance and histological findings and evaluated ultrasound criteria for predicting malignancy...
November 28, 2016: BMC Veterinary Research
https://www.readbyqxmd.com/read/27873108/flushing-in-neuro-endocrinology
#9
REVIEW
Fady Hannah-Shmouni, Constantine A Stratakis, Christian A Koch
Cutaneous flushing is a common presenting complaint in endocrine disorders. The pathophysiology of flushing involves changes in cutaneous blood flow triggered by multiple intrinsic factors that are either related to physiology or disease. Flushing can be divided into episodic or persistent causes. Episodic flushing is mediated by the release of endogenous vasoactive mediators or medications, while persistent flushing results in a fixed facial erythema with telangiectasia and cyanosis due to slow-flowing deoxygenated blood in large cutaneous blood vessels...
September 2016: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/27869719/targeted-molecular-imaging-in-adrenal-disease-an-emerging-role-for-metomidate-pet-ct
#10
REVIEW
Iosif A Mendichovszky, Andrew S Powlson, Roido Manavaki, Franklin I Aigbirhio, Heok Cheow, John R Buscombe, Mark Gurnell, Fiona J Gilbert
Adrenal lesions present a significant diagnostic burden for both radiologists and endocrinologists, especially with the increasing number of adrenal 'incidentalomas' detected on modern computed tomography (CT) or magnetic resonance imaging (MRI). A key objective is the reliable distinction of benign disease from either primary adrenal malignancy (e.g., adrenocortical carcinoma or malignant forms of pheochromocytoma/paraganglioma (PPGL)) or metastases (e.g., bronchial, renal). Benign lesions may still be associated with adverse sequelae through autonomous hormone hypersecretion (e...
November 18, 2016: Diagnostics
https://www.readbyqxmd.com/read/27865588/pediatric-patients-with-pheochromocytoma-and-paraganglioma-should-have-routine-preoperative-genetic-testing-for-common-susceptibility-genes-in-addition-to-imaging-to-detect-extra-adrenal-and-metastatic-tumors
#11
Bruna Babic, Dhaval Patel, Rachel Aufforth, Yasmine Assadipour, Samira M Sadowski, Martha Quezado, Naris Nilubol, Tamara Prodanov, Karel Pacak, Electron Kebebew
BACKGROUND: Pediatric pheochromocytomas and paragangliomas are rare with limited data on the optimal management approach. The aim of this study was to determine the role of genetic testing and imaging to detect extra-adrenal and/or metastatic tumors in pediatric pheochromocytomas and paragangliomas. METHODS: We performed a retrospective study of 55 patients diagnosed at ≤21 years of age with pheochromocytomas and paragangliomas with analysis of data on genetic testing and multimodal imaging...
January 2017: Surgery
https://www.readbyqxmd.com/read/27852633/predictors-of-malignancy-in-patients-with-pheochromocytomas-paragangliomas-asian-indian-experience
#12
Kranti Khadilkar, Vijaya Sarathi, Rajeev Kasaliwal, Reshma Pandit, Manjunath Goroshi, Gaurav Malhotra, Abhay Dalvi, Ganesh Bakshi, Anil Bhansali, Rajesh Rajput, Vyankatesh Shivane, Anurag Lila, Tushar Bandgar, Nalini S Shah
BACKGROUND AND AIMS: Malignant transformation of pheochromocytomas/paragangliomas (PCC/PGL) is a rare occurrence, and predictive factors for the same are not well understood. This study aims to identify the predictors of malignancy in patients with PCC/PGL. MATERIALS AND METHODS: We performed a retrospective analysis of 142 patients with either PCC or PGL registered at our institute between 2000 and 2015. Records were evaluated for clinical parameters like age, gender, familial/syndromic presentation, symptomatic presentation, biochemistry, size, number and location of tumours and presence of metastases and mode of its diagnosis...
November 2016: Endocrine Connections
https://www.readbyqxmd.com/read/27833956/diagnostic-reevaluation-of-17-cases-of-pheochromocytoma-a-retrospective-study
#13
Nora Lupşan, Liliana Resiga, Adina Bianca Boşca, Carmen Georgiu, DoiniŢa Crişan, Claudiu Mirescu, Anne Marie Constantin, Ioan Şimon, Alina Simona Şovrea
A rare neuroendocrine tumor, the pheochromocytoma (PCC) raises problems due both the limited experience of the researchers in this field and its pathogenic mechanisms, still not fully elucidated. The malignant potential of this tumor cannot be predicted based on its macro- or microscopic aspects, but on the presence of metastases. The aims of this study were: (1) the reevaluation of data for a pertinent and complete tumor diagnostic and prognostic pattern; (2) the statistical correlation of all investigated parameters with the malignant form and the survival rate in order to obtain a possible predictor of malignancy; (3) the potential identification of initially diagnosed benign tumors that become malignant in time...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/27824970/a-pediatric-malignant-paraganglioma-and-brief-review-of-the-literature
#14
Jinhua Zi, Chao Ma, Changyun Xu, Yongli Bai
: A 10 years old boy presented to our hospital complaining of belly pain. He had a retroperitoneal mass diagnosed by ultrasound 3 days before. During that time he was found to have slight hypertension. Computed tomography (CT) showed a left retroperitoneal mass with edge enhancement and central necrosis indicating pheochromocytoma (PCC). Vanillymandelic acid (VMA) in the 24 hours urine sample was not elevated. Pheochromocytoma was suspected given his hypertension and the ultrasound and CT findings...
