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Table of Contents
Year : 2021  |  Volume : 5  |  Issue : 4  |  Page : 260-262

Challenging anesthesia in pediatric pheochromocytoma and paraganglioma tumors resection

Department of Anesthesiology, Pain Management, and Intensive Care, Faculty of Medicine, Udayana University/Sanglah General Hospital Bali, Bali, Indonesia

Date of Submission08-Jun-2021
Date of Decision14-Aug-2021
Date of Acceptance25-Aug-2021
Date of Web Publication24-Nov-2021

Correspondence Address:
Dr. Putu Kurniyanta
Department of Anesthesiology and Intensive Care, Faculty of Medicine, Udayana University, Jl. PB Sudirman Denpasar, Bali 80225.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/bjoa.BJOA_67_21

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Pheochromocytoma (PCC) and paraganglioma (PGL) are chromaffin cell tumors that secrete catecholamines and are some of the rarest pediatric tumors. Perioperative care poses a challenge for the anesthetist. Hemodynamic regulation, surgery manipulation, and perioperative care require special attention. These tumors provide major treatment challenges as well as a high risk of hypertensive crisis-related cardiovascular consequences. We present a successfully managed case of PCC removal in a 13-year-old male. He presented with typical hypertensive crisis symptoms (i.e., vomiting, headaches, and seizures), and he was managed well to prepare him for surgery. We anticipated hemodynamic alterations during the surgery and controlled them with a combination of antihypertension, vasodilator, and epidural analgesia. The safe perioperative care of such patients requires good communication between an experienced multidisciplinary team of surgeons, pediatric endocrinologists, and anesthetists.

Keywords: Adrenal gland neoplasms, catecholamines, hemodynamics, perioperative care

How to cite this article:
Kurniyanta P, Putra KH. Challenging anesthesia in pediatric pheochromocytoma and paraganglioma tumors resection. Bali J Anaesthesiol 2021;5:260-2

How to cite this URL:
Kurniyanta P, Putra KH. Challenging anesthesia in pediatric pheochromocytoma and paraganglioma tumors resection. Bali J Anaesthesiol [serial online] 2021 [cited 2022 May 28];5:260-2. Available from: https://www.bjoaonline.com/text.asp?2021/5/4/260/330954

  Introduction Top

Pediatric cancers such as pheochromocytoma (PCC) and paraganglioma (PGL) are uncommon. The chromaffin cell tumors PCC and PGL produce catecholamines and belong to the neuroendocrine tumor family. In children, they are responsible for 0.5%–2% of secondary hypertension cases. In these patients, hypertension is a key clinical symptom, and the treatment is permanent. The annual incidence of PCC and PGLs is two to five occurrences per million, with only 10% of cases occurring in youngsters.[1],[2]

PCC is a tumor that develops from chromaffin cells in the adrenomedullary system, generating catecholamines. PGL is a tumor that develops from sympathetic or parasympathetic ganglia extra-adrenal chromaffin cells. The sympathetic paravertebral ganglia are found in the thorax, abdomen, and pelvis, whereas the parasympathetic ganglia are found in the neck and at the base of the skull along the glossopharyngeal and vagal nerves.[1],[2]

  Case Report Top

A 15-year-old male, 36 kg, was referred from a district hospital to our facility, presenting with two episodes of seizures one day before admission. Each seizure was brief, lasting about 2 min, which began with severe headache and vomiting. On presentation, he was conscious and hypertensive (200/100 mmHg). He was diagnosed with a hypertensive crisis, and we started intravenous (IV) nicardipine titration at 0.5–4.5 mcg/kg/min rate, phenobarbital 3 mg/kg/day IV, furosemide 40 mg every 12 h IV, and captopril 50 mg every 8 h orally. The goal for hemodynamic regulation was a 20% reduction of mean arterial pressure and a heart rate of around 80–100 beats/min. The next day, we performed an abdominal CT scan and it revealed an adrenal gland mass highly suspect for PCC and PGL. Other radiological examinations were unremarkable. On the eighth day of treatment, we managed to reach all our preoperative targets regarding his hemodynamic status, and surgery was scheduled on the 12th day of hospitalization.

