Pheochromocytoma
Pheochromocytomas are rare tumors originating in the adrenal medulla. They may be sporadic or in the context of a hereditary syndrome. A considerable number of pheochromocytomas carry germline or somatic gene mutations, which are inherited in the autosomal dominant way. All patients should undergo g...
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Sciendo
2019
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oai:doaj.org-article:0732cad180d4417c8701aa79527cbde52021-12-02T19:07:37ZPheochromocytoma1336-032910.2478/enr-2019-0020https://doaj.org/article/0732cad180d4417c8701aa79527cbde52019-07-01T00:00:00Zhttps://doi.org/10.2478/enr-2019-0020https://doaj.org/toc/1336-0329Pheochromocytomas are rare tumors originating in the adrenal medulla. They may be sporadic or in the context of a hereditary syndrome. A considerable number of pheochromocytomas carry germline or somatic gene mutations, which are inherited in the autosomal dominant way. All patients should undergo genetic testing. Symptoms are due to catecholamines over production or to a mass effect. Diagnosis is confirmed by raised plasma or urine metanephrines or normetanephrines. Radiology assists in the tumor location and any local invasion or metastasis. All the patients should have preoperative preparation with α-blockers and/or other medications to control hypertension, arrhythmia, and volume expansion. Surgery is the definitive treatment. Follow up should be life-long.Farrugia Frederick-AnthonyCharalampopoulos AnestisSciendoarticlepheochromocytomaepidemiologygeneticspathologysymptomsradiologytreatmentsurgerymedicationDiseases of the endocrine glands. Clinical endocrinologyRC648-665ENEndocrine Regulations, Vol 53, Iss 3, Pp 191-212 (2019) |
| institution |
DOAJ |
| collection |
DOAJ |
| language |
EN |
| topic |
pheochromocytoma epidemiology genetics pathology symptoms radiology treatment surgery medication Diseases of the endocrine glands. Clinical endocrinology RC648-665 |
| spellingShingle |
pheochromocytoma epidemiology genetics pathology symptoms radiology treatment surgery medication Diseases of the endocrine glands. Clinical endocrinology RC648-665 Farrugia Frederick-Anthony Charalampopoulos Anestis Pheochromocytoma |
| description |
Pheochromocytomas are rare tumors originating in the adrenal medulla. They may be sporadic or in the context of a hereditary syndrome. A considerable number of pheochromocytomas carry germline or somatic gene mutations, which are inherited in the autosomal dominant way. All patients should undergo genetic testing. Symptoms are due to catecholamines over production or to a mass effect. Diagnosis is confirmed by raised plasma or urine metanephrines or normetanephrines. Radiology assists in the tumor location and any local invasion or metastasis. All the patients should have preoperative preparation with α-blockers and/or other medications to control hypertension, arrhythmia, and volume expansion. Surgery is the definitive treatment. Follow up should be life-long. |
| format |
article |
| author |
Farrugia Frederick-Anthony Charalampopoulos Anestis |
| author_facet |
Farrugia Frederick-Anthony Charalampopoulos Anestis |
| author_sort |
Farrugia Frederick-Anthony |
| title |
Pheochromocytoma |
| title_short |
Pheochromocytoma |
| title_full |
Pheochromocytoma |
| title_fullStr |
Pheochromocytoma |
| title_full_unstemmed |
Pheochromocytoma |
| title_sort |
pheochromocytoma |
| publisher |
Sciendo |
| publishDate |
2019 |
| url |
https://doaj.org/article/0732cad180d4417c8701aa79527cbde5 |
| work_keys_str_mv |
AT farrugiafrederickanthony pheochromocytoma AT charalampopoulosanestis pheochromocytoma |
| _version_ |
1718377128099053568 |