A 68-year-old womale was sent out to the emergency department (ED) bereason of alterations in her level of consciousness. The patient had actually polydipsia and also proficient a weight loss of around 6 kg, which was connected with steady general weakness over a duration of numerous months. Intermittent diaphoresis and palpitation via occasional headache were also provided. She had actually no recognized underlying systemic condition, such as hyperstress or cardiac dysrhythmia. She denied taking any type of medication. She smoked 0.2 packs per day for 3 years, but denied taking either alcohol or illicit drugs. She denied a family members background of diabetes mellitus (DM) or any kind of familial illness.

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At the ED, the patient’s body mass index was underweight at 17.6 kg/m2. Her body temperature was 38.0 °C, via a blood pressure of 199/118 mmHg, pulse price 129 beats per minute, and also respiratory price 18 breaths per minute. The oxygen saturation was 93 % while she was breathing in ambient air. The laboratory information proved a newly-diagnosed DM complex with a hyperosmolar hyperglycemic state (serum glucose at 1836 mg/dl, serum sodium 124 mmol/l, hemoglobin A1C 14.4 %, and also efficient osmolality 350 mOsm/kg). After enough hydration, insulin therapy and appropriate antibiotics for suspected aspiration pneumonia, the patient’s blood sugar enhanced and also the fever before subsided. During hospitalization, blood pressures were 100–120/60–70 mmHg without any kind of authorize of hyperanxiety or signs suggestive of orthostatic changes.

However before, on the 20th hospital day, the patient had actually a second bout of fever before via sepsis-prefer presentation (high fever before 39.3 °C, tachycardia 110 beats per minute, tachypnea, however without leukocytosis) that was refractory to antibiotic therapy. Abdominal sonography was percreated to search for the infection emphasis, which revealed an intra-abdominal tumor near the liver and kidney. A subsequent computed tomography shown a 4-cm solid heterogeneous mass lesion in the right adrenal gland (Fig. 1a). Based on the patient’s clinical presentations (markedly elevated blood press at ED, background of intermittent palpitation, and unexplained fever), a pheochromocytoma was extremely most likely. Collection of 24-h urine for catecholamine and vanillylmandelic acid (VMA) levels verified the isolated elevation of epinephrine levels. Repeated arsenal of urinary examicountry for catecholamines and VMA levels were performed after the patient was complimentary from her acute illness proved similar results (Table 1). Pre-operative therapy through α- and β-blockade treatment were given to the patient, and she underwent uneventful laparoscopic ideal adrenalectomy. The pathological examination evidenced our diagnosis of pheochromocytoma (Fig. 1b). The patient stayed symptom-totally free via normal urinary catecholamines excretion after 6 months of post-operative follow-up. Her DM is presently under excellent glycemic regulate (hemoglobin A1C 6.1 %) making use of an dental antidiabetic agent (metformin 250 mg daily). In addition, she gradually gained weight to a body mass index of 23.4 kg/m2.


Abdominal muscle computed tomography mirrors a 4 cm solid heterogeneous mass lesion in the ideal adrenal gland also through interior low attenuation foci. No regional adenopathy, vascular invasion or metastatic illness is noticeable (a). Pheochromocytoma cells stained with hematoxylin and eosin stain, at ×100 magnification under a microscope. The tumor cells have huge round vesicular nuclei and also abundant basophilic to amphophilic cytoplasm. Increased vascularity is detailed. Tbelow is no increased mitotic count (b)

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Table 1 Pre-operative and also post-operative urinary catecholamine parameters
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Pheochromocytoma is a rare catecholamine-producing tumor that most generally presents via episodes of headaches, sweating, palpitations, and paroxysmal or continual hyperanxiety. However, clinical manifestations of pheochromocytoma are very variable, and also deserve to mimic numerous other illness, such as acute coronary syndrome, heart faiattract, and also sepsis <1>. Paroxysmal or sustained hypertension is just one of the significant presentations of pheochromocytoma. However before, excess catecholamine secretions in a patient via pheochromocytoma deserve to likewise bring about cardiovascular complications without hypertension <2>. As such, a timely diagnosis and therapy of pheochromocytoma is extremely important.

