A 68-year-old mrs was sent to the emergency department (ED) since of alterations in her level the consciousness. The patient had polydipsia and also experienced a weight loss of about 6 kg, i beg your pardon was connected with progressive basic weakness end a duration of numerous months. Intermittent diaphoresis and also palpitation through occasional headache were also noted. She had actually no known underlying systemic disease, such together hypertension or cardiac dysrhythmia. She denied taking any kind of medication. She acting 0.2 packs per day because that 3 years, but denied taking either alcohol or illicit drugs. She denied a family background of diabetes mellitus (DM) or any type of familial diseases.

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At the ED, the patient’s human body mass index was underweight in ~ 17.6 kg/m2. Her body temperature was 38.0 °C, v a blood pressure of 199/118 mmHg, pulse price 129 beats every minute, and also respiratory price 18 breaths per minute. The oxygen saturation was 93 % when she to be breathing in approximately air. The laboratory data verified a newly-diagnosed DM complex with a hyperosmolar hyperglycemic state (serum glucose at 1836 mg/dl, serum salt 124 mmol/l, hemoglobin A1C 14.4 %, and effective osmolality 350 mOsm/kg). After adequate hydration, insulin therapy and also appropriate antibiotics because that suspected aspiration pneumonia, the patient’s blood sugar improved and also the fever subsided. During hospitalization, blood pressures to be 100–120/60–70 mmHg without any sign of hypertension or indicators suggestive of orthostatic changes.

However, ~ above the 20th hospital day, the patient had actually a second bout the fever v sepsis-like presentation (high fever 39.3 °C, tachycardia 110 beats per minute, tachypnea, however without leukocytosis) the was refractory to antibiotic therapy. Abdominal sonography was performed to search for the infection focus, which revealed an intra-abdominal tumor near the liver and kidney. A succeeding computed tomography shown a 4-cm hard heterogeneous fixed lesion in the best adrenal gland (Fig. 1a). Based upon the patient’s clinical gift (markedly elevated blood press at ED, history of intermittent palpitation, and unusual fever), a pheochromocytoma was very likely. Arsenal of 24-h urine for catecholamine and vanillylmandelic mountain (VMA) levels showed the isolated elevation of epinephrine levels. Repeated collection of urinary examination because that catecholamines and also VMA levels were performed after the patient was cost-free from she acute condition showed comparable results (Table 1). Pre-operative treatment through α- and β-blockade therapy were given to the patient, and she underwent uneventful laparoscopic right adrenalectomy. The pathological examination evidenced our diagnosis of pheochromocytoma (Fig. 1b). The patient remained symptom-free through normal urinary catecholamines excretion ~ 6 months the post-operative follow-up. Her DM is presently under an excellent glycemic control (hemoglobin A1C 6.1 %) utilizing an dental antidiabetic certified dealer (metformin 250 mg daily). Moreover, she gradually gained weight to a body mass index of 23.4 kg/m2.


Abdominal computed tomography mirrors a 4 cm heavy heterogeneous fixed lesion in the right adrenal gland with internal low attenuation foci. No local adenopathy, vascular intrusion or metastatic disease is noticeable (a). Pheochromocytoma cell stained with hematoxylin and also eosin stain, in ~ ×100 magnification under a microscope. The tumor cells have huge round vesicular nuclei and also abundant basophilic come amphophilic cytoplasm. Increased vascularity is noted. Over there is no enhanced mitotic counting (b)

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Table 1 Pre-operative and also post-operative urinary catecholamine parameters
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Pheochromocytoma is a rarely catecholamine-producing tumor that most typically presents through episodes that headaches, sweating, palpitations, and paroxysmal or continual hypertension. However, clinical manifestations of pheochromocytoma are extremely variable, and also can mimic several other diseases, such as acute coronary syndrome, heart failure, and sepsis <1>. Paroxysmal or continual hypertension is among the significant presentations of pheochromocytoma. However, overfill catecholamine secretions in a patient through pheochromocytoma deserve to also an outcome in cardiovascular complications there is no hypertension <2>. Therefore, a stylish diagnosis and also treatment the pheochromocytoma is really important.