Anesthesia management during induction, surgery manipulation, and hemodynamic control posed a very challenging problem. A standard patient monitoring was applied, along with invasive arterial blood pressure and central venous catheter. Baseline vital signs were quite stable, with blood pressure 156/74 mmHg and 110 beats/min pulse. The patient was premedicated with 2 mg of midazolam and clonidine 45 mcg in the preparation room. General anesthesia was administered with Fentanyl 5 mcg/kg, Propofol 2 mg/kg, and Rocuronium 1.2 mg/kg to facilitate intubation in the operating theater. We used continuous propofol 20–35 mg/h combined with epidural analgesia using plain bupivacaine 0.25% 10 ml. The patient’s vitals were stable during the course of the surgery, but there were abrupt changes in vitals during the manipulation of the tumor by the surgeon. Several hypertensive events occurred during the surgery, which we successfully managed with Nicardipine 2–4.5 mcg/kg/min and Nitroglycerine 3–5 mcg/kg/min.

During direct surgical manipulations by the surgeon, these highly vascular tumors cause catecholamine release. The presence of PGL near the abdominal aorta was further complicating the tumor resection. The resultant effect was hypertension during tumor manipulation since these three tumors shared a common characteristic. After resection of the adrenal and PGL tumor, the patient became hypotensive and tachycardic with blood pressure 75/43 mmHg and pulse 115–133 beats/min. So we stopped both antihypertensive agents and gave the patient fluid resuscitation with 1,500 ml ringer lactate solution and 500 ml packed red cell transfusion. We also started continuous dobutamine at 2.5–7.5 mcg/kg/min and norepinephrine at 0.05–0.70 mcg/kg/min to stabilize the blood pressure.

The surgical team was also notified regarding the hemodynamic changes, which may be caused by tumor ligation with contracted plasma volume, surgical bleeding, and anesthesia-induced vasodilatation. The hemodynamic changes during the surgery are presented in [Figure 1]. Two masses were resected from the right adrenal and left adrenal glands [Figure 2], and one mass from the paraaortic area. The mass was then sent for pathology evaluation, which was suggestive of PCC of the right and left adrenals with PCC of the Adrenal gland Scaled Score (PASS) score of 6 and PGL in the aorta (PASS score 8). The total duration of the operation was 5 h 20 min.
Figure 1: Hemodynamic findings (systolic: blue, diastolic: red, heart rate: black)

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Figure 2: Phechromocytoma (A) and paraganglioma tumor (B)

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After the surgery, the patient was extubated and treated in the Pediatric Intensive Care Unit (PICU). In the PICU, the patient was treated with continuous nicardipine drip titration, epidural analgesia, Captopril 50 mg every 8 h orally, paracetamol, and IV Ceftriaxone 750 mg every 12 h. Blood pressure fluctuations in the PICU were 110–150 for systolic blood pressure and 65–90 for diastolic blood pressure. The patient recovered well and was discharged 14 days after the surgery.

  Discussion Top

In pediatrics, the typical age of presentation for PCCs and PGLs is 11–13 years old, with an M:F ratio of 2:1. The clinical presentation varies, although persistent hypertension is evident in 60%–90% of patients. Adults, on the other hand, are more likely to have paroxysmal hypertension. Headaches, palpitations, sweating, and other signs of catecholamine excess such as pallor, tremor, anxiety, orthostatic hypotension, and syncope are all common clinical presentations.[1],[2]

Dilated cardiomyopathy, ventricular failure, myocardial infarction, arrhythmia, stroke, and other cardiovascular ischemic disorders can result from long-term exposure to catecholamines buildup. The presence of PGL near the abdominal aorta is a unique condition that can also cause hypertension during tumor manipulation. These three tumors posed challenges to anesthetists and surgeons because of their similar characteristics and difficult location.

