Pheochromocytomas and paragangliomas

SG Tevosian, HK Ghayee - Endocrinology and Metabolism …, 2019 - endo.theclinics.com
Pheochromocytomas (PCCs) are rare neuroendocrine tumors. About 80% to 85% of these
cancers arise from chromaffin cells residing in the adrenal medulla. The remaining 15% to …

Intricacies of the molecular machinery of catecholamine biosynthesis and secretion by chromaffin cells of the normal adrenal medulla and in pheochromocytoma and …

AMA Berends, G Eisenhofer, L Fishbein… - Cancers, 2019 - mdpi.com
The adrenal medulla is composed predominantly of chromaffin cells producing and
secreting the catecholamines dopamine, norepinephrine, and epinephrine. Catecholamine …

A phase 2 trial of sunitinib in patients with progressive paraganglioma or pheochromocytoma: the SNIPP trial

GM O'Kane, S Ezzat, AM Joshua, I Bourdeau… - British journal of …, 2019 - nature.com
Background Pheochromocytoma (PCC) and paraganglioma (PGL) are uncommon
neoplasms with high morbidity in advanced stages. Effective systemic treatments are limited …

Mito-nuclear communication by mitochondrial metabolites and its regulation by B-vitamins

JJE Janssen, S Grefte, J Keijer, VCJ De Boer - Frontiers in physiology, 2019 - frontiersin.org
Mitochondria are cellular organelles that control metabolic homeostasis and ATP
generation, but also play an important role in other processes, like cell death decisions and …

Pheochromocytomas and paragangliomas: bypassing cellular respiration

A Cascón, L Remacha, B Calsina, M Robledo - Cancers, 2019 - mdpi.com
Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that
show the highest heritability of all human neoplasms and represent a paradoxical example …

Updates on the role of molecular alterations and NOTCH signalling in the development of neuroendocrine neoplasms

C von Arx, M Capozzi, E López-Jiménez… - Journal of clinical …, 2019 - mdpi.com
Neuroendocrine neoplasms (NENs) comprise a heterogeneous group of rare malignancies,
mainly originating from hormone-secreting cells, which are widespread in human tissues …

[HTML][HTML] The emerging role of succinate dehyrogenase genes (SDHx) in tumorigenesis

E Nazar, F Khatami, H Saffar… - International Journal of …, 2019 - ncbi.nlm.nih.gov
Transformation of a normal cell to cancerous one is dependent on the accumulation of
several genetic and epigenetic alterations. One of the candidate driver genetic alterations …

[HTML][HTML] A systematic review on the genetic analysis of paragangliomas: primarily focused on head and neck paragangliomas

A Guha, Z Musil, A Vícha, T Zelinka, K Pacák, J Astl… - Neoplasma, 2019 - ncbi.nlm.nih.gov
Paragangliomas and Pheochromocytomas are rare, mostly benign neuroendocrine tumors,
which are embryologically derived from neural crest cells of the autonomic nervous system …

Synonymous but not silent: a synonymous VHL variant in exon 2 confers susceptibility to familial pheochromocytoma and von Hippel-Lindau disease

SK Flores, Z Cheng, AM Jasper, K Natori… - The Journal of …, 2019 - academic.oup.com
Abstract Context von Hippel-Lindau (VHL) disease, comprising renal cancer,
hemangioblastoma, and/or pheochromocytoma (PHEO), is caused by missense or …

SDHC epi-mutation testing in gastrointestinal stromal tumours and related tumours in clinical practice

RT Casey, R Ten Hoopen, E Ochoa, BG Challis… - Scientific Reports, 2019 - nature.com
The enzyme succinate dehydrogenase (SDH) functions in the citric acid cycle and loss of
function predisposes to the development of phaeochromocytoma/paraganglioma (PPGL) …