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What’s Causing This Teenager’s Chest Pain and Syncope Symptoms?

A 17-year-old presented to the emergency department (ED) after his mother witnessed him having a syncopal episode. He also complained of intermittent left-sided chest pain, which he described as nonradiating, sharp, pulsating, and partially alleviated by walking. He rated the pain’s intensity as 6 on a 10-point scale.

Four days before presentation, he had experienced transient lightheadedness, diaphoresis, and left-sided chest pain, which had spontaneously resolved. Two days later, similar symptoms awoke him from sleep and lasted for a few minutes; during that episode, he also was dyspneic, collapsed while getting out of bed, and regained consciousness spontaneously.

The review of systems was otherwise unremarkable, and there was no family history of cardiovascular, oncologic or neurologic disease. He denied drug use.

On physical examination in the ED, he was diaphoretic and in moderate physical distress. He was afebrile with normal vital signs. Cardiac examination revealed a regular heart rate and a nondisplaced point of maximal impulse. There were no murmurs, rubs, gallops, or jugular venous distention. Chest wall examination results were unremarkable, with equal and clear breath sounds. Cranial nerves were intact. He had nontender, mobile lymphadenopathy of the left anterior cervical chain, bilateral supraclavicular nodes, and right axillary nodes. The rest of the examination findings were normal.

Results of cardiac enzymes tests, urinalysis, and electrocardiography (ECG) were normal. An initial chest radiograph was ordered, the results of which are shown below.

Based on his presentation and imaging study results, what is causing this teen’s chest pain and syncope?

(Answer and discussion on next page)

Answer: Mediastinal mass

A chest radiograph showed a widened mediastinum with a prominent cardiac silhouette (Figure 1). Further diagnostic workup was obtained; a computed tomography scan of the chest showed an extensive infiltrating mediastinal mass (Figure 2), and an echocardiogram exhibited early diastolic collapse of the right ventricle and atrium, mitral valve flow variation, and a pericardial effusion indicating tamponade physiology.

Figure: A chest radiograph of a 17-year-old with chest pain and syncope showed a wide mediastinum and prominent cardiac silhouette.

Results of cardiac enzymes tests and urinalysis were normal, but his white blood cell count was 28,900/µL (reference range, 4,000-10,000/µL). The peripheral blood smear showed anisopoikilocytosis, polychromasia, thrombocytopenia, and leukocytosis with 44% blasts; no Auer rods were seen. The lactate dehydrogenase level was 1,555 U/L (reference range, 105-333 U/L). Results of flow cytometry testing were consistent with a diagnosis of T-cell acute lymphoblastic leukemia.

Chest pain and syncope are common pediatric symptoms. The most common causes are benign and rarely life threatening, and they include musculoskeletal, pulmonary, psychogenic, and gastrointestinal etiologies.1,2 Cardiac disease accounts for only 1% of cases of pediatric chest pain and syncope.1-3

Although malignant etiologies of chest pain and syncope can be present in adults,4-7 they are rare in children. In a study of 3,700 children with chest pain, for example, only 1.3% of cases were associated with syncope.2 In adults, few published cases describe syncope without chest pain as the presenting symptom in a malignancy-associated cardiac mass.8,9 A report of a case in an adolescent describes exercise-induced syncope with superior vena cava syndrome secondary to a mediastinal mass, but the patient had no chest pain or respiratory symptoms.10

Figure: Coronal view (left) and sagittal view (right) computed tomography scans showed a soft tissue mass infiltrating the 17-year-old’s mediastinum.

Mediastinal masses usually present with nonspecific symptoms such as cough, chest pain, fever, and dyspnea.11 Because most serious causes of pediatric chest pain and syncope are associated with cardiac conditions, current guidelines place little emphasis on malignant etiologies.

Given the frequency of chest pain and syncope in the pediatric population, standardized clinical assessment and management plans (SCAMPs) are available in many states to help reduce unnecessary testing.1,2,12-15 According to SCAMPs for pediatric syncope and chest pain, only an abnormal cardiac history and examination findings, along with an abnormal ECG result, warrant further testing and referral to a specialist. Otherwise, reassurance and supportive treatment are recommended. Furthermore, abnormal lymphadenopathy on physical examination is not included in SCAMP criteria for further testing, and chest imaging is not part of the recommended diagnostic workup. However, our patient’s case demonstrates that benign cardiac evaluation results and a normal ECG do not preclude the need for further testing.

