|Year : 2019 | Volume
| Issue : 14 | Page : 118-120
Palpitation in a known hypertensive patient, looking beyond the obvious: A case of pheochromocytoma coexisting with hypothyroidism
Akolade O Idowu1, Andrew O Mene2, Omotayo F Salami3, Theophilus O Ajiro4, Daniel E Ezuduemoih4, Clement G Inyang5
1 Department of Medicine, Ben Carson School of Medicine, Babcock University Ilishan Remo; Department of Medicine, Babcock University Teaching Hospital, Ilishan Remo, Nigeria
2 Department of Surgery, Ben Carson School of Medicine, Babcock University Ilishan Remo, Ilishan Remo, Nigeria
3 Department of Anaesthesia, Ben Carson School of Medicine, Babcock University Ilishan Remo, Ilishan Remo, Nigeria
4 Department of Medicine, Babcock University Teaching Hospital, Ilishan Remo, Nigeria
5 NNPC Medical Services Mosimi, Ogun State, Nigeria
|Date of Submission||01-Jan-2019|
|Date of Acceptance||01-Jun-2019|
|Date of Web Publication||04-Oct-2019|
Dr. Akolade O Idowu
Department of Medicine, Ben Carson School of Medicine, Babcock University Ilishan Remo, Ogun State
Source of Support: None, Conflict of Interest: None
Pheochromocytoma is an uncommon tumor arising from the chromaffin cells of the adrenal medulla. It is a potential life-threatening endocrine disorder. We report a case of a 56-year-old woman who presented on account of recurrent episodes of palpitation with frontal headache that was associated with visual flashes and episodic sweating. Abdominopelvic computed tomography scan showed a well-circumscribed homogeneous mass within the left adrenal gland. The urinary metanephrine and normetanephrine were markedly raised, with an assessment of pheochromocytoma made. Pheochromocytoma should be strongly considered in any patient presenting with palpitations and other neurological symptoms after ruling out cardiac abnormalities. It is important to screen for secondary cause of hypertension in a newly diagnosed hypertensive with ultrasound, which is cheap and readily available. Likewise, ambulatory blood pressure monitoring is highly recommended in any patient with strong suspicion of pheochromocytoma but with normal blood pressure to demonstrate the lability in the pattern.
Keywords: Abdominal ultrasound, ambulatory blood pressure monitor, hypothyroidism, Nigeria, pheochromocytoma
|How to cite this article:|
Idowu AO, Mene AO, Salami OF, Ajiro TO, Ezuduemoih DE, Inyang CG. Palpitation in a known hypertensive patient, looking beyond the obvious: A case of pheochromocytoma coexisting with hypothyroidism. N Niger J Clin Res 2019;8:118-20
|How to cite this URL:|
Idowu AO, Mene AO, Salami OF, Ajiro TO, Ezuduemoih DE, Inyang CG. Palpitation in a known hypertensive patient, looking beyond the obvious: A case of pheochromocytoma coexisting with hypothyroidism. N Niger J Clin Res [serial online] 2019 [cited 2019 Oct 20];8:118-20. Available from: http://www.mdcan-uath.org/text.asp?2019/8/14/118/268525
| Introduction|| |
Pheochromocytoma is an uncommon tumor arising from the chromaffin cells of the adrenal medulla. A similar neoplasm arising from the chromaffin cells located outside the adrenal medulla is termed extra-adrenal pheochromocytoma, which may be located within or outside the abdomen. It can occur as an isolated endocrine disorder or in association with some other neuroendocrine neoplasm as a syndrome.
Pheochromocytoma is a potential life-threatening endocrine disorder, especially when the diagnosis is missed. A previous study examining series of necropsy report of suspected cases showed that pheochromocytoma accounted for 55% of total cause of mortality, with 3 of 4 cases undiagnosed before death.
Palpitations which are defined as abnormal awareness of heart beat have several cardiac and noncardiac causes, but the occurrence of palpitations with other neurological symptoms should make pheochromocytoma to be considered as a very likely diagnosis.
The estimated incidence of pheochromocytoma in the general populace and hypertensive populace has been found to be between 0.005% and 0.1% and between 0.1% and 0.2%, respectively, which has made the diagnosis to be perceived as a remote possibility by many clinicians. In addition, the nonspecific nature of the symptoms of pheochromocytoma which tends to mimic several disorders, but with the catastrophic consequence of missing the diagnosis, provided the justification for this report.
