Phäochromozytom

Phäochrozytom is a rare tumor that arises from chromaffin cells in the adrenal medulla or, less commonly, from sympathetic ganglia in the abdomen or thorax. These tumors produce and secrete excessive amounts of catecholamines, primarily epinephrine and norepinephrine, and sometimes dopamine. The word phäochrozytom originates from Greek, meaning "dark-colored cell tumor," referring to the characteristic darkening of tumor tissue when exposed to oxidizing agents used in histological staining.

The clinical presentation of phäochrozytom is highly variable and often episodic. The classic triad of symptoms includes headaches, palpitations, and excessive sweating. Other common manifestations can be severe hypertension, anxiety, tremors, nausea, vomiting, and a feeling of impending doom. Hypertension can be sustained or paroxysmal. Diagnosis relies on biochemical testing to measure elevated levels of catecholamines and their metabolites (metanephrines and vanillylmandelic acid) in urine and/or blood. Imaging techniques such as CT or MRI are used to localize the tumor.

Treatment for phäochrozytom is primarily surgical removal of the tumor. Preoperative medical management is crucial to control blood pressure and heart rate using alpha-adrenergic blockers, followed by beta-adrenergic blockers if necessary, to prevent life-threatening hypertensive crises during surgery. In cases where surgery is not feasible or in the presence of metastatic disease, medical therapy aims to control catecholamine excess and manage symptoms. Phäochrozytom can be associated with certain genetic syndromes, such as multiple endocrine neoplasia (MEN) types 2A and 2B, and neurofibromatosis type 1, which should be considered during the diagnostic workup. Prognosis is generally good with successful surgical resection, although malignant phäochrozytom can metastasize and has a poorer outlook.