September 2016: Hellenic Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/27821293/metastatic-pheochromocytoma-to-liver-without-elevation-of-metanephrines-and-catecholamines
#15
Javier López-Gómez, Ma Alejandra Salazar-Álvarez, Rodrigo Y Adame, Alejandro Alfaro-Goldaracena, Erwin R Flores-Vazquez, Sergio H Gonzalez-Infante, Alejandro E Padilla-Rosciano, Horacio N López-Basave
INTRODUCTION: Malignant pheochromocytoma represents 10% of all patients with pheochromocytoma. Of these cases, only 5-9% presents without elevation of metanephrines and catecholamines. PRESENTATION OF CASE: A 43-year-old female patient presented with an abdominal tumor. An exploratory laparotomy was performed and the final report was a pheochromocytoma. After ten years, multiple liver lesions were detected and surgical treatment was performed. Pathological evaluation revealed a malignant pheochromocytoma with negative margins after 5 years of follow-up without evidence of disease...
2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27812903/expanding-the-horizon-of-robotic-surgery-to-large-pelvic-paraganglioma
#16
Santosh Kumar, M Pragatheeswarane, Aditya Prakash Sharma, Kshitij Bishnoi, Manjeet Kumar Sharma, Vikas Kumar Panwar, Sameer Sethi
INTRODUCTION: Paragangliomas are the most common extra-adrenal pheochromocytoma. Symptomatic and growing tumors demand surgical extirpation. Minimally invasive techniques allow surgeons to perform the procedure without wide exposure and mobilization of intra-abdominal organs. To our knowledge, we present the fourth case of robotic excision of a large retroperitoneal paraganglioma. MATERIALS AND METHODS: An 18-year-old unmarried female presented with occipital headache for past 3 years...
November 3, 2016: Journal of Robotic Surgery
https://www.readbyqxmd.com/read/27811202/targeting-pi3k-mtor-signaling-exerts-potent-antitumor-activity-in-pheochromocytoma-in-vivo
#17
Misu Lee, Ninelia Minaskan, Tobias Wiedemann, Martin Irmler, Johannes Backers, Behrooz H Yousefi, Georgios Kaissis, Rickmer Braren, Iina Laitinen, Natalia S Pellegata
Pheochromocytomas (PCCs) are mostly benign tumors, amenable to complete surgical resection. However, 10-17% of cases can become malignant, and once metastasized, there is no curative treatment for this disease. Given the need to identify effective therapeutic approaches for PCC, we evaluated the antitumor potential of the dual PI3K/mTOR inhibitor BEZ235 against these tumors. We employed an in vivo model of endogenous PCCs (MENX mutant rats), which closely recapitulate the human tumors. Mutant rats with PCCs were treated with 2 doses of BEZ235 (20 and 30 mg/kg), or with placebo, for 2 weeks...
November 3, 2016: Endocrine-related Cancer
https://www.readbyqxmd.com/read/27810406/perioperative-management-of-adrenalectomy-and-inferior-vena-cava-reconstruction-in-a-patient-with-a-large-malignant-pheochromocytoma-with-vena-caval-extension
#18
Stephen H Gregory, Suraj M Yalamuri, Sharon L McCartney, Syed A Shah, Julie A Sosa, Sanziana Roman, Brian J Colin, Claude Lentschener, Ray Munroe, Saumil Patel, Jared W Feinman, John G T Augoustides
No abstract text is available yet for this article.
July 18, 2016: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/27790441/risk-stratification-in-paragangliomas-with-pass-pheochromocytoma-of-the-adrenal-gland-scaled-score-and-immunohistochemical-markers
#19
Maithili Mandar Kulkarni, Siddhi Gaurish Sinai Khandeparkar, Sanjay D Deshmukh, R R Karekar, Vandana L Gaopande, Avinash R Joshi, Mrunal V Kesari, R R Shelke
INTRODUCTION: Paragangliomas (PGLs) are rare tumours that arise in sympathetic and parasympathetic paraganglia and are derived from neural crest cells. Presence of metastasis is the only absolute criterion for malignancy. There is no single histo-morphological feature indicating malignant potential and multiple parameters have been proposed to prognosticate the individual case. This includes studies conducted using Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and Immunohistochemical (IHC) markers...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27753873/ish-aha-2-a-case-of-chronic-indolent-pheochromocytoma-which-caused-medically-controlled-hypertension-but-treatment-resistant-diabetes-mellitus
#20
Hae-Young Lee, Chan-Soon Park, Sang-Hoon Na, Kyung-Jin Kim, Chan Joo Lee, Sungha Park
A 47-year-old woman was admitted via emergency department due to dyspnea NYHA Fc II-III aggravated for 2 months after upper respiratory infection. Her height and body weight were 161 cm / 67 kg. Initial vital signs were 110/70 mmHg - 112 BPM - 24/min - 36.5°C. Chest PA showed cardiomegaly and pulmonary congestion (Figure 1). B-natriuretic peptide level was markedly increased (2002 pg/mL, normal range ≤ 100 pg/mL). The echocardiographic examination showed severely dilated LV cavity (61/72 mm) and severe LV systolic dysfunction (EF 28%) with normal left ventricular wall thickness (9/11 mm) (Figure 2)...
September 2016: Journal of Hypertension
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