In this article, we also report and discuss the coexistence of hypothyroidism with pheochromocytoma, a finding which has only being reported once in the literature to the best of our knowledge.
| Case Report|| |
A 56-year-old woman presented at a peripheral hospital in the preceding 2 years on account of recurrent episodes of palpitation which was acute in onset, regular in rhythm with each episode lasting 2 min before aborting spontaneously. She also complained of recurrent frontal headache that was throbbing in nature and occurs intermittently, 7 out of 10 in severity, relieved by ingestion of analgesics, and was associated with visual flashes and episodic sweating. On examination, she was found to be hypertensive; her initial general workup showed primary hypothyroidism, with diagnosis of tension-type headache by a neurologist. However, she presented at our facility on account of persistent palpitation and headache with recent-onset epigastric pain, dizziness, vomiting, and generalized body weakness of 2 weeks' duration.
There was positive history of constipation, but no heat or cold intolerance, muscle weakness or paresthesia or family history of endocrine disorder. She denied usage of recreational drugs or self-medication and no psychiatric illness. She was diagnosed diabetic 2 weeks before presentation. Her drug history includes tablets amlodipine, atenolol, metformin/vildagliptin, and levothyroxine.
Physical examination revealed a middle-aged woman, who was acutely ill-looking and mildly dehydrated. The following were the findings noted on systemic examination: pulse rate 85 beats/min and blood pressure (BP) 128/82 mmHg with first and second heart sound heard. There was epigastric tenderness on abdominal examination. No significant finding in other systems.
The full blood count and erythrocyte sedimentation rate was normal. The electrolyte/urea/creatinine result showed azotemia. Thyroid function test revealed elevated thyroid-stimulating hormone and reduced free thyroid hormones. Random blood glucose was 80 mg/dl. Liver function test showed mildly elevated liver enzymes. The abdominopelvic computed tomography (CT) scan showed a well circumscribed homogeneous mass within the left adrenal gland [Figure 1]. The electrocardiogram report was normal. The value of urinary metanephrine and urinary normetanephrine was 2301 nmol/24 h (375–1506) and 3277 nmol/24 h (573–1932), respectively. The esophagogastroduodenoscopy carried out showed erosive gastritis.
|Figure 1: The coronal image of the contrast-enhanced abdominopelvic computed tomography scan of the patient with the arrow pointing at the left adrenal mass|
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The assessment of pheochromocytoma was made, and the urology team were invited to review and comanage the patient. She was initially commenced on tablets telmisartan 80 mg daily, aldomet 250 mg tid, indapamide 1.5 mg daily, rabeprazole 20 mg bid, and metformin/vildagliptin 1 g/50 mg daily. However, monitoring of the BP on hourly basis showed a BP range of 109/49–280/130 within a 24-h period. As soon as it was available, tablet phenoxybenzamine 20 mg bid was commenced, to which was added tablet propranolol 40 mg bid after 48 h for effective adrenergic receptors' blockage. She subsequently had open left adrenalectomy, and the histology of adrenal gland sample confirmed pheochromocytoma. She achieved sustained clinical improvement and was discharged home on the 11th day postopertative. She is currently on routine follow-up visit, urinary catecholamine level has returned to normal, and she is asymptomatic.
| Discussion|| |
There is only one available literature on pheochromocytoma and hypothyroidism coexisting in the same patient. The simultaneous involvement of adrenal gland and thyroid gland which manifests as pheochromocytoma and medullary thyroid cancer, respectively, with affection of other system in the body is a widely recognized features of multiple endocrine neoplasia 2.
The triad of palpitation, headache, and episodic sweating is seen in more than half of patients with pheochromocytoma. Palpitation is a common presentation in the symptomatology of pheochromocytoma arising from increased elaboration of catecholamines, but which is rarely linked to the latter at the early stage, leading to serious sequelae when diagnosed late. The patient complained of palpitation and other neurological symptoms such as headache, sweating, and dizziness which have a high discriminative value for pheochromocytoma; but, due to low index of suspicion, this diagnosis was not considered initially. Palpitation is a nonspecific symptom, with most of the etiology being benign; but, in some cases, it may be a feature of life-threatening condition. Other causes of excessive catecholamines' production leading to palpitation apart from pheochromocytoma include period of stress, exercise, and medications.
Paroxysmal hypertension is seen in about half of patients with pheochromocytoma. One of the postulated mechanisms in the pathophysiology of hypertension in a hypothyroid patient is the increased production of catecholamine as evidenced by higher plasma concentration of norepinephrine in hypothyroid patient in comparison to euthyroid patient. Another pathway implicated is the increased activity of the sympathetic nervous system. It is assumed that the additive effect of catecholamine excess in both pheochromocytoma and hypothyroidism may worsen the hypertension in affected patient than in patients with pheochromocytoma who are euthyroid. However, a further study will be needed to shed more light on this hypothesis.