It is important to differentiate benign causes of chest pain and syncope from serious causes. Because a delay in diagnosis can be fatal or result in a poor long-term prognosis, a thorough physical examination and a detailed patient history to uncover any signs of more serious etiologies should be a part of the initial evaluation (Table). Painless lymphadenopathy in the setting of chest pain and syncope may suggest malignancy; in such cases, chest imaging and timely evaluation are indicated. Mediastinal masses should be included in the differential diagnosis of chest pain and syncope in children, especially after cardiac causes are ruled out. Immediate evaluation is needed when a mediastinal mass is detected.

Carlos A. Pérez, MD, is a resident in the Division of Child and Adolescent Neurology in the Department of Pediatrics at the University of Texas Health Science Center at Houston.

David E. McMann, MD, is a resident in the Division of Community and General Pediatrics in the Department of Pediatrics at the University of Texas Health Science Center at Houston.

Lynnette Mazur, MD, MPH, is a professor in the Division of Community and General Pediatrics in the Department of Pediatrics at the University of Texas Health Science Center at Houston. 

James R. Murphy, PhD, is a professor in the Division of Pediatric Infectious Diseases in the Department of Pediatrics at the University of Texas Health Science Center at Houston.

Guenet Degaffe, MD, is an assistant professor in the Division of Community and General Pediatrics in the Department of Pediatrics at the University of Texas Health Science Center at Houston.

References

1. Friedman KG, Alexander ME. Chest pain and syncope in children: a practical approach to the diagnosis of cardiac disease. J Pediatr. 2013;163(3):896-901.

2. Saleeb SF, Li WYV, Warren SZ, Lock JE. Effectiveness of screening for life-threatening chest pain in children. Pediatrics. 2011;128(5):e1062-e1068.

3. Wang T-F, Chu S-C, Wu M-H, Lo RY-Y, Li C-C. Recurring syncope as initial presenting symptom of non-small cell lung cancer — a case report. Tzu Chi Med J. 2006;18(5):378-381.

4. Shenoy C. An uncommon cause of syncope. QJM. 2008;101(3):241.

5. Di Valentino M, Menafoglio A, Wyttenbach R, Gallino A. An unusual case of recurrent syncope. J Am Coll Cardiol. 2011;58(16):1728.

6. Hahn B, Rao S, Shah B. Case report of precursor B-cell lymphoblastic lymphoma presenting as syncope and cardiac mass in a nonimmunocompromised child. Pediatr Emerg Care. 2007;23(8):576-579.

7. Driscoll DJ, Glicklich LB, Gallen WJ. Chest pain in children: a prospective study. Pediatrics. 1976;57(5):648-651.

8. Chen HY. Recurrent syncope as initial symptom in apical intrathoracic tumor. J Clin Med Res. 2012;4(1):77-80.

9. Manojkumar R, Sharma A, Grover A. Secondary lymphoma of the heart presenting as recurrent syncope. Indian Heart J. 2001;53(2):221-223.

10. Cabeza Martín B, Pérez Suárez E, Iglesias Bouzas MI, Pérez Martínez A, Tamariz Martel A, Aleo E. Syncope in the debut of a mediastinal mass [in Spanish]. Arch Argent Pediatr. 2012;110(2):e29-e31.

11. Juanpere S, Cañete N, Ortuño P, Martínez S, Sanchez G, Bernado L. A diagnostic approach to the mediastinal masses. Insights Imaging. 2013;4(1):29-52.

12. Farias M, Jenkins K, Lock J, et al. Standardized clinical assessment and management plans (SCAMPs) provide a better alternative to clinical practice guidelines. Health Aff (Millwood). 2013;32(5):911-920.

13. Friedman KG, Kane DA, Rathod RH, et al. Management of pediatric chest pain using a standardized assessment and management plan. Pediatrics. 2011;128(2):239-245.

14. Verghese GR, Friedman KG, Rathod RH, et al. Resource utilization reduction for evaluation of chest pain in pediatrics using a novel standardized clinical assessment and management plan (SCAMP). J Am Heart Assoc. 2012;1(2):jah3-e000349.

15. Rathod RH, Farias M, Friedman KG, et al. A novel approach to gathering and acting on relevant clinical information: SCAMPs. Congenit Heart Dis. 2010;5(4):343-353.