Psychosomatics 39:283-287, June 1998
© 1998 The Academy of Psychosomatic Medine
Factitious Pheochromocytoma
One Patient History and Literature Review
Theodore A. Stern, M.D., and
M. Cornelia Cremens, M.D.
Received June 24, 1996; revised October 10, 1996; accepted October 18, 1996. From the Psychiatric Consultation Service, Massachusetts General Hospital (MGH), and the Department of Psychiatry, Harvard Medical School, Boston, MA. Address reprint requests to Dr. Stern, MGH, Department of Psychiatry, 100 Fruit St., WRN 605, Boston, MA 02114.
Key Words: Factitious Disorder • Pheochromocytoma • Factitious Pheochromocytoma
Chronic factitious illness with physical signs and symptoms is a malady characterized by recurrent episodes of intentionally feigned illness; physical signs can be simulated, exaggerated, aggravated, and/or self-induced to assume the sick role and not for economic gain.1
Other characteristics of the disorder (e.g., a feigned illness of a dramatic and urgent nature, a dramatic but plausible history, a willingness to undergo elaborate and risky procedures and treatments, a history of multiple hospitalizations and frequent travel, and frequent departures from the hospital against medical advice) are often seen.1,2 The characteristic triad of symptoms in Munchausen's syndrome that differ from factitious disorder include chronic and extreme factitious disorder; itinerancy; and pseudologica fantastica.
Cases of chronic factitious illness involving every organ system have been reported and have been responsible for expensive and unnecessary medical evaluations.2–4 As medical technology advances, so too will the means by which persons attempt to act out their psychopathology at the hands of physicians.2
We report the case of a young man with a factitious pheochromocytoma, highlight aspects of his workup that prevented unnecessary surgery, and review the literature on this disorder.
Case Report
Mr. A., a 27-year-old, single, white male attorney, was admitted to the Massachusetts General Hospital for control of new-onset, poorly controlled hypertension, accompanied by attacks of chest pain, nausea, vomiting, and diarrhea. The admitting physician was concerned that Mr. A. had either a pheochromocytoma or renal artery stenosis.
Upon admission to the hospital, Mr. A. was evaluated by the endocrine service. He was described as a "fascinating 27-year-old attorney" with a possible extra-adrenal pheochromocytoma.
Mr. A.'s medical history was significant for a motor vehicle accident at age 16, complicated by a cervical subluxation with subsequent C2–C4 fusion, supraspinatus nerve entrapment, and a left-clavicular reduction; all abnormalities were corrected surgically under general anesthesia without complication. At the age of 20, Mr. A. underwent an uncomplicated appendectomy and cholescystectomy. However, while recovering from surgery in the college infirmary, Mr. A. was told his blood pressure was elevated. No further evaluation of his hypertension was recommended.
According to the patient, he was well for the next 7 years, until he was assaulted by his brother 6 weeks before the current admission. A week later, Mr. A. noted the onset of episodes characterized by throbbing headache, nausea, vomiting, profound diaphoresis, palpitations, tremor, lightheadedness, and left-sided chest pain that radiated down his left arm with tingling in the left hand. After each episode, he felt "drained" and reported suffering several bouts of watery diarrhea. He noted increased thirst and urination for the 5 weeks before admission. Typically, the episodes occurred once a day, lasted about an hour, and were increasing in frequency and severity. Only one episode was witnessed in the hospital by a nurse, who said Mr. A. was flushed.
Two weeks before the current admission, Mr. A. was hospitalized for similar symptoms but with several hours of left-sided chest pain at a community hospital. A complete cardiac workup was performed; the hospital course was notable only for refractory hypertension. No cardiac etiology was found, and he was discharged on atenolol 50 mg twice a day.
The day after discharge from the community hospital, Mr. A. presented to our outpatient walk-in clinic with a blood pressure of 150/110 mmHg. His dose of atenolol was increased to 100 mg twice a day, and a clonidine patch (0.1 mg/day) was added without effect on his blood pressure. A paroxysmal hypertensive episode was noted in the walk-in clinic, with a blood pressure of 160/120 mmHg recorded. An abdominal computed tomography (CT) scan without contrast revealed a mild fullness in the right adrenal gland without evidence of a mass, and a left adrenal gland that was of normal size. The 24-hour urine collection had elevated levels of vanillylmandelic acid (VMA) (27.5 mg per 24 hours, normal range: 1–8 mg); metanephrines (1.38 mg per 24 hours, normal range: 0–0.9 mg); and creatinine (2,376 mg per 24 hours) suggestive of a pheochromocytoma. Mr. A. was admitted to maximize control of his labile hypertension and to intensify his workup for a possible pheochromocytoma.
