The Protean Manifestations of Pheochromocytoma

Abstract

The treacherous and deceptive nature of pheochromocytoma makes it crucial to detect and treat it promptly; otherwise it will almost certainly be fatal from cardiovascular complications or metastases. Hypertension occurring in patients with pheochromocytomas is sustained in about 50% and paroxysmal in the remainder; however, many patients remain normotensive. Hypertension attacks may be precipitated by physical activity, postural changes, anxiety, certain foods or wine, some drugs, operative procedures, etc. Cardinal manifestations are paroxysmal hypertension, headache, palpitations +/- tachycardia, inappropriate sweating; anxiety, tremulousness, pallor (rarely flushing), chest and abdominal pains; nausea and vomiting often occur. Hypercatecholaminemia manifestations are more common and pronounced when paroxysmal hypertension occurs, but persons with familial pheochromocytoma may be asymptomatic. Protean manifestations of pheochromocytoma may simulate many conditions, some of which may have elevated plasma and urine catecholamines and their metabolites. Baro-reflex failure, postural tachycardia syndrome, sleep apnea, carcinoid, renal failure, and pseudopheochromocytoma may be diagnostic challenges. The history, physical examination, biochemical testing (after eliminating interfering drugs, when possible) for plasma and urinary metanephrines can usually establish or exclude presence of pheochromocytomas. Occasionally a clonidine suppression test is needed to differentiate neurogenic from pheochromocytic hypertension. Manifestations suggesting hypercatecholaminemia without hypertension are highly atypical of pheochromocytoma. Pheochromocytoma may present as panic attacks, pre-eclampsia, cardiomyopathy, infection with fever and leucocytosis, diabetes, migraine, shock, Cushing's syndrome, multiple organ failure with lactic acidosis, neurological manifestations, transitory electrocardiogram abnormalities, constipation, intestinal obstruction, visual impairment, convulsions, etc. The key to diagnosis is always to think of pheochromocytoma in the differential diagnosis of hypertension.

Malignant pheochromocytoma in the anterior mediastinum with sternal invasion: a case report

“…The most common symptoms of pheochromocytomas are headache, blurred vision, sweating, heart palpitation, and flushing (8,9). When pheochromocytomas appear in the mediastinum, patients often complain of palpitation, headache, nausea, vomiting, chest pain, or chest tightness (14-17).…”

“…The malignant ones account for approximately 10% of all pheochromocytomas (6,7). Patients who suffer from pheochromocytomas mainly complain of hypertension, headache, sweating, palpitation, apprehension, tremor, pallor or flushing of the face, nausea and vomiting, chest pain, abdominal pain, or limb paresthesias (8,9). These symptoms are regarded as the result of the increased secretion of epinephrine and norepinephrine.…”

Pheochromocytoma or Münchausen syndrome: the masquerade is up

“…Pheochromocytoma is a rare, deceptive and potentially deadly catecholamine-producing tumor, the recognition of which is often obscured and delayed by other more obvious diagnoses [1]. Diagnosis of pheochromocytoma is, however, also likened to finding a needle in a haystack so that most frequently when the tumor is suspected, the manifestations reflect an entirely different clinical condition.…”

Phasing-in plasma metanephrines determination

“…Alternatively, phaeochromocytomas also exhibit less demonstrative cardiovascular features such as chronic hypertension (3,4). Thus, from a cardiologist or a hypertension specialist point of view, phaeochromocytoma should be excluded when certain clinical features are encountered.…”