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Hongyan Wang, Bin Wu, Zichuan Yao, Xianqing Zhu, Yunzhong Jiang and Song Bai

Introduction A pheochromocytoma is a rare neuro-endocrine tumor that arises from the chromaffin cells of the adrenal medulla with an incidence of 0.2–0.8 cases/100,000 persons/year, which present in 0.1–1% of patients with hypertension and

Open access

Joakim Crona, Alberto Delgado Verdugo, Dan Granberg, Staffan Welin, Peter Stålberg, Per Hellman and Peyman Björklund

Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare tumours arising from chromaffin cells in adrenal medulla and autonomous ganglia. A majority of these tumours have a low proliferation and seldom metastasize. The understanding

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Henrik Falhammar, Magnus Kjellman and Jan Calissendorff

Introduction Pheochromocytomas are neuroendocrine tumors arising from chromaffin cells of the adrenal medulla or in extra-adrenal paraganglia producing catecholamines. Tumors outside the adrenals are usually called extra

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Xinlei Chen, Liru Hu, Caojie Liu, Guangcheng Ni and Yuwei Zhang

Introduction Pheochromocytoma and paraganglioma (PPGL) are tumors composed of chromaffin cells, which commonly produce one or more catecholamines: epinephrine, norepinephrine and dopamine. These tumors are rarely silent, and they are

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Natalie Rogowski-Lehmann, Aikaterini Geroula, Aleksander Prejbisz, Henri J L M Timmers, Felix Megerle, Mercedes Robledo, Martin Fassnacht, Stephanie M J Fliedner, Martin Reincke, Anthony Stell, Andrzej Januszewicz, Jacques W M Lenders, Graeme Eisenhofer and Felix Beuschlein

Introduction Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors that derive from adrenomedullary tissue (pheochromocytomas) in about 85% of cases and from extra-adrenal chromaffin tissue (paragangliomas) in the remainder

Open access

Qiuli Liu, Gang Yuan, Dali Tong, Gaolei Liu, Yuting Yi, Jun Zhang, Yao Zhang, Lin-ang Wang, Luofu Wang, Dianzheng Zhang, Rongrong Chen, Yanfang Guan, Xin Yi, Weihua Lan and Jun Jiang

neuroendocrine tumor (NET) or cystadenoma, pheochromocytoma (Pheo) and clear cell renal cell carcinoma (RCC) ( 1 , 2 ). VHL disease is primarily caused by inactivation of the VHL tumor-suppressive protein. A dysfunctional VHL leads to reduced ubiquitylation and

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Caojie Liu, Qingguo Lv, Xinlei Chen, Guangcheng Ni, Liru Hu, Nanwei Tong and Yuwei Zhang

Introduction Pheochromocytomas and paragangliomas (PPGL) are rare catecholamine-secreting tumors with a low incidence of 0.8 per 100,000 individuals/year in the general population ( 1 ). However, the incidence among the hypertensive population

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Yiqiang Huang, Lin-ang Wang, Qiubo Xie, Jian Pang, Luofu Wang, Yuting Yi, Jun Zhang, Yao Zhang, Rongrong Chen, Weihua Lan, Dianzheng Zhang and Jun Jiang

Introduction Pheochromocytomas/paragangliomas (PCC/PGLs) are tumors, arose from neural crest-derived chromaffin cells, produce and secrete catecholamines ( 1 , 2 , 3 ). PCCs are tumors of the adrenal medulla and PGLs originate from

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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 and Nalini S Shah

Introduction Malignant pheochromocytoma and paraganglioma (PCC/PGL) comprise approximately 10–20% of all pheochromocytomas and paragangliomas ( 1 ). According to the World Health Organization classification, the only accepted criterion for

Open access

Rui M B Maciel, Cleber P Camacho, Lígia V M Assumpção, Natassia E Bufalo, André L Carvalho, Gisah A de Carvalho, Luciana A Castroneves, Francisco M de Castro Jr, Lucieli Ceolin, Janete M Cerutti, Rossana Corbo, Tânia M B L Ferraz, Carla V Ferreira, M Inez C França, Henrique C R Galvão, Fausto Germano-Neto, Hans Graf, Alexander A L Jorge, Ilda S Kunii, Márcio W Lauria, Vera L G Leal, Susan C Lindsey, Delmar M Lourenço Jr, Léa M Z Maciel, Patrícia K R Magalhães, João R M Martins, M Cecília Martins-Costa, Gláucia M F S Mazeto, Anelise I Impellizzeri, Célia R Nogueira, Edenir I Palmero, Cencita H C N Pessoa, Bibiana Prada, Débora R Siqueira, Maria Sharmila A Sousa, Rodrigo A Toledo, Flávia O F Valente, Fernanda Vaisman, Laura S Ward, Shana S Weber, Rita V Weiss, Ji H Yang, Magnus R Dias-da-Silva, Ana O Hoff, Sergio P A Toledo and Ana L Maia

(MEN2B; OMIM #162300), according to its clinical manifestations, characterized by a high penetrance of medullary thyroid carcinoma (MTC), and to a lesser degree, pheochromocytoma (PHEO) and primary hyperparathyroidism (PHPT) ( 1 , 2 , 3 , 4