In the hospital, inconsistencies (e.g., flushing, markedly increased VMA levels with a minimal increase in metanephrine) were noted in his signs, symptoms, and laboratory results. A 90-minute episode of nausea, vomiting, pounding headache, mild palpitations, and watery diarrhea was reported 2 days after admission. However, he did not notify the nursing staff as he was instructed by the medical team. A second episode, described by Mr. A. as the "worst one yet," occurred that night; severe chest pain radiating to the left arm, hypertension, increased heart rate, fever, headache, nausea, and vomiting were reported. His pain and blood pressure were poorly controlled after the administration of several medications. Thus, Mr. A. was transferred to the medical intensive care unit (ICU) for closer monitoring and for aggressive intravenous therapy to control his heart rate and blood pressure. The endocrinology fellow noted the minimal amount of diaphoresis and the absence of pallor were inconsistent with pheochromocytoma. A subsequent review of the abdominal CT scan confirmed only a fullness and an irregularly shaped right adrenal gland without evidence of a mass. The fellow suggested that a reference laboratory perform a more sensitive assay for VMA, while the ICU staff monitor his signs and symptoms more frequently and carefully. Psychiatric consultation was sought at this time to rule out a panic disorder, but Mr. A. refused to see the psychiatrist. Upon his transfer to the cardiac step-down unit for further monitoring, Mr. A. sent the ICU nurses a large arrangement of flowers and a three-foot-tall strawman scarecrow as a gesture of "thanks."
The endocrinology fellow encouraged Mr. A. to speak with the psychiatrist to determine if anxiety or panic contributed to his symptoms. In addition, the fellow suggested at this time the possibility of a falsely elevated VMA level due to ingestion of vanilla extract.
The psychiatric consultants indicated that the patient was inducing his episodes and agreed with the endocrine fellow's suspicion of ingested vanilla extract; panic disorder or drug-seeking behavior could not be ruled out. Mr. A. introduced himself as an attorney, but later it was revealed that he was only a paralegal.
His antihypertensive medications were found in his bedside table; he had not been taking his medications as prescribed, which raised the suspicion of the primary nurse, who searched for other clues and found an empty bottle of vanilla extract.
Medical records from prior hospitalizations in other states revealed an extensive history of hospitalizations for a variety of complaints and multiple negative evaluations for pheochromocytoma. At one hospital, a sampling from his right adrenal artery was complicated by a hemorrhage that resulted in the slight abnormality found in his right adrenal on the abdominal CT scan.
Mr. A. was confronted with this newly found information; he was calm and unconcerned that the ruse was over. Although offered inpatient or outpatient psychiatric treatment, he refused all psychiatric services. All his medications were discontinued. He was discharged and scheduled to follow-up with his outpatient physician.
Mr. A. returned for follow-up with his outpatient physician and presented her with an expensive ring in appreciation for her concern.
Discussion
Pheochromocytomas are uncommon; about 0.1% of the hypertensive population have such a tumor.5 The tumor occurs in the chromaffin cells (derived embrionically from the neural crest) of the adrenal medulla and sympathetic ganglia.5 The prevalence of pheochromocytomas is equal in men and women, and it typically presents in the third to fifth decade of life. Clinically, pheochromocytomas may present as sustained hypertension refractory to conventional treatment (as appeared in the case of Mr. A.), with paroxysmal symptoms that occur in 50% of the cases, as a hypertensive crisis, or with an unexplained hypertensive reaction to surgery or trauma.6
Paroxysmal symptoms may persist for 10–60 minutes and may occur spontaneously or be precipitated by anxiety, exercise, anesthesia, diagnostic procedures, or an increase in intra-abdominal pressure.6 Catecholamine release is not mediated neurally, because pheochromocytomas are not innervated.7 When paroxysms occur with pheochromocytomas, a sudden increase in blood pressure, tachycardia, pallor, palpitations, sudden onset of headache, atypical chest pain, profuse diaphoresis, anxiety, atypical epigastric pain, nausea, and vomiting may develop.6 The differential diagnosis of these symptoms encompasses a wide range of systems and etiologies (Table 1).5–7 However, the presence of a paroxysmal headache, tachycardia, and diaphoresis in a hypertensive patient increases the likelihood that a pheochromocytoma exists (with a sensitivity and specificity of >90%).6
Documented signs in patients with pheochromocytoma include hypertension (paroxysmal in 50%), orthostatic hypotension, micturition syncope, pallor, weight loss, fever, tremor, hyperglycemia, hypercalcemia, hematuria, retinopathy, cafe au lait spots, lactic acidosis in the absence of shock, and an increased hematocrit.6
The diagnosis can be confirmed with biochemical testing of urinary catecholamines (VMA, metanephrines, epinephrine, and norepinephrine) in a 24-hour urine sample.5 These tests are especially accurate when the patient is experiencing the symptoms.5 An approximate urinary creatinine level confirms the validity of the sample. Elevated metanephines are the most sensitive indicator of pheochromocytoma. Elevated VMA levels are the least specific, and they can be elevated with the ingestion of foods (e.g., bananas, coffee, nuts, citrus fruits, and vanilla extract) high in vanillin.5 VMA is elevated erroneously by the spectrophotometric interference of the earlier mentioned foods.5 In our case, Mr. A. ingested large quantities of vanilla extract in order to elevate these levels (Table 2).5 The reference laboratory was able to detect a normal VMA level in the serum exposed to the vanilla extract. Many factors can stimulate the production of endogenous catecholamines that may complicate the diagnosis of pheochromocytoma. (Table 2).5
Three case reports of factitious pheochromocytoma exist in the literature.8–10 The first was a 35-year-old woman who surreptitiously injected epinephrine that resulted in symptoms consistent with pheochromocytoma.8 The second was a 16-year-old girl who ingested large amounts of phenylpropanolamine.9 The third case was a patient who consciously altered autonomic function with valsalva maneuvers.10
About 10% of all patients with factitious disorder are thought to qualify for the diagnosis of Munchausen's syndrome (or chronic factitious disorder with physical symptomatology).11 Nadelson12 distinguished factitious disorder from Munchausen's syndrome by the presence of sociopathic traits (e.g., pathological lying, posing as someone else, and the pervasive commitment to the patient role). Eisendrath11,13 regarded patients with Munchausen's syndrome as untreatable.
