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Year : 2021  |  Volume : 8  |  Issue : 5  |  Page : 255-258

A cardiogenic shock in PICU turned out to be a bilateral pheochromocytoma: A case report

Department of Pediatrics, Pramukhswami Medical College, Anand, Gujarat, India

Date of Submission17-Jun-2021
Date of Decision26-Jul-2021
Date of Acceptance03-Aug-2021
Date of Web Publication28-Sep-2021

Correspondence Address:
Dr. Jay Shah
Department of Pediatrics, Pramukhswami Medical College and Shree Krishna Hospital, Gokalnagar, Anand-Sojitra Highway, Karamsad, Anand - 388 325, Gujarat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcc.jpcc_39_21

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Pheochromocytomas (PCCs) are rare catecholamine-secreting tumors arising from adrenomedullary chromaffin cells, usually causing hypertension, palpitation, and headache. It is usually investigated as a secondary cause of hypertension. However, rarely it can present in hypotension. In “PCC crisis,” the patient presents with hypotension, multiple organ dysfunction, leaving the physician with diagnostic difficulties. In this case, we present an adolescent male presented with hypotensive cardiogenic shock, and multiple organ dysfunction on admission, which during investigation turned out to be bilateral PCC.

Keywords: Hypertension, pheochromocytoma, shock

How to cite this article:
Shah J, Patel Z, Patel S, Kumar A. A cardiogenic shock in PICU turned out to be a bilateral pheochromocytoma: A case report. J Pediatr Crit Care 2021;8:255-8

How to cite this URL:
Shah J, Patel Z, Patel S, Kumar A. A cardiogenic shock in PICU turned out to be a bilateral pheochromocytoma: A case report. J Pediatr Crit Care [serial online] 2021 [cited 2021 Oct 26];8:255-8. Available from: http://www.jpcc.org.in/text.asp?2021/8/5/255/326863

  Introduction Top

A pheochromocytoma (PCC) is defined as a tumor deriving from adrenomedullary chromaffin cells that produce one or more catecholamines: Epinephrine, norepinephrine, and dopamine.[1] Typical symptoms include headache, palpitations, excessive sweating, and intermittent or persistent hypertension. Other symptoms such as abdominal pain, shock, respiratory distress syndrome, pulmonary edema, hyperthermia, and cardiogenic shock occur less frequently.[2] PCC crisis is an endocrine emergency associated with significant mortality, defined as the acute severe presentation of catecholamine-induced hemodynamic instability causing end-organ damage or dysfunction.[3] The case presented here serves to illustrate an unexpected presentation of this insidious tumor.

  Case Report Top

A 14-year-male presented to our hospital with c/o not passing stool for 4 days, yellowish discoloration of eyes since 3 to 4 days, cough, and acute onset of breathlessness since 1 day. He did not have fever, vomiting, loose stool, rash, or altered sensorium.

On examination, he was in decompensated shock. Cool peripheries, Pulse-137/min central pulses feeble, and peripheral pulses were not palpable. Respiratory rate-54/min, blood pressure (BP)-not recordable, SPO2 100% on oxygen by facemask.

Although in critical condition from the onset, he had sustained no trauma and used no drugs. He had no recent physical complaints that seemed significant to the presenting symptoms. His past medical history was unremarkable. As it was a fluid refractory shock, the inotropes were started and later increased. Subsequently, Vasopressin has to be added as it was catecholamine-resistant decompensated shock. Initial blood gas analysis revealed severe metabolic and lactic acidosis and bedside echo showed severe left ventricle dysfunction [Table 1] and [Table 2]. He also had acute kidney injury and hepatitis. The child started maintaining BP 12 h after admission and inotropes tapering were initiated. There was an improvement in urine output and acidosis resolved. But on the next day, the child started developing hypertension (BP 180/120) which required labetalol infusion and later on shifted to oral antihypertensive. Despite three antihypertensive drugs BP was poorly controlled. On evaluation for a secondary cause of hypertension-bilateral Doppler reported normal but suggested left suprarenal adrenal mass. Magnetic resonance imaging revealed bilateral adrenal mass consistent with PCC. To rule out multiple sites and metastasis, a whole-body positron emission tomography scan was done. It showed no active uptake apart from the bilateral adrenal region [Figure 1]. The biochemical investigation showed elevated levels of serum metanephrines [Table 1] and [Table 2]. After adequate preoperative preparation, laparoscopic removal of the bilateral adrenal tumor was done [Figure 2]. The intraoperative and immediate postoperative period was uneventful. Histopathological examination of the mass revealed left malignant and right benign PCC. The child was discharged on the 5th day postoperatively on a single antihypertensive.
Table 1: Routine investigation

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Table 2: Special investigation

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Figure 1: PET CT Showing Increased uptake in bilateral adrenal region

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Figure 2: Post-operative adrenal tumor

