Pheochromocytoma and Paraganglioma
المؤلف:
Marcello Ciaccio
المصدر:
Clinical and Laboratory Medicine Textbook 2021
الجزء والصفحة:
p357
2025-10-05
156
Pheochromocytoma and paraganglioma are rare neuroendocrine tumors arising from chromaffin cells. These cells originate from the neural crest and, upon migration, localize in the adrenal medulla and in the ganglia of the sympathetic chains of the thorax and abdomen, where they may give rise to tumors. In 85% of cases, the tumor originates in the adrenal medulla, where most of the chromaffin cells of our organ ism are located, and it is called pheochromocytoma; in the remaining 15% of cases, it can be found extra-adrenal, in the sympathetic paraganglia (mainly located in the retroperitoneum and the thorax), where it is called paraganglioma. Paraganglioma also refers to other parasympathetic tumors in the head, neck, and anterior thorax regions. However, unlike sympathetic paragangliomas and pheochromocytomas, they generally do not secrete catecholamines and are evident only by a compressive effect on surrounding vascular and nervous structures.
Pheochromocytomas and paragangliomas can occur as sporadic or as components of a genetically transmitted syn drome. Pheochromocytomas can occur at any age but are most common between the fourth and fifth decades of life and rare in infancy. 10% of cases are bilateral, 25% hereditary, and most are benign. Mainly catecholamine-secreting tumors produce noradrenaline, some noradrenaline and adrenaline, a few only adrenaline, and minimal part dopa mine; the latter is more frequently associated with a malignant phenotype. These tumors are characterized by extreme variability in the clinical presentation, which includes signs and symptoms related to the production of catecholamines and their metabolites; among these, the most common are headache, sweating, and palpitations, which constitute a classical triad. The most relevant clinical feature is hypertension, which can be persistent (50%) or paroxysmal (45%). The classic triad and hypertension raises a strong diagnostic suspicion of pheochromocytoma. However, it may remain asymptomatic for years and, in some cases, grow to a considerable size before symptoms appear.
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