Conclusion
Mr. A. had physical signs and symptoms suggestive of pheochromocytoma, but when it became apparent that his symptomatology was voluntary and self-induced, the diagnosis of Munchausen's syndrome and factitious pheochromocytoma was confirmed. Interestingly, Mr. A.'s gift of a strawman to the ICU nurses forewarned of his underlying diagnosis of factitious illness. According to Webster's dictionary,14 a strawman (ca 1900) is a weak or imaginary opposition as argument or adversary) set up to be easily confused; or a person set up to serve as a cover for an usually questionable transaction. Mr. A.'s hoax was destined to be uncovered. Were it not for an astute endocrinology fellow, the discovery of an empty bottle of vanilla extract, and determination of a falsely elevated VMA level, Mr. A. might well have undergone an adrenalectomy (given his abnormal abdominal CT scan) for presumed pheochromocytoma. When confronted with the details of his VMA test results that confirmed exogenous vanilla extract and not endogenous production of VMA, he did not own up to his deceit. However, while he agreed to outpatient medical follow-up with his primary care physician, he refused psychiatric treatment. Attention to inconsistencies in clinical signs, symptoms, and the clinical history, in combination with an awareness of the underlying psychodynamics involved, can facilitate the recognition of Munchausen's syndrome when it is present.
ACKNOWLEDGMENTS
The authors thank Carol Birnbaum, M.D., Lewis A. Chodosh, M.D., Ph.D., and Ann Li, M.D., for their assistance in this case.
REFERENCES
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American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, 4th Edition. Washington, DC, American Psychiatric Association, 1994, pp. 471–475
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Stern TA: Munchausen's syndrome revisited. Psychosomatics 1980; 21:329–336[Abstract/Free Full Text]
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Cohen LM, Shapiro E, Manson JE, et al: The high cost of treating a psychiatric disorder as a medical/surgical illness. Psychosomatics 1985; 26:453–455[Free Full Text]
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Folks DG, Freeman AM: Munchausen's syndrome and other factitious illnesses. Psychiatr Clin North Am 1985; 8:263–278[Medline]
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Sheps SG, Jiang NS, Klee GG, et al: Recent developments in the diagnosis and treatment of pheochromocytoma. Mayo Clin Proc 1990; 65:88–95[Medline]
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St. John Sutton MG, Sheps SG, Lie JT: Prevalence of clinically unsuspected pheochromocytoma. Mayo Clin Proc 1981; 5:6:354–360
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Bravo EL: Pheochromocytoma: new concepts and future trends. Kidney Int 1991; 40:544–556[Medline]
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Keiser HR: Surreptitious self-administration of epinephrine resulting in `pheochromocytoma.' JAMA 1991; 266:1553–1555[Abstract/Free Full Text]
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Hyams JS, Leichtner AM, Breiner RG, et al: Psuedopheochromocytoma and cardiac arrest associated with phenylpropanolamine. JAMA 1985; 253:1609–1610[Abstract/Free Full Text]
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Kailasam MT, Parmer RJ, Stone RA, et al: Factitious pheochromocytoma. Am J Hypertens 1995; 8:651–655[Medline]
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Feldman MD, Eisendrath SJ (eds): The Spectrum of Factitious Disorders. Washington, DC, American Psychiatric Press, 1996
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Nadelson T: The Munchausen spectrum: borderline character features. Gen Hosp Psychiatry 1979; 1:11–17[Medline]
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Eisendrath SJ: Facititious physical disorders: treatment without confrontation. Psychosomatics 1989; 30:383-387[Abstract/Free Full Text]
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Webster's Ninth New Collegiate Dictionary. Springfield, MA, Mirriam-Webster, 1990, p. 1165
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Ann Intern Med,
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