Disease relevance of Paraganglioma, Extra-Adrenal

• Hereditary nonchromaffin paragangliomas (PGL; glomus tumors; MIM 168000) are mostly benign, slow-growing tumors of the head and neck region, inherited from carrier fathers in an autosomal dominant fashion subject to genomic imprinting [1].
• Hyperparathyroidism, chemodectoma, thymoma, and myasthenia gravis [2].
• Biopsies of human normal adrenal medulla, adrenal pheochromocytoma, and chemodectoma were studied for the presence of basic fibroblast growth factor (bFGF) [3].
• The triad of pulmonary chondroma, extra-adrenal paraganglioma, and gastric leiomyosarcoma has been reported in five patients, all young women [4].
• Carney's triad: gastric leiomyosarcoma, pulmonary chondroma and extra-adrenal paraganglioma in young females [5].

High impact information on Paraganglioma, Extra-Adrenal

• Of two patients with hyperparathyroidism, one had an associated chemodectoma, and the other had a thymoma and myasthenia gravis [2].
• RET is expressed but not mutated in extra-adrenal paragangliomas [6].
• Multiple extra-adrenal paragangliomas associated with catecholamine cardiomyopathy [7].
• The same finding on a negative MIBG scan in two patients with exclusively dopamine-secreting chemodectomas has been previously reported [8].
• While studying the uptake of iodine-123-metaiodobenzylguanidine ([123I]MIBG) in chemodectomas, we coincidentally detected catecholamine secreting tumors in 5 out of 14 patients [9].

Chemical compound and disease context of Paraganglioma, Extra-Adrenal

• Intrathoracic chemodectoma with noradrenaline secretion [10].
• Of 32 formalin fixed lung tumour resection specimens 6 were cases of SCC, 25 non-SCC and 1 a chemodectoma [11].
• Vertebral metastatic chemodectoma: imaging and therapeutic octreotide. Case report [12].
• Removal of catecholamine-secreting chemodectoma. The use of neuroleptanaesthesia, adrenergic blockade and sodium nitroprusside [13].
• Moreover, 111In-pentetreotide scintigraphy permits a good classification of patients with or without somatostatin receptors in the chemodectoma in the application of pharmacological therapy with somatostatin analogues to inoperable tumours [14].

Anatomical context of Paraganglioma, Extra-Adrenal

• A case of catecholamine-secreting chemodectoma of the neck in a 47-year-old male who also had temporal lobe epilepsy is described [13].
• Surgical specimens of 65 adrenal and 27 extra-adrenal paragangliomas, the latter comprising 11 carotid body, five jugulotympanic, one aorticopulmonary, eight aorticosympathetic and two visceral autonomic tumours, were examined immunocytochemically for the presence of glial fibrillary acid protein (GFAP) and S-100 protein [15].

Gene context of Paraganglioma, Extra-Adrenal

• In benign soft-tissue tumors, 1 chemodectoma and 1 neurothekeoma showed low MRP expression; however, no case showed co-expression of MRP and MDR1 [16].
• Immunoreactive somatostatin in catecholamine-producing extra-adrenal paraganglioma [17].
• Immunohistochemical demonstration of chromogranin A, chromogranin B, and secretogranin II in extra-adrenal paragangliomas [18].
• Twelve sympathetic and 14 parasympathetic extra-adrenal paragangliomas were investigated immunohistochemically with antibodies against chromogranin A, chromogranin B, and secretogranin II [18].
• The association of these two tumours and extra-adrenal paraganglioma has been described as a triad by Carney [5].

Analytical, diagnostic and therapeutic context of Paraganglioma, Extra-Adrenal

• Iodine-123-metaiodobenzylguanidine scintigraphy in patients with chemodectomas of the head and neck region [9].

References