International consensus on initial screening and follow-up of asymptomatic SDHx mutation carriers
L Amar, K Pacak, O Steichen, SA Akker… - Nature Reviews …, 2021 - nature.com
Approximately 20% of patients diagnosed with a phaeochromocytoma or paraganglioma
carry a germline mutation in one of the succinate dehydrogenase (SDHx) genes (SDHA …
carry a germline mutation in one of the succinate dehydrogenase (SDHx) genes (SDHA …
[HTML][HTML] Succinate, an intermediate in metabolism, signal transduction, ROS, hypoxia, and tumorigenesis
Succinate is an important metabolite at the cross-road of several metabolic pathways, also
involved in the formation and elimination of reactive oxygen species. However, it is …
involved in the formation and elimination of reactive oxygen species. However, it is …
Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline
Objective: The aim was to formulate clinical practice guidelines for pheochromocytoma and
paraganglioma (PPGL). Participants: The Task Force included a chair selected by the …
paraganglioma (PPGL). Participants: The Task Force included a chair selected by the …
New perspectives on pheochromocytoma and paraganglioma: toward a molecular classification
A molecular biology–based taxonomy has been proposed for pheochromocytoma and
paraganglioma (PPGL). Data from the Cancer Genome Atlas revealed clinically relevant …
paraganglioma (PPGL). Data from the Cancer Genome Atlas revealed clinically relevant …
Clinical consensus guideline on the management of phaeochromocytoma and paraganglioma in patients harbouring germline SDHD pathogenic variants
Patients with germline SDHD pathogenic variants (encoding succinate dehydrogenase
subunit D; ie, paraganglioma 1 syndrome) are predominantly affected by head and neck …
subunit D; ie, paraganglioma 1 syndrome) are predominantly affected by head and neck …
An overview of 20 years of genetic studies in pheochromocytoma and paraganglioma
Paragangliomas and pheochromocytomas (PPGL) are rare neuroendocrine tumours
characterized by a strong genetic determinism. Over the past 20 years, evolution of PPGL …
characterized by a strong genetic determinism. Over the past 20 years, evolution of PPGL …
Succinate dehydrogenase (SDH)‐deficient neoplasia
AJ Gill - Histopathology, 2018 - Wiley Online Library
The succinate dehydrogenase (SDH) complex is a key respiratory enzyme composed of four
subunits: SDHA, SDHB, SDHC and SDHD. Remarkably, immunohistochemistry for SDHB …
subunits: SDHA, SDHB, SDHC and SDHD. Remarkably, immunohistochemistry for SDHB …
Tumour risks and genotype–phenotype correlations associated with germline variants in succinate dehydrogenase subunit genes SDHB, SDHC and SDHD
Background Germline pathogenic variants in SDHB/SDHC/SDHD are the most frequent
causes of inherited phaeochromocytomas/paragangliomas. Insufficient information …
causes of inherited phaeochromocytomas/paragangliomas. Insufficient information …
Von Hippel–Lindau and hereditary pheochromocytoma/paraganglioma syndromes: clinical features, genetics, and surveillance recommendations in childhood
Abstract Von Hippel–Lindau disease (vHL) is a hereditary tumor predisposition syndrome
that places affected individuals at risk for multiple tumors, which are predominantly benign …
that places affected individuals at risk for multiple tumors, which are predominantly benign …
The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma …
Pheochromocytomas, intra-adrenal paraganglioma, and extra-adrenal sympathetic and
parasympathetic paragangliomas are neuroendocrine tumors derived from adrenal …
parasympathetic paragangliomas are neuroendocrine tumors derived from adrenal …