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Med Bull Sisli Etfal Hosp. 2020; 54(2): 159-168 | DOI: 10.14744/SEMB.2020.18794 | |||
Pheochromocytoma and paraganglioma: from epidemiology to clinical findingsNurcihan Aygun, Mehmet UludagDepartment of Genaral Surgery, Health Sciences University, Sisli Hamidiye Etfal Medical Practice and Research Center, Istanbul, TurkeyPheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors. Pheochromocytomas arise from chromaffin cells in the adrenal medulla, and PGLs arise from chromaffin cells in the ganglia of the autonomic nervous system. Paragangliomas originate from sympathetic or parasympathetic ganglia in the abdomen, thorax, and pelvis. The majority of PCC and sympathetic PGL are endocrine active tumors causing clinical symptoms by secreting excess catecholamines (norepinephrine, epinephrine, dopamine) and their metabolites. The incidence of PCC and PGL ranges between 2 and 8 per million, with a prevalence between 1: 2500 and 1: 6500. It peaks between the 3rd and 5th decades of life, and approximately 20% of cases are pediatric patients. The prevalence among patients with hypertension in outpatient clinic ranges between 0.1-0.6% in adults and between 2-4.5% in the pediatric age group. 10-49% of these tumors are detected incidentally in imaging techniques performed for other reasons. However, 4-8% of adrenal incidentalomas are PCCs. Of these neuroendocrine tumors, 80-85% are PCCs and 15-20% are PGLs. Pheochromocytoma and Paraganglioma: From Epidemiology to Clinical FindingsNurcihan Aygun, Mehmet UludagDepartment of Genaral Surgery, Health Sciences University, Sisli Hamidiye Etfal Medical Practice and Research Center, Istanbul, TurkeyPheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors. Pheochromocytomas arise from chromaffin cells in the adrenal medulla, and PGLs arise from chromaffin cells in the ganglia of the autonomic nervous system. Paragangliomas originate from sympathetic or parasympathetic ganglia in the abdomen, thorax, and pelvis. The majority of PCC and sympathetic PGL are endocrine active tumors causing clinical symptoms by secreting excess catecholamines (norepinephrine, epinephrine, dopamine) and their metabolites. The incidence of PCC and PGL ranges between 2 and 8 per million, with a prevalence between 1: 2500 and 1: 6500. It peaks between the 3rd and 5th decades of life, and approximately 20% of cases are pediatric patients. The prevalence among patients with hypertension in outpatient clinic ranges between 0.1-0.6% in adults and between 2-4.5% in the pediatric age group. 10-49% of these tumors is detected incidentally in imaging techniques performed for other reasons. However, 4-8% of adrenal incidentalomas are PCCs. Of these neuroendocrine tumors, 80-85% are PCCs and 15-20% are PGLs. Nurcihan Aygun, Mehmet Uludag. Pheochromocytoma and Paraganglioma: From Epidemiology to Clinical Findings. Med Bull Sisli Etfal Hosp. 2020; 54(2): 159-168 Sorumlu Yazar: Nurcihan Aygun |
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