Different catecholamine secretions native pheochromocytoma have different effects top top blood glucose and also blood press levels <3>. About 40–50 % of patients through pheochromocytomas clinically present as glucose intolerance or DM, which room such typical pathologic conditions that they can hold-up the fashionable diagnosis the pheochromocytoma. Hyperglycemia has actually been aramuseum.orged to aggravation of insulin resistance resulted in by catecholamine overabundance in pheochromocytoma. The inhibitory effect of catecholamines top top insulin cheap is assumed to be mediated by α-2 adrenergic receptors. The isolated key of epinephrine level in our case had a profound effect on glucose intolerance, inducing lipolysis and hepatic gluconeogenesis, but inhibited peripheral glucose utilization. Around 54 % that adrenergic producing tumors in the adrenal glands space diagnosed in patient of progressed age, similar to our case. The possible mechanism that led to our patient’s presenting with DM complex with hyperosmolar hyperglycemic state may have actually been that the diverted catecholamine excess with epinephrine secreting pheochromocytoma result in rise in lipolysis, which diminished insulin secretion and increased glucose manufacturing from the liver. Although our patient, a couple of months prior to admission presented clinically with intermittent catecholamine overabundance that consisted of intermittent diaphoresis, palpitation, and also occasional headache, there was no evidence of hypertension found during admission. However, unlike the usual presentation where one would intend hypotension from volume depletion at the elevation of the hyperglycemic hyperosmolar state, she had marked hypertension. Additionally, DM patients facility with hyperosmolar hyperglycemic state usually have impaired awareness or have actually a bed-ridden status, which was not regular with our patient. The is therefore important to think about pheochromocytoma as among our differential diagnoses in one ambulatory patient presenting with marked hypertension at the elevation of hyperosmolar hyperglycemic state, specifically when the clinical presentation is various from the usual signs and also symptoms.

Although dimensions of plasma-free metanephrines or urinary fractionated metanephrines room the many sensitive and most reliable indications for the exclusion of pheochromocytoma, these were unavailable in our medical center <4>. In this case, just the classic biochemical exams that encompass collection the 24-h to pee catecholamines and also VMA were performed. However, urine catecholamine concentration increase during pathophysiological or pharmacological adrenosympathetic stimulation, which have the right to lead come the misdiagnosis the pheochromocytomas. Additionally, this bioamines room also very sensitive to diets, medications and adrenosympathetic stimulation, make the interpretations of laboratory results unreliable in the diagnosis and also monitoring the pheochromocytomas. Because that these reasons, a repeated collection of urine because that catecholamines and also VMA levels were performed as soon as the patience recovered from she acute illness. The results confirmed persistent elevation of epinephrine levels, thus, supporting the diagnosis of an epinephrine-producing adrenal tumor. Although VMA has a high specificity rate, the VMA level in our situation was an unreliable marker because that pheochromocytoma.

On early stage presentation, our patient was underweight, and also this might have been due to chronic catecholamine overproduction resulted in by pheochromocytoma that may have actually led come the boost in proinflammatory cytokines and also the hypermetabolic state characterized by boosted resting energy expenditure. Adrenalectomy resulted in the normalization the the power metabolism in our case followed by rise in body mass index and body fat content v a to decrease in inflammation markers and also cytokines <5>. The is therefore of utmost importance, come look because that an undiscovered endocrinopathy with hypermetabolic state in cases of an underweight patience presenting v a hyperosmolar hyperglycemic state not having any previous background of DM.

Here we highlight the importance of completely pheochromocytoma-complicating DM in a case presenting v hyperosmolar hyperglycemic state with significant hypertension and also fever but without a focus of infection.

Conflict of interest

Authors declare that they have actually no conflict of interest.

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All procedures complied with were in accordance with the moral standards of the responsible committees (institutional and national) and also with the Helsinki explanation of 1975, together revised in 2008.

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Informed consent

Informed consent was acquired from the participant because that being contained in this study according to principles Committees requirements.


Division that Endocrinology and Metabolism, department of internal Medicine, Wan Fang Hospital, Taipei clinical 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, university of Medicine, Taipei clinical University, Taipei, Taiwan

Ting-Wei Lee

Department of basic Medicine, college of Medicine, Taipei clinical University, Taipei, Taiwan

Ting-I Lee


Cite this article

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

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Received: 16 January 2015

Accepted: 15 February 2015

Published: 03 march 2015

Issue Date: September 2015

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