The genetics of paragangliomas

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Genetics of hereditary head and neck paragangliomas.

BACKGROUND The purpose of this study was to give an overview on hereditary syndromes associated with head and neck paragangliomas (HNPGs). METHODS Our methods were the review and discussion of the pertinent literature. RESULTS About one third of all patients with HNPGs are carriers of germline mutations. Hereditary HNPGs have been described in association with mutations of 10 different gene...

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Recent Advances in the Genetics of Pheochromocytomas and Paragangliomas

Paragangliomas and pheochromocytomas are rare neuroendocrine tumors, which secrete catecholamines, with the same embryological origin from the neural crest cells. Pheochromocytomas develop from the adrenal medulla, while paragangliomas are extraadrenal tumors, evolving from the autonomic sympathetic and parasympathetic nervous chains. In the last 10 years, molecular medicine has discovered nove...

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Genetics and clinical characteristics of hereditary pheochromocytomas and paragangliomas.

Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors of the adrenal glands and the sympathetic and parasympathetic paraganglia. They can occur sporadically or as a part of different hereditary tumor syndromes. About 30% of PCCs and PGLs are currently believed to be caused by germline mutations and several novel susceptibility genes have recently been discovered. The...

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Paragangliomas of the Spine.

AIM Paragangliomas of the spine are rare tumors. Clinical presentations and courses of spinal paragangliomas are varied, and there are no standard principles of treatment to date. The purpose of this study was to explore the diagnosis, treatment and prognosis of spinal paragangliomas. MATERIAL AND METHODS The clinical data of 7 consecutive cases, with complete medical records and follow-up re...

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[Paragangliomas of the carotid body].

61 Abstract. – Cervical paragangliomas are uncommon benign or malignant neoplasms, originated by stem cells of neural crest. It is not easy nowadays to define properly their biological behaviour, the possible multiple location and the association with Multiple Endocrine Neoplasms. After a wide review about recent diagnostic, pathological and clinical acquisition, authors report their caseload o...

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ژورنال

عنوان ژورنال: European Annals of Otorhinolaryngology, Head and Neck Diseases

سال: 2012

ISSN: 1879-7296

DOI: 10.1016/j.anorl.2012.04.007