Disease Information for Pheochromocytoma: Definition

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  • A usually benign, well-encapsulated, lobular, vascular tumor of chromaffin tissue of the adrenal medulla or sympathetic paraganglia; The cardinal symptom, reflecting the increased secretion of epinephrine and norepinephrine, is hypertension, which may be persistent or intermittent; During severe attacks, there may be headache, sweating, palpitation, apprehension, tremor, pallor or flushing of the face, nausea and vomiting, pain in the chest and abdomen, and paresthesias of the extremities; The incidence of malignancy is as low as 5% but the pathologic distinction between benign and malignant pheochromocytomas is not clear; (Dorland, 27th ed; DeVita Jr et al, Cancer: Principles & Practice of Oncology, 3d ed, p1298)

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    rule of tens: 10% bilateral,10%familial[MEN2],10% incidentaloma,10% malignant,10% extra-adrenal, 10% of seconday hypertension cases, 10% recurrence after resection

    [MKSAP13]

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