<abstract xmlns="http://www.w3.org/1999/xhtml"><p>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.</p></abstract>

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.

Pheochromocytoma

Endocrine Regulations

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

{"article-title":"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...

Pheochromocytoma

Pubblicato online: 24 ago 2019

Pagine: 191 - 212

DOI: https://doi.org/10.2478/enr-2019-0020

Parole chiave
pheochromocytoma, epidemiology, genetics, pathology, symptoms, radiology, treatment, surgery, medication

© 2019 Frederick-Anthony Farrugia et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Pheochromocytoma

Pubblicato online: 24 ago 2019

Pagine: 191 - 212

DOI: https://doi.org/10.2478/enr-2019-0020

Parole chiavepheochromocytoma, epidemiology, genetics, pathology, symptoms, radiology, treatment, surgery, medication

© 2019 Frederick-Anthony Farrugia et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Parole chiave
pheochromocytoma, epidemiology, genetics, pathology, symptoms, radiology, treatment, surgery, medication