Genetic background of tumors originating from adrenomedullar and extraadrenal chromaffin tissue--update
Vanuga, P.; Pura, M.; Kreze, A.
Vnitrni Lekarstvi 56(12): 1296-1302
2010
ISSN/ISBN: 0042-773X PMID: 21261119 Document Number: 639839
It is anticipated that an inherited/familial forms of pheochromocytomas cause approximately 20% of all pheochromocytomas. Therefore, the classic "rule of 10" axioma used to remember the key features of disorder is invalid. Various mutations in several genes have been identified, which underly syndromes with paragangliomas and/or pheochromocytomas. The more candidate genes, the less numbers of patients with apparently sporadic forms of the disorder. This review has summarized the current knowledge of the genetic background of tumors orginating from adrenomedullar and extra-adrenal chromaffin tissue.