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E-case #8

Feb 19-23

Vyn Reese M.D.

I.D. The patient is a 59 year old male presenting with headaches, chest pain and hypertension.

H.P.I.  The patient was concerned about hypertension at work, up to 160/120.  He also noted headaches, vague chest discomfort, nausea and shortness of breath.  The headaches were bifrontal, and usually associated with tremors.  The headaches would last only a few minutes and would resolve spontaneously.  They were becoming more frequent over the last six months.  The patient felt his symptoms were related to a job change that he made about six months earlier.  There was no relationship to food, urination, bowel movements or exercise.  He had been normotensive on numerous previous clinic visits, but had a blood pressure of 160/100 in the emergency room two days ago.

P.M.H. Remarkable for two episodes of bleeding ulcers felt to be secondary to NSAIDS. Medication: Ranitidine for GERD.

O.  B/P 140/80 P. 60 Wt.124 lbs. [unchanged]

            Alert, pleasant, anxious male

Neck-no palpable thyromegaly, or masses. No lid lag or tremor.

Chest-clear to A&P  Cardiac- regular rhythm, no audible murmurs, rubs or gallops.

Abdomen-non tender, no palpable masses or organomegaly.

Extremities-no edema

            Lab: EKG- w.n.l., CBC- Normal, Comprehensive chemistry panel-normal, and TSH normal.

 

Questions:

  1. What is the differential diagnosis in this case?

  2. Why is it important to make this diagnosis promptly?

  3. What tests are necessary to make the diagnosis?  How do you localize the problem?

  4. Is there effective therapy?

  5. How would you follow this patient in the future?

Discussion case #8:

  1. The most likely diagnostic possibilities in this case are: anxiety/panic disorder, hyperthyroidism [unlikely with normal TSH], pheochromocytoma, and drug use i.e. amphetamines, pseudoephedrine or ephedrine.  The classic triad of pheochromocytoma is: headaches, diaphoresis, and palpitations associated with paroxysmal or sustained hypertension.  The "spells" which occur with pheochromocytomas are also often marked by nausea, anxiety, and chest pain.  These spells are short-lived, usually lasting only a few minutes.

  2. Pheochromocytomas are rare making up less than 1% of all cases of hypertension.  Undiagnosed pheochromocytomas have a high mortality rate predominantly secondary to cerebrovascular catastrophes and myocardial infarctions.  Many anesthetic agents are extremely dangerous when administered to patients with unsuspected pheochromocytomas.  These drugs can cause a massive release of catecholamines from the tumor which can lead to severe hypertension or cause hypotension and shock given vasodilatory effects on volume depleted patients with pheos.  Even minor surgery can be fatal.

  3. The best modern screening test for pheochromocytoma are twenty-four hour urine for catecholamines and metanephrines.  In this case the twenty-four hour urine for total metanephrines was 3,083 mg./24 hr [normal 140-820].  Levels over 1,800 mg./24 hr. are diagnostic.  The tumor can then be localized by abdominal CAT or MRI scans.  This patient had a four cm. mass in the Organ of Zuckerkandl, an extra adrenal site, on CAT.  Ten percent of pheochromocytomas are extra adrenal, ten percent are malignant, and ten percent are familial.

  4. Surgical resection for non-malignant pheochromcytomas is curative.  Patients must be treated for two-three weeks pre operatively with alpha adrenergic agents, terazosin or doxazosin and occasionally selective beta blockers may need to be added to normalize blood pressure and reverse volume depletion.  This patient's tumor was not malignant and he did well.

  5. Ten percent of pheochromocytomas recur.  In this patient annual twenty-four hour urine for catecholamines and metanephrines have been negative.  Pheochromocytomas are associated with MEN II, Von Recklinghausen's Disease, and Von-Hippel Lindau Syndrome.  None of these disorders were present in this case.    

References:

1.Phaeochromocytoma-recent progress in its management. C. Prys-Roberts, Br. J. Anaesth 2000: 85:44-57.

2.Pheochromocytoma Update on Diagnosis, Localization, and Management.S.Werbel and K. Ober, Medical Clin. North America, Vol. 79, Vol. 1, Jan. 1995.