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Anna Angelousi, Georgios K Dimitriadis, Georgios Zografos, Svenja Nölting, Gregory Kaltsas, and Ashley Grossman

carcinomas, as well as evidence from current clinical applications of molecular targeted treatment (MTT). Phaeochromocytomas and paragangliomas Introduction Phaeochromocytomas (PCs) arise from the adrenal chromaffin cells, whereas paragangliomas

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Jay S Fonte, Jeremyjones F Robles, Clara C Chen, James Reynolds, Millie Whatley, Alexander Ling, Leilani B Mercado-Asis, Karen T Adams, Victoria Martucci, Tito Fojo, and Karel Pacak

Introduction Pheochromocytoma (PHEO) and paragangliomas (PGLs) are tumors arising from chromaffin cells of the adrenal medulla or extraadrenal paraganglionic tissues respectively ( DeLellis et al . 2004 ). These tumors express the cellular membrane

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Tomáš Zelinka, Henri J L M Timmers, Anna Kozupa, Clara C Chen, Jorge A Carrasquillo, James C Reynolds, Alexander Ling, Graeme Eisenhofer, Ivica Lazúrová, Karen T Adams, Millie A Whatley, Jiří Widimský Jr, and Karel Pacak

Introduction Pheochromocytomas (PHEO) are catecholamine-producing tumors arising from chromaffin cells of the adrenal medulla. Paragangliomas (PGL) are tumors arising either from extra-adrenal chromaffin cells, and can originate either from

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Nicola Tufton, Rahul Ghelani, Umasuthan Srirangalingam, Ajith V Kumar, William M Drake, Donato Iacovazzo, Kassiani Skordilis, Daniel Berney, Ma’en Al-Mrayat, Bernard Khoo, and Scott A Akker

associated phenotypes. We suggest that SDHA paraganglioma-related disease is not a low-risk condition as first described. Of our six index cases, two developed metastatic disease and a further one had local vascular invasion. One patient developed multiple

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Noriko Kimura, Kazuhiro Takekoshi, Akira Horii, Ryo Morimoto, Tsuneo Imai, Yutaka Oki, Tomohito Saito, Sanae Midorikawa, Tadashi Arao, Chiho Sugisawa, Masanobu Yamada, Yuichi Otuka, Isao Kurihara, Kokichi Sugano, Minoru Nakane, Atsushi Fukuuchi, Takumi Kitamoto, Jun Saito, Tetsuo Nishikawa, and Mitsuhide Naruse

Dear Editor Pheochromocytoma (PCC) and paraganglioma (PGL) are genetically and phenotypically heterogeneous catecholamine-producing neoplasms. They can occur sporadically or as a part of hereditary disease. Approximately 30% of PCC/PGL are believed

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Alberto Cascón, Lucía Inglada-Pérez, Iñaki Comino-Méndez, Aguirre A de Cubas, Rocío Letón, Jaume Mora, Mónica Marazuela, Juan Carlos Galofré, Miguel Quesada-Charneco, and Mercedes Robledo

Dear Editor: Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors that arise from the adrenal medulla or from the extra-adrenal sympathetic and parasympathetic paraganglia respectively. Now we know that more than 30% of

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Raquel G Martins, Joana B Nunes, Valdemar Máximo, Paula Soares, Joana Peixoto, Telmo Catarino, Teresa Rito, Pedro Soares, Luísa Pereira, Manuel Sobrinho-Simões, Ana Paula Santos, Joana Couto, Rui Henrique, Joana Matos-Loureiro, Paula Dias, Isabel Torres, and Jorge Lima

Dear Editor We would like to report a genetic screening of SDHB , SDHC , SDHD and SDHAF2 genes (hereafter abbreviated to SDHx ) in patients with paragangliomas (PGL) and phaeochromocytomas (PCC) from northern Portugal. PGL and PCC are

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R Vassilopoulou-Sellin

Background: Tumors of the paraganglionic system represent a distinct, albeit uncommon, clinical entity characterized by catecholamine hypersecretion and hemodynamic instability; initial pathologic examination often cannot predict benign vs malignant behavior. An analysis of the clinical outcome of patients with known malignant tumors may serve to enhance the initial evaluation and therapeutic plan of all patients presenting with pheochromocytoma or paraganglioma.

Methods: At the University of Texas M D Anderson Cancer Center, 30 patients with malignant abdominal paraganglioma and 20 patients with malignant pheochromocytoma were diagnosed between 1971 and 1995. Their medical records were reviewed with particular attention to clinical characteristics and disease outcome.

Results: Among the 30 patients with paraganglioma, 73% were men, and 90% were younger than 50 years at the time of diagnosis. Sixteen patients have remained alive with persistent disease 0.2 to 25 years after initial diagnosis while eight patients died of their disease within 0.8 to 32 years. Regional recurrence and skeletal metastases were the most prominent events. Among the 20 patients with pheochromocytoma, 60% were men and 70% were younger than 50 years at the time of diagnosis. Ten patients have remained alive with persistent disease 0.8 to 20 years after initial diagnosis while five patients died of their disease within 1.5 to 39 years. Hypertension was a prominent presenting feature and regional recurrence was the most frequent pattern of treatment failure.

Conclusions: Important clinical differences distinguish adrenal pheochromocytomas from extra-adrenal, abdominal paragangliomas. Patients with paragangliomas are, as a group, younger men, more likely to have malignant lesions and a more aggressive clinical course. Patients with malignant pheochromocytomas usually present with hypertension, are somewhat older, and have less aggressive disease.


We thank the staff of the Department of Medical Informatics for database retrieval and the clinical faculty who participated in the patients' care. We thank Teo Spear for expert preparation of the manuscript. We thank Terry Smith, biostatistician, for her critical review and suggestions.

Open access

Ruth T Casey, Benjamin G Challis, Alison Marker, Deborah Pitfield, Heok K Cheow, Ashley Shaw, Soo-Mi Park, Helen L Simpson, and Eamonn R Maher

Dear Editor, We have read with great interest the recent article by Tufton and coworkers reporting on the risk of metastasis in patients with paraganglioma (PGL) tumours associated with germline SDHA mutations ( Tufton et al . 2017 ). Herein

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Thibault Bahougne, Alessio Imperiale, Gerlinde Averous, Gerard Chabrier, Nelly Burnichon, Anne Paule Gimenez-Roqueplo, Nassim Dali-Youcef, Rossella Libe, Eric Baudin, Catherine Roy, Herve Lang, and Laurence Kessler

Dear Editor, Pheochromocytoma (PHEO) and extra-adrenal paraganglioma (PGL) are neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the autonomic nervous system, respectively. These neoplasms are malignant in