Different catecholamine secretions from pheochromocytoma have different impacts on blood glucose and also blood pressure levels <3>. About 40–50 % of patients via pheochromocytomas clinically present as glucose intolerance or DM, which are such common pathologic conditions that they can delay the timely diagnosis of pheochromocytoma. Hyperglycemia has actually been jiyuushikan.orged to aggravation of insulin resistance led to by catecholamine excess in pheochromocytoma. The inhibitory effect of catecholamines on insulin secretion is thought to be mediated by α-2 adrenergic receptors. The isolated elevation of epinephrine levels in our instance had a prodiscovered result on glucose intolerance, inducing lipolysis and also hepatic gluconeogenesis, but inhibited peripheral glucose utilization. About 54 % of adrenergic creating tumors in the adrenal glands are diagnosed in patients of advanced age, equivalent to our situation. The possible mechanism that led to our patient’s presenting through DM complicated through hyperosmolar hyperglycemic state might have actually been that the isolated catecholamine excess via epinephrine secreting pheochromocytoma brought about a rise in lipolysis, which lessened insulin secretion and enhanced glucose production from the liver. Although our patient, a couple of months before admission presented clinically with intermittent catecholamine excess that contained intermittent diaphoresis, palpitation, and occasional headache, tright here was no evidence of hyperstress uncovered during admission. However, unprefer the usual presentation wright here one would suppose hypostress and anxiety from volume depletion at the height of the hyperglycemic hyperosmolar state, she had actually marked hyperstress. Furthermore, DM patients complicated via hyperosmolar hyperglycemic state commonly have actually impaired consciousness or have actually a bed-ridden status, which was not constant with our patient. It is therefore important to consider pheochromocytoma as among our differential diagnoses in an ambulatory patient presenting with noted hyperstress at the elevation of hyperosmolar hyperglycemic state, particularly as soon as the clinical presentation is various from the usual indicators and symptoms.

Although dimensions of plasma-cost-free metanephrines or urinary fractionated metanephrines are the many sensitive and also many trusted signs for the exclusion of pheochromocytoma, these were uneasily accessible in our medical facility <4>. In this instance, only the conventional biochemical exams that include repertoire of 24-h urine catecholamines and also VMA were percreated. However, urine catecholamine concentrations boost throughout pathophysiological or pharmacological adrenosympathetic stimulation, which deserve to result in the misdiagnosis of pheochromocytomas. In addition, these bioamines are also incredibly sensitive to diets, medications and also adrenosympathetic stimulation, making the interpretations of laboratory outcomes undependable in the diagnosis and surveillance of pheochromocytomas. For these reasons, a repeated collection of urine for catecholamines and also VMA levels were perdeveloped once the patient respanned from her acute disease. The results confirmed persistent elevation of epinephrine levels, for this reason, sustaining the diagnosis of an epinephrine-producing adrenal tumor. Although VMA has a high specificity price, the VMA level in our case was an unreliable marker for pheochromocytoma.

On initial presentation, our patient was underweight, and also this can have actually been as a result of chronic catecholamine overmanufacturing resulted in by pheochromocytoma that might have actually resulted in the increase in proinflammatory cytokines and also the hypermetabolic state identified by increased relaxing power expenditure. Adrenalectomy brought about the normalization of the power metabolism in our case followed by a rise in body mass index and also body fat content through a decrease in inflammatory markers and also cytokines <5>. It is therefore of utmost importance, to look for an unfound endocrinopathy with hypermetabolic state in situations of an underweight patient presenting via a hyperosmolar hyperglycemic state not having any kind of previous history of DM.

Here we highlight the prominence of integrating pheochromocytoma-complicating DM in a case presenting via hyperosmolar hyperglycemic state with noted hypertension and also fever yet without a focus of infection.

Conflict of interest

Authors declare that they have actually no conflict of interemainder.

Statement of humale and also animal rights

All measures complied with were in accordance via the moral criteria of the responsible committees (institutional and national) and also via the Helsinki Declaration of 1975, as revised in 2008.

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Indeveloped consent

Informed consent out was derived from the participant for being had in this research according to Ethics Committees demands.


Division of Endocrinology and also Metabolism, Department of Internal Medicine, Wan Fang Hospital, Taipei Medical University, 111 Shin Lung Roadway Section 3, Taipei, Taiwan

I-Shuan Lee, Ting-Wei Lee, Chun-Jen Chang, Yu-Mei Chien & Ting-I Lee

Graduate Institute of Clinical Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan

Ting-Wei Lee

Department of General Medicine, College of Medicine, Taipei Medical College, Taipei, Taiwan

Ting-I Lee


Cite this article

Lee, IS., Lee, TW., Chang, CJ. et al. Pheochromocytoma presenting as hyperglycemic hyperosmolar syndrome and unexplained fever. Intern Emerg Med 10, 753–755 (2015). https://doi.org/10.1007/s11739-015-1217-5