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Harrison's Principles of Internal Medicine, 18e
| Part 16. Endocrinology and Metabolism > Section 1. Endocrinology > |
Chapter 343. Pheochromocytoma
Sections: Pheochromocytoma: Introduction, Further Readings.
Topics Discussed: pheochromocytoma.
Excerpt:"Pheochromocytomas and paragangliomas are catecholamine- producing tumors derived from the sympathetic or parasympathetic nervous system. These tumors may arise sporadically or be inherited as features of multiple endocrine neoplasia type 2 or several other pheochromocytoma-associated syndromes. The diagnosis of pheochromocytomas provides a potentially correctable cause of hypertension, and their removal can prevent hypertensive crises that can be lethal. The clinical presentation is variable, ranging from an adrenal incidentaloma to a patient in hypertensive crisis with associated cerebrovascular or cardiac complications.Pheochromocytoma is estimated to occur in 2–8 of 1 million persons per year, and about 0.1% of hypertensive patients harbor a pheochromocytoma. Autopsy series reveal prevalence of 0.2%. The mean age at diagnosis is about 40 years, although the tumors can occur from early childhood until late in life. The "rule of tens" for pheochromocytomas states that about 10% are bilateral, 10% are extraadrenal, and 10% are malignant. However, these percentages are higher in the inherited syndromes.Pheochromocytomas and paragangliomas synthesize and store catecholamines, which include norepinephrine (noradrenaline), epinephrine (adrenaline),..."
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