ILLUSTRATIVE CASE
Extra-adrenal Paraganglioma Presenting as Acute Chest Pain Mimicking Acute Myocardial Infarction in a 10-Year-Old Boy Ming-Yu Hsieh, MD,*† Teng-Fu Tsao, MD,*‡ Yu-Hua Chao, MD,§k Pei-Fen Liao, MD,§ and Ji-Nan Sheu, MD, PhD*§ Abstract: Extraadrenal paragangliomas are rare pediatric tumors with variable symptoms that cause difficultly in diagnosis and delayed treatment. We report the case of a 10-year-old boy who presented to the pediatric emergency department with acute chest pain and dyspnea mimicking a non–ST-segment elevation acute myocardial infarction. He was subsequently found to have an extraadrenal paraganglioma after a series of imaging and laboratory examinations. The mass was subsequently removed, and the diagnosis of extraadrenal paraganglioma was confirmed histologically. Acute coronary syndrome as the presenting feature of extraadrenal paragangliomas in pediatric population is an even rarer clinical entity. Key Words: extraadrenal paraganglioma, chest pain, acute myocardial infarction (Pediatr Emer Care 2014;30: 818–821)
P
heochromocytomas are rare catecholamine-secreting neuroendocrine tumors arising from chromaffin tissue in the adrenal medulla or extraadrenal paraganglia that account for less than 0.1% of the hypertensive population.1 Extraadrenal paragangliomas are rarer (10% of all pheochromocytomas) and seen mainly in the second and third decades of life.2 Patients with pheochromocytoma may present with variable symptoms, usually manifesting with headache, palpitation, diaphoresis, and paroxysmal hypertension.1,2 Pheochromocytomas have been rarely associated with acute myocardial infarction (AMI) and other cardiovascular complications, especially in the pediatric population.3–9 This report is of a 10-year-old boy with an extraadrenal paraganglioma unusually presenting as acute chest pain and dyspnea mimicking a non–ST-segment elevation AMI (NSTEMI). To our knowledge, this is the first report of an extraadrenal paraganglioma presenting as acute coronary syndrome in children.
CASE A previously healthy 10-year-old boy was referred to the pediatric emergency department with the complaint of acute chest pain and dyspnea after running in school. His chest pain was characterized by acute onset, tightness and fullness in the chest, and exacerbation with associated exercise. He did not have any medical history of hypertension, Kawasaki disease, and underlying cardiac disease. His body temperature was 36.2°C, pulse rate was 124 beats per minute, and blood pressure 105/65 mm Hg on arrival. His heart sounds were regular without murmur. Chest x-ray film was normal, From the *School of Medicine, Chung Shan Medical University; and Departments of †Pediatric Surgery, ‡Medical Imaging, and §Pediatrics, Chung Shan Medical University Hospital; and kInstitute of Medicine, Chung Shan Medical University, Taichung, Taiwan. Disclosure: The authors declare no conflict of interest. Reprints: Ji-Nan Sheu, MD, PhD, Department of Pediatrics, School of Medicine, Chung Shan Medical University, No. 110, Section 1, Jianguo North Rd, Taichung 402, Taiwan (e‐mail: [email protected]). Copyright © 2014 by Lippincott Williams & Wilkins ISSN: 0749-5161
818
www.pec-online.com
and electrocardiogram showed a sinus tachycardia without signs of myocardial ischemia (Fig. 1). Blood investigations revealed elevated troponin I level (3.31 ng/mL; reference, 0–0.4 ng/mL), with normal levels of creatine kinase (CK, 158 IU/L; reference, 32–180 IU/L) and creatine kinase-myocardial band (CK-MB, 14 IU/L; reference, 0–24 IU/L). The patient was admitted to the pediatric intensive care unit under the initial impression of a NSTEMI. However, echocardiography showed a normal-sized heart with good left ventricular contractility and normal-sized coronary arteries. There was no regional wall motion abnormality or pericardial effusion, indicating that no local infarction was present. On chest computed tomography, an ill-defined, poor enhancing mass 5.0 2.5 cm in size in the right upper abdomen, near the right adrenal gland region, was an incidental finding (Fig. 2A, B). Subsequent abdominal magnetic resonance imaging revealed a paraadrenal gland mass closely attached to the posterior wall of the inferior vena cava (Fig. 2C, D). Subsequently, the 24-hour urinary catecholamines and vanillylmandelic acid values revealed markedly elevated norepinephrine level (370.3 μg/d; reference, 11.1–85.5 μg/d) but within normal levels of epinephrine (