Our patient presented with hypertension, headache, nausea, vomiting, and a history of seizures. High blood pressure was recognized during a seizure. There was no previous hypertension treatment or history of hypertension in the family. However, in the intermediate ward, he appeared conscious and required a combination of antihypertensive agents to control blood pressure. Preoperative α-blockade is a routine procedure and should be started at least 7–14 days before surgery. The goal is to control arterial pressure before surgery and then restore blood volume thereafter. Phenoxybenzamine and doxazosin are two common alpha-blockers.[3],[4] Tachycardia may result from epinephrine, a dopamine secreting tumor, or a reflex tachycardia secondary to α2-receptor blockade.

To avoid any upsurges in catecholamine release, the main aim is to overcome the patient’s anxiousness before the operation. At least one night before the surgery, a long-acting benzodiazepine (such as lorazepam or diazepam) should be used.[5] The anesthetic technique should avoid drugs that induce catecholamine release, minimize hemodynamic responses to tumor manipulation, and provide easy access to treat episodes of hypotension, particularly after tumor devascularization.

Surgical manipulation can cause severe hypertension, bradycardia, and tachyarrhythmias. Vasodilators are used to treat hypertensive crises, whereas alpha-blockers are used to treat tachyarrhythmias. Phentolamine, sodium nitroprusside, glyceryl trinitrate, nicardipine, esmolol, and labetalol have all been effectively utilized.[1],[2],[6] A selective α2-adrenergic agonist (i.e., dexmedetomidine) has been successfully reported as controlling hemodynamics during PCC resection.[5] Epidural analgesia was chosen as an adjunct to pain control and also to help control blood pressure. A vasoactive drug, such as DA-1, norepinephrine, and vasopressin, can also help, especially in cases of refractory circulatory shock, when acute vasopressin treatment is increasingly recommended.[5],[6],[7],[8]

  Conclusion Top

PCC and PGL anesthetic treatment presents a distinct challenge to the anesthetist. To create a safe perioperative plan, you must first grasp the underlying pathophysiology and pharmacology. The safe perioperative care of these patients requires good communication between an experienced multidisciplinary team of surgeons, pediatric endocrinologists, and anesthetists.

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

Bholah R, Bunchman TE. Review of pediatric pheochromocytoma and paraganglioma. Front Pediatr 2017;5:155.  Back to cited text no. 1
Babiker A, Al Hamdan W, Kinani S, Kazzaz Y, Habeb A, Al Harbi T, et al. Perioperative control of paroxysmal hypertension using esmolol with alpha-blockade in a child with a germline mutated paraganglioma. Endocrinol Diabetes Metab Case Rep 2021;2021:20-0101. doi:10.1530/EDM-20–0101.  Back to cited text no. 2
Ramakrishna H. Pheochromocytoma resection: Current concepts in anesthetic management. J Anaesthesiol Clin Pharmacol 2015;31:317-23.  Back to cited text no. 3
[PUBMED]  [Full text]  
Jia X, Guo X, Zheng Q. Perioperative management of paraganglioma and catecholamine-induced cardiomyopathy in child: A case report and review of the literature. BMC Anesthesiol 2017;17:142.  Back to cited text no. 4
Thius AD, Kurniyanta P, Putra KAH, Sinardja K. Perioperative anesthetic management in pediatric with pheochromocytoma tumor resection. Bali Journal of Anesthesiology 2019;3:125-8. doi:10.15562/bjoa.v3i2.155  Back to cited text no. 5
Teng L, Kit S, Wai C, Fai L. Anesthetic management of pheochromocytoma in pediatric patient—Case report. Open Journal of Anesthesiology 2021;11:175-83. doi: 10.4236/ojanes.2021.116017.  Back to cited text no. 6
Muse IO, Vivek K, Bloomgarden NA, Singla A, Geller DS. Perioperative management of metastatic paraganglioma-pheochromocytoma of the humerus with the aid of regional anesthesia. Case Rep Anesthesiol 2020;2020:2482793.  Back to cited text no. 7
Jeon S, Cho AR, Ri HS, Lee HJ, Hong JM, Lee D, et al. The effect of combined epidural-general anesthesia on hemodynamic instability during pheochromocytoma and paraganglioma surgery: A multicenter retrospective cohort study. Int J Med Sci 2020;17:1956-63.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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