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Pheochromocytoma: “A Disease With a Thousand Faces”

2009; Wiley; Volume: 11; Issue: 2 Linguagem: Inglês

10.1111/j.1751-7176.2009.00077.x

1751-7176

Marvin Moser,

Hormonal Regulation and Hypertension

Ron Cheney, the well-known character actor, was called the Man of a Thousand Faces. The recent publication of 4 case reports on pheochromocytoma in The Journal of Clinical Hypertension highlights a disease with multiple presentations1–4—perhaps not a thousand faces but enough to confuse and surprise.5 Hypertension is generally a symptom-free disease, although, as we all have learned, many patients with even slightly elevated blood pressure experience a flushed face, early-morning occipital headaches that disappear on arising, or just do not feel “right.” Some studies have reported that as blood pressure is lowered, patients feel better and that quality of life, as measured by questionnaires, improves. Most patients, however, with uncomplicated hypertension before the onset of heart failure or severe vascular disease remained relatively asymptomatic. Pheochromocytoma is a term that we remember from medical school. We tend to forget about it because, in the course of our training, we probably never saw a case, but we were always reminded to rule it out in hypertensive patients. Pheochromocytoma is “hypertension with symptoms” and may present, as Ron Cheney did, in many forms—“the great mimic.” The typical triad of symptoms is palpitations, headaches, and inappropriate sweating, not in the groin area or underarms but on the face, chest, or back. In addition, panic or anxiety attacks, abdominal pain, nausea and vomiting, shortness of breath, weakness, dyspnea, weight loss, and other symptoms may be present. The case reports in The Journal of Clinical Hypertension (JCH) highlight cyclical high and low blood pressures and various electrocardiographic (ECG) abnormalities as presenting features of pheochromocytomas. In addition, shock may be an initial presentation, various types of ventricular dyskinesias may be noted, and a cerebrovascular accident or pulmonary edema may be the first sign of catecholamine excess. These may be the symptoms that lead to a visit to the emergency department or a doctor’s office. When any of these episodes are noted in the presence of elevated blood pressure or a fluctuating blood pressure, pheochromocytoma, although a rarity, should be suspected. One of the first papers we wrote was on pheochromocytoma with calcification simulating a cholelithiasis. A 42-year-old man presented with a history of right upper quadrant pain, nausea, and vomiting.6 This was intermittent over a long period. Radiographic findings showed a circular calcific shadow in the right upper quadrant; the patient was admitted for a cholecystectomy. Examination revealed a blood pressure of 130/80 mm Hg. The nurse informed me, however, that when she took the pressure, it was well over 200 mm Hg. About 30 minutes after my first examination, the patient began to sweat profusely. His pulse rate was 120 beats per minute, he experienced a severe occipital headache, and his blood pressure was 200–220 mm Hg/130–140 mm Hg. I knew nothing about a pheochromocytoma but remembered hearing about it as a third-year medical student. It was apparent, after researching the causes of paroxysmal hypertension, that this was indeed a rare case of pheochromocytoma. Subsequent evaluation determined that the circular calcification was in the right adrenal gland. The gallbladder was normal. Although speckled calcification secondary to hemorrhage in the tumor had been reported in a few cases, this type of calcification seemed to add another unusual presentation of this multifaceted tumor. Incidentally, cholelithiasis is not uncommon with pheochromocytoma. Several years later we reported on several cases of pheochromocytoma in patients who were admitted with a diagnosis of ischemic heart disease, various arrhythmias (with ST-segment changes on electrocardiography, and evidence of left ventricular hypertrophy.7 ECG changes fluctuated as the blood pressure was lowered or raised pharmacologically. These patients were originally being treated as patients with coronary heart disease. Since then we have seen only about 25 cases of pheochromocytoma during the past 45 to 50 years in a practice that specialized in hypertension. Patients have varied in age from 4 to 70 years and have invariably been thin, although one case represented a very rare example of a “fat” pheo. All of the patients had many of the symptoms listed above. Pheochromocytomas have masqueraded as repeat panic attacks, anxiety neuroses, thyroid disease (a common accompaniment of pheochromocytoma), or diabetes (also not uncommon with this tumor). The recent cases in JCH describe patients with episodes of heart failure, cyclic bouts of hypertension and hypotension, intracerebral hemorrhage, and various degrees of ventricular akinesia or dyskinesia with ECG changes. The bottom line is that whenever a patient is seen with widely fluctuating blood pressures that are: (1) shock-like on some occasions; (2) elevated on others; (3) or are persistently elevated with the specific symptoms noted above, pheochromocytoma should be considered. The diagnostic evaluation today is a great deal simpler than years ago when we used drug testing with tetraethyl ammonium chloride, phenoxybenzamine, phentolamine, or histamine. All of these tests posed a degree of risk.7 Chemical testing with plasma and urinary catecholamines have been perfected; computed tomography and other scans will define the lesions.5 The most important issue with a pheochromocytoma is its discovery—these are rarely malignant and surgery is curative—one of the few truly curative causes of hypertension. Thus the great interest in this disease with a thousand faces.