A Case Report of a Pheochromocytoma Presenting With Neurological Manifestations

Abstract

A 51 year-old Caucasian female with a past medical history of hypertension, coronary artery disease and cerebrovascular accident presented to the emergency room with acute onset dysarthria and right-sided hemiparesis. The patient reported that she noticed these symptoms when she woke that morning, but had resolved by the time she reached the emergency room, an hour later. She denied experiencing palpitations, headache, chest pain or shortness of breath.On review of systems, it was revealed she had intermittent nausea and vomiting for a year with increasing frequency over the past two days. She denied blood or bile in the emesis. She also admitted to a 50-pound weight loss over the past year. Both esophagogastroduodenoscopy and colonoscopy were normal within the past year.Her medical history was significant for coronary artery disease complicated by two myocardial infarctions the previous year. She had also suffered from two past cerebrovascular accidents -the second of which was complicated by hemorrhage and seizure. Despite several anti-hypertensive medications, the patient's blood pressure remained uncontrolled.On admission, the patient was afebrile, had an elevated blood pressure of 167/108, pulse of 95 beats/minute, respiratory rate of 18 breathes/minute, and pulse oximetry of 97% on room air. Generally, the patient appeared comfortable. Cardiovascular exam was benign and pulmonary exam revealed decreased breath sounds bilaterally. Abdominal exam was also benign with no masses appreciated. Neurological exam was significant for slight dysarthria, right homonymous hemianopsia, and right-sided facial droop. Upper and lower extremity strength testing was significant for weakness at 4/5 strength on the left side. Comparatively, the patient's strength was 5/5 in the upper and lower extremities on the right side. Additionally, she was hyper-reflexive on the right side compared to the left. Sensation was intact throughout. These neurological deficits were consistent with her baseline deficits from past strokes.CT of the head was significant for an old left middle cerebral artery stroke, but there was no evidence of an acute process. A CT of the abdomen was performed in the emergency room, which revealed an incidental finding of a 6.7 x 5.4 cm right sided adrenal mass (Figure 1).The laboratory work-up revealed elevated catecholamines: plasma norepinephrine of 41.6, plasma metanephrine of 27.2, fractionated urine norepinephrine of 9390 and fractionated urine metanephrine of 8231.Diagnoses of transient ischemic attack and pheochromocytoma were made. Her blood pressure medications were changed to the alpha-blocker, phenoxybenzamine. A beta-blocker was later added for additional blood pressure control. The mass was surgically removed four weeks later. DiscussionA pheochromocytoma is a tumor that results in excess secretion of the catecholamines epinephrine and norepinephrine. It arises from chromaffin cells of the medulla of the adrenal gland, but it can also be located in extra-adrenal, retroperitoneal, pelvic or thorac...

Extensive Fever of Unknown Origin (FUO) Workup to Unmask Pheochromocytoma

“…Epinephrine acts on alpha-and beta-adrenergic receptors while norepinephrine acts on the same receptors, except beta 2 adrenergic receptors (vasodilatory action). This results in peripheral vasoconstriction by alpha receptor agonism and increased heart rate by beta 1 agonism [2]. Therefore, pre-operative medical management aims at initial alpha antagonism followed by beta antagonism.…”

“…Pheochromocytomas are rare neoplasms, with an estimated annual incidence of 0.8 per 100,000 personyears [1]. The annual incidence is likely to be an underestimate as autopsy studies suggest an 8% prevalence of adrenal masses and of these 4.2% are diagnosed as pheochromocytoma [2]. Pheochromocytoma accounts for 5% of adrenal incidentalomas [3].…”

“…The level of catecholamines and their metabolites in the plasma and urine provide 95% of the evidence of the disease [11]. Plasma levels of free metanephrines have a high sensitivity (99%) and specificity (89%) in diagnosing pheochromocytoma [2]. Interfering medications such as tricyclic antidepressants, nasal decongestants, etc.…”

Cyclical attacks of hypertension and hypotension in a young adult

“…El feocromocitoma se puede presentar con complicaciones cardiovasculares tales como infarto agudo de miocardio, encefalopatía hipertensiva y accidente cerebrovascular. Se han publicado casos en los que se asocian manifestaciones neurológicas en el contexto de un accidente isquémico transitorio y manifestaciones coronarias en el contexto de una angina estable, tal como se (5) presentaron en nuestro caso en forma de una angina estable y un accidente isquémico transitorio .…”