Extra-adrenal Paraganglioma Presenting as Acute Chest Pain Mimicking Acute Myocardial Infarction in a 10-Year-Old Boy Ming-Yu Hsieh, MD,*† Teng-Fu Tsao, MD,*‡ Yu-Hua Chao, MD,§k Pei-Fen Liao, MD,§ and Ji-Nan Sheu, MD, PhD*§ Abstract: Extraadrenal paragangliomas are rare pediatric tumors with variable symptoms that cause difficultly in diagnosis and delayed treatment. We report the case of a 10-year-old boy who presented to the pediatric emergency department with acute chest pain and dyspnea mimicking a non–ST-segment elevation acute myocardial infarction. He was subsequently found to have an extraadrenal paraganglioma after a series of imaging and laboratory examinations. The mass was subsequently removed, and the diagnosis of extraadrenal paraganglioma was confirmed histologically. Acute coronary syndrome as the presenting feature of extraadrenal paragangliomas in pediatric population is an even rarer clinical entity. Key Words: extraadrenal paraganglioma, chest pain, acute myocardial infarction (Pediatr Emer Care 2014;30: 818–821)
P
heochromocytomas are rare catecholamine-secreting neuroendocrine tumors arising from chromaffin tissue in the adrenal medulla or extraadrenal paraganglia that account for less than 0.1% of the hypertensive population.1 Extraadrenal paragangliomas are rarer (10% of all pheochromocytomas) and seen mainly in the second and third decades of life.2 Patients with pheochromocytoma may present with variable symptoms, usually manifesting with headache, palpitation, diaphoresis, and paroxysmal hypertension.1,2 Pheochromocytomas have been rarely associated with acute myocardial infarction (AMI) and other cardiovascular complications, especially in the pediatric population.3–9 This report is of a 10-year-old boy with an extraadrenal paraganglioma unusually presenting as acute chest pain and dyspnea mimicking a non–ST-segment elevation AMI (NSTEMI). To our knowledge, this is the first report of an extraadrenal paraganglioma presenting as acute coronary syndrome in children.
CASE A previously healthy 10-year-old boy was referred to the pediatric emergency department with the complaint of acute chest pain and dyspnea after running in school. His chest pain was characterized by acute onset, tightness and fullness in the chest, and exacerbation with associated exercise. He did not have any medical history of hypertension, Kawasaki disease, and underlying cardiac disease. His body temperature was 36.2°C, pulse rate was 124 beats per minute, and blood pressure 105/65 mm Hg on arrival. His heart sounds were regular without murmur. Chest x-ray film was normal, From the *School of Medicine, Chung Shan Medical University; and Departments of †Pediatric Surgery, ‡Medical Imaging, and §Pediatrics, Chung Shan Medical University Hospital; and kInstitute of Medicine, Chung Shan Medical University, Taichung, Taiwan. Disclosure: The authors declare no conflict of interest. Reprints: Ji-Nan Sheu, MD, PhD, Department of Pediatrics, School of Medicine, Chung Shan Medical University, No. 110, Section 1, Jianguo North Rd, Taichung 402, Taiwan (e‐mail: [email protected]). Copyright © 2014 by Lippincott Williams & Wilkins ISSN: 0749-5161
818
www.pec-online.com
and electrocardiogram showed a sinus tachycardia without signs of myocardial ischemia (Fig. 1). Blood investigations revealed elevated troponin I level (3.31 ng/mL; reference, 0–0.4 ng/mL), with normal levels of creatine kinase (CK, 158 IU/L; reference, 32–180 IU/L) and creatine kinase-myocardial band (CK-MB, 14 IU/L; reference, 0–24 IU/L). The patient was admitted to the pediatric intensive care unit under the initial impression of a NSTEMI. However, echocardiography showed a normal-sized heart with good left ventricular contractility and normal-sized coronary arteries. There was no regional wall motion abnormality or pericardial effusion, indicating that no local infarction was present. On chest computed tomography, an ill-defined, poor enhancing mass 5.0 2.5 cm in size in the right upper abdomen, near the right adrenal gland region, was an incidental finding (Fig. 2A, B). Subsequent abdominal magnetic resonance imaging revealed a paraadrenal gland mass closely attached to the posterior wall of the inferior vena cava (Fig. 2C, D). Subsequently, the 24-hour urinary catecholamines and vanillylmandelic acid values revealed markedly elevated norepinephrine level (370.3 μg/d; reference, 11.1–85.5 μg/d) but within normal levels of epinephrine (
