Abstract

Palpation of an abdominal mass and the presence of difficult-to-control hypertension should make us consider the possible existence of a pheochromocytoma. Malignant pheochromocytoma accounts for 2-11% of neuroendocrine tumors, catecholamine producing tumors, in which the typical clinical triad is not always present because of the variety of their clinical presentations. Diagnosis is confirmed through the nondipper pattern in the ambulatory blood pressure monitoring (ABPM) study, the measurement of plasma metanephrines and chromogranin A, as well as the imaging tests. Pheochromocytomas have a bad prognosis, survival being less than 50% at 5 years. The main treatment is tumor excision. They are usually resistant to radiotherapy, and response to chemotherapy is minimum. Thus, the treatment approach is often mainly palliative.

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