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Malignant pheochromocytoma

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https://www.readbyqxmd.com/read/28199934/mixed-corticomedullary-adrenal-carcinoma-case-report-comparison-in-features-treatment-and-prognosis-with-the-other-two-reported-cases
#1
Mhd Belal Alsabek, Riad Alhmaidi, Bader Ghazzawi, Ghiath Hamed, Alhadi Alseoudi
INTRODUCTION: Adrenal corticomedullary adenoma was reviewed in many cases in PubMed Library, While the coincidence corticomedullary adrenal carcinoma in the same gland was just described in two cases in the medical literature. Our case is the third to be reported and was treated with surgery and adjuvant chemotherapy and followed for two years. PRESENTATION OF CASE: A 50-year-old man suffered from a mass effect in the left abdominal side. While the laboratory showed a mild elevation in the levels of both serum cortisol and 24h urine cortisol, radiological images were highly suggested an adrenal malignant tumor without metastasis...
January 31, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28197025/current-perioperative-management-of-pheochromocytomas
#2
REVIEW
Rashmi Ramachandran, Vimi Rewari
Neuroendocrine tumors which have the potential to secrete catecholamines are either associated with sympathetic adrenal (pheochromocytoma) or nonadrenal (paraganglioma) tissue. Surgical removal of these tumors is always indicated to cure and prevent cardiovascular and other organ system complications associated with catecholamine excess. Some of these tumors have malignant potential as well. The diagnosis, localization and anatomical delineation of these tumors involve measurement of catecholamines and their metabolic end products in plasma and urine, (123)I-metaiodobenzylguanidine scintigraphy, computed tomography, and/or magnetic resonance imaging...
January 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28196587/pheochromocytomas-and-paragangliomas-genetically-diverse-and-minimalist-all-at-once
#3
Patricia L M Dahia
Pheochromocytomas and paragangliomas are infrequent, genetically heterogeneous neuroendocrine tumors. In this issue of Cancer Cell, Fishbein et al. report novel driver genes, pathogenic mechanisms, and markers of malignancy. Notably, the new findings support the long-held view that these tumors distinctively arise from a single driver event, either inherited or acquired.
February 13, 2017: Cancer Cell
https://www.readbyqxmd.com/read/28187001/molecular-markers-of-paragangliomas-pheochromocytomas
#4
REVIEW
Svetlana O Zhikrivetskaya, Anastasiya V Snezhkina, Andrew R Zaretsky, Boris Y Alekseev, Anatoly V Pokrovsky, Alexander L Golovyuk, Nataliya V Melnikova, Oleg A Stepanov, Dmitry V Kalinin, Alexey A Moskalev, George S Krasnov, Alexey A Dmitriev, Anna V Kudryavtseva
Paragangliomas/pheochromocytomas comprise rare tumors that arise from the extra-adrenal paraganglia, with an incidence of about 2 to 8 per million people each year. Approximately 40% of cases are due to genetic mutations in at least one out of more than 30 causative genes. About 25-30% of pheochromocytomas/paragangliomas develop under the conditions of a hereditary tumor syndrome a third of which are caused by mutations in the VHL gene. Together, the gene mutations in this disorder have implicated multiple processes including signaling pathways, translation initiation, hypoxia regulation, protein synthesis, differentiation, survival, proliferation, and cell growth...
February 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28181818/recommendations-for-the-management-of-adrenal-incidentalomas-what-is-pertinent-for-radiologists
#5
Anju Sahdev
Adrenal incidentalomas are unsuspected, asymptomatic adrenal masses detected on imaging. Most are non-functioning benign adrenocortical adenomas, but can represent other benign lesions or lesions requiring therapeutic intervention including adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis. This review summarises and highlights radiological recommendations within the recently issued guidelines for the management of adrenal incidentalomas from European Society of Endocrinology Clinical Practice in collaboration with European Network for Study of Adrenal Tumours...
February 9, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28134695/multiple-endocrine-neoplasia-type-2b-unmasked-by-18f-fdg-pet-ct-and-131i-mibg-spect-ct
#6
Xun Sun, Maher Mohamad Rajab Arnous, Xiaoli Lan
F-FDG PET/CT was performed to detect an occult malignancy in a 26-year-old woman with complicated medical history which included paroxysmal hypertension and significantly elevated tumor marker. The images revealed lesions in the thyroid, lymph nodes, and bilateral adrenal glands. Further I-MIBG SPECT/CT revealed intense activity in the lesion in the left adrenal gland, which was consistent with pheochromocytoma. The pathology examination after subsequent neck biopsy demonstrated medullary thyroid carcinoma...
January 27, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28121933/successful-treatment-of-metastatic-pheochromocytoma-in-the-spine-with-cement-augmentation
#7
Siyi Cai, Xiangyi Kong, Chengrui Yan, Yong Liu, Xi Zhou, Guixing Qiu
Metastatic pheochromocytoma in the spine is rare, and there is no standard curative management. Treatment via open surgery is often risky in the perioperative period, while osteoplasty by cement augmentation is a less invasive option.We describe 2 patients with recurrence of pheochromocytoma involving the spine and the pelvis who were successfully treated with osteoplasty by cement augmentation. A 31-year-old female underwent cement augmentation for a pelvic lesion 6 months after the resection and screw fixation of an L3 lesion...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28108930/controlling-tumor-progression-with-cyclophosphamide-vincristine-and-dacarbazine-treatment-improves-survival-in-patients-with-metastatic-and-unresectable-malignant-pheochromocytomas-paragangliomas
#8
Shiko Asai, Takuyuki Katabami, Mika Tsuiki, Yasushi Tanaka, Mitsuhide Naruse
Evidence has not been established to support that combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) improves survival in patients with malignant pheochromocytoma and paraganglioma (M-PPGL). To investigate the efficacy of CVD for this disease, we retrospectively analyzed data of 23 patients with metastatic and unresectable M-PPGL (mean age, 41.7 ± 15.4 years) who received at least 2 cycles of this regimen. The follow-up period after initiation of CVD ranged from 0.3 to 13...
January 20, 2017: Hormones & Cancer
https://www.readbyqxmd.com/read/28081522/adrenal-giant-cystic-pheochromocytoma-treated-by-posterior-retroperitoneoscopic-adrenalectomy
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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