Pheochromocytoma

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pheochromocytoma

[‚fē·ō‚krō·mō·sī ′tō·mə]
(medicine)
A tumor of the sympathetic nervous system composed principally of chromaffin cells; found most often in the adrenal medulla.

Pheochromocytoma

 

(also medullary chromaffinoma), a tumor of the cortical layer of the adrenal glands or sympathetic paraganglia that is characterized by the increased secretion of epinephrine and norepinephrine. The condition usually develops in youth or early middle age. The chief symptom is secondary hypertension, which may be persistent (often reaching a critical point with increased arterial pressure) or intermittent. The disorder is rarely found in its latent form, or without hypertension.

During severe attacks, pheochromocytoma is marked by a sudden and sharp increase in arterial pressure, draining of blood from the skin and mucous membranes, coldness of the extremities, chills, palpitation, headache, dizziness, shortness of breath, and tachycardia or, less frequently, bradycardia. The blood usually shows increased levels of sugar and leucocytosis. Casts, or cylinders, and protein are observed in the urine. The attack may last from several minutes to several hours; copious urination often accompanies its ending. Hypertensive crises may result from emotional disturbances, physical stress, chilling of the body, overheating, deep abdominal palpation, or pressure exerted on the tumor by body movements. The attacks grow more frequent and severe with the advance of the disease. Death may occur as a result of brain hemorrhage or severe cardiac insufficiency (pulmonary edema). Pheochromocytoma during pregnancy endangers both the mother and the fetus; early removal of the tumor is therefore indicated—or, alternatively, termination of the pregnancy.

The diagnosis of pheochromocytoma is based on its characteristic symptoms during an attack, the results of urine analysis (that is, sharply increased secretion of catecholamines), and X-ray examinations (specifically, roentgen diagnosis through the injection of oxygen into the cellular tissue surrounding the adrenal glands). The condition is also identified through its rapid improvement following the intravenous injection of adrenolytic agents, such as troparphene and phentolamine. Surgery is the method of treating the disease.

REFERENCE

Feokhromotsitoma. Moscow, 1965.

N. R. PALEEV

References in periodicals archive ?
Keywords: Pheochromocytoma, Appendicitis, Hypertension.
Approximately 59% of sporadic pheochromocytomas or paragangliomas which are diagnosed in children of <18 years old and up to 70% of those that are diagnosed in children of <10 years old are associated with germline mutations (7).
Treatment for malignant pheochromocytomas and paragangliomas: 5 years of progress.
This is reportedly the first US FDA-approved drug for pheochromocytoma and the approval of Azedra has been awarded to Progenics Pharmaceuticals Inc.
It was reported a case of pheochromocytoma observed in a 25-year-old, donkey.
Neuroendocrine tumor of the pancreas and bilateral adrenal pheochromocytomas. A rare manifestation of von Hippel-Lindau disease in childhood.
Recommendations for somatic and germline genetic testing of single pheochromocytoma and paraganglioma based on findings from a series of 329 patients.
Malignant pheochromocytomas and paragangliomas - The importance of a multidisciplinary approach.
Importantly, clinical-grade inhibitors of HIF2A exist and are in early clinical trials for a variety of conditions, including pheochromocytomas. "Thus, this discovery can potentially have an impact on patients' lives," she concluded.
Prejbisz et al., "Extra-adrenal and adrenal pheochromocytomas associated with a germline SDHC mutation," Nature Clinical Practice Endocrinology & Metabolism, vol.
Wubbenhorst et al., "Whole-exome sequencing identifies somatic ATRX mutations in pheochromocytomas and paragangliomas," Nature Communications, vol.
Pheochromocytoma, the "great masquerader," is a neoplasm of neuroectodermal chromaffin cells which produces excess catecholamines.