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  Discussion Top

The typical manifestations of PCC include hypertension, palpitation, pallor, tremor, headache, and diaphoresis.[2] However, the present case featured hypotension, shock, and multiple organ dysfunction on admission. Later, in the process of treatment, the patient developed hypertensive urgency. All pediatric hypertension is recommended to screen for secondary causes, among which renal (parenchymal and vascular) diseases are the largest categories, accounting for 70% to 90% for all cases.[4] During the process of screening, renal disease was ruled out in this case. According to Newell's report, severe PCC could include severe hypertension or hypotension, multi-organ system failure, high fever, and encephalopathy.[5] A PCC without sustained hypotension is classed as “type A crisis,” while a PCC with sustained hypotension is classed as “type B crisis,” which is often presented with shock and multi-organ dysfunction.[4] This child presented as a “type B crisis.” Cardiovascular manifestations are the most common presentations of PCC crisis, including cardiomyopathy, myocardial infarction, arrhythmia, and cardiogenic shock.[6] In this case, the patient presented with shock, severe left ventricular dysfunction, and raised troponin. As he had had symptoms of upper respiratory tract infection with multiple organ dysfunction syndrome, it was hard to distinguish the catecholamine-induced cardiomyopathy from the viral myocarditis. The prolonged stimulation by catecholamine weakens the myocardium contractility.[6] In the present case, the patient had abnormal liver function (alanine aminotransferase [ALT] of 593 IU/L and aspartate aminotransferase of 880 IU/L) which later recovered. Common viral hepatitis was ruled out. Eun et al. reported a case of PCC with abnormal liver function in 2014 with an ALT of 317 IU/L.[7] The abnormal liver function was thought to be associated with repeated overproduction of hepatocytes, which was secondary to the increased resistance of liver arterioles and veins, and decreased blood flow and oxygen led by overstimulation of norepinephrine on a-adrenalin receptors.[8]

The treatment of PCC initially focuses on hemodynamic stabilization of the patient with aggressive intravascular fluid resuscitation as first-line therapy, complemented by vasopressors and inotropes as well as an invasive arterial BP monitoring for additional cardiovascular support if needed. This is then followed by medical treatment with an alpha-blocker such as phenoxybenzamine or prazosin and a beta-blocker such as esmolol.[9] The alpha-blocker should be started at a low dose and up titrated before initiation of beta-blocker therapy to avoid unopposed alpha-receptor stimulation by the excreted catecholamines. This medical therapy reduces the risk of intraoperative hemodynamic changes during tumor resection.[10] Our patient was managed preoperatively with prazosin, metoprolol, and nifedipine and after tumor removal, BP was controlled on nifedipine only. It was continued at discharge and discontinue in follow-up.

Cardiogenic hypotensive shock can be a rare presentation in the PCC crisis. Early diagnosis is essential to initiate adequate therapy and reverse left ventricular dysfunction at an early stage.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, et al. Pheochromocytoma and paraganglioma: An endocrine society clinical practice guideline. J Clin Endocrinol Metab 2014;99:1915-42.  Back to cited text no. 1
Solorzano CC, Parks J, Prinz RA. Pheochromocytoma presenting with multiple organ failure. Am Surg 2008;74:1119-21.  Back to cited text no. 2
Whitelaw BC, Prague JK, Mustafa OG, Schulte KM, Hopkins PA, Gilbert JA, et al. Phaeochromocytoma [corrected] crisis. Clin Endocrinol (Oxf) 2014;80:13-22.  Back to cited text no. 3
Wu R, Tong N, Chen X, Xu S, Zhang F, Tang L, et al. Pheochromocytoma crisis presenting with hypotension, hemoptysis, and abnormal liver function: A case report. Medicine (Baltimore) 2018;97:e11054.  Back to cited text no. 4
Newell KA, Prinz RA, Pickleman J, Braithwaite S, Brooks M, Karson TH, et al. Pheochromocytoma multisystem crisis. A surgical emergency. Arch Surg 1988;123:956-9.  Back to cited text no. 5
Prejbisz A, Lenders JW, Eisenhofer G, Januszewicz A. Cardiovascular manifestations of phaeochromocytoma. J Hypertens 2011;29:2049-60.  Back to cited text no. 6
Eun C, Ahn J, Seo J, Kim N. 2014. Pheochromocytoma with markedly abnormal liver function tests and severe leukocytosis. Endocrinol Metabolism 2014;29:83.  Back to cited text no. 7
Wang P, Tait SM, Chaudry IH. Sustained elevation of norepinephrine depresses hepatocellular function. Biochim Biophys Acta 2000;1535:36-44.  Back to cited text no. 8
Steppan J, Shields J, Lebron R. Pheochromocytoma presenting as acute heart failure leading to cardiogenic shock and multiorgan failure. Case Rep Med 2011;2011:596354.  Back to cited text no. 9
Van Braeckel P, Carlier S, Steelant PJ, Weyne L, Vanfleteren L. Perioperative management of phaeochromocytoma. Acta Anaesthesiol Belg 2009;60:55-66.  Back to cited text no. 10


  [Figure 1], [Figure 2]

  [Table 1], [Table 2]


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