Abdominal ultrasound has been found to be reliable in detecting the presence of adrenal mass and to characterize it into either benign or malignant lesion. It is also cheap and relatively simple to use in ruling out pheochromocytoma. Pheochromocytoma appears on ultrasound scan as a homogeneous or complex mass/cystic change in a solid mass. It is worthy of note that the abdominal CT scan of the patient showed a well-circumscribed homogenous and hypodense mass, which would have otherwise supported the diagnosis of adrenal adenoma than pheochromocytoma in the absence of other ancillary investigations.
Abdominal ultrasound has a sensitivity and specificity of 100% in detecting adrenal mass >3 cm which is comparable to abdominal CT scan and magnetic resonance imaging (MRI) of 100% and 100%, respectively, but can detect adrenal mass <3 cm in 65.2% of cases as compared to abdominal CT scan and MRI of 100% and 100%, respectively.
Ambulatory blood pressure monitoring (ABPM) device though expensive and not widely available helps in diagnosing hypertension with high accuracy. It will be quite useful to show the lability of the BP in pheochromocytoma patient. The lability of the BP was first demonstrated in our patient after continuous monitoring of the BP hourly over 24 h. Considering the propensity of pheochromocytoma to cause lethal hypertensive crisis arising from massive release of catecholamine into the circulation, the place of ABPM in a patient with pheochromocytoma having normal BP during evaluation cannot be overemphasized.
24-h ABPM has been found to be very important in distinguishing essential from secondary causes of hypertension, as it is useful in demonstrating the circadian pattern of BP fluctuations, as the distortion of this pattern characterized by nocturnal rise of BP is a well-recognized cardiovascular risk factor.
| Conclusion|| |
Pheochromocytoma should be strongly considered in any patient presenting with palpitations and other neurological symptoms after ruling out cardiac abnormalities.
It is important to screen for secondary cause of hypertension including pheochromocytoma in a newly diagnosed hypertensive with ultrasound, which is cheap and readily available. Likewise, ABPM is highly recommended in any patient with strong suspicion of pheochromocytoma but with normal BP to demonstrate the lability in the pattern.
| Consent|| |
Informed consent was obtained from the patient for the publication of this case report and the accompanying images.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Sutton MG, Sheps SG, Lie JT. Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series. Mayo Clin Proc 1981;56:354-60.
Mittendorf EA, Evans DB, Lee JE, Perrier ND. Pheochromocytoma: Advances in genetics, diagnosis, localization, and treatment. Hematol Oncol Clin North Am 2007;21:509-25.
Pederson LC, Lee JE. Pheochromocytoma. Curr Treat Options Oncol 2003;4:329-37.
Bartolomei C, Gianchecchi D, Chiavistelli P, Lenzi R. Pheochromocytoma and autoimmune hypothyroidism. Minerva Med 1992;83:485-6.
Cotesta D, Petramala L, Serra V, Pergolini M, Crescenzi E, Zinnamosca L, et al.
Clinical experience with pheochromocytoma in a single centre over 16 years. High Blood Press Cardiovasc Prev 2009;16:183-93.
Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd
ed. Philadelphia (PA): Saunders; 2004. p. 1420.
Coulombe P, Dussault JH, Walker P. Plasma catecholamine concentrations in hyperthyroidism and hypothyroidism. Metabolism 1976;25:973-9.
Polikar R, Kennedy B, Ziegler M, O'Connor DT, Smith J, Nicod P. Plasma norepinephrine kinetics, dopamine-beta-hydroxylase, and chromogranin-A, in hypothyroid patients before and following replacement therapy. J Clin Endocrinol Metab 1990;70:277-81.
Fan J, Tang J, Fang J, Li Q, He E, Li J, et al.
Ultrasound imaging in the diagnosis of benign and suspicious adrenal lesions. Med Sci Monit 2014;20:2132-41.
Suzuki Y, Sasagawa, Suzuki H, Izumi T, Kaneko H, Nakada T, et al.
The role of ultrasonography in the detection of adrenal masses: Comparison with computed tomography and magnetic resonance imaging. Int Urol Nephrol 2001;32:303-6.
Yu R, Nissen NN, Chopra P, Dhall D, Phillips E, Wei M. Diagnosis and treatment of pheochromocytoma in an academic hospital from 1997 to 2007. Am J Med 2009;122:85-95.
Wang YC, Koval AM, Nakamura M, Newman JD, Schwartz JE, Stone PW. Cost-effectiveness of secondary screening modalities for hypertension. Blood Press Monit 2013;18:1-7.