Pediatric Pheochromocytoma Clinical Presentation

  • Author: Patricia Myriam Vuguin, MD, MSc; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Oct 4, 2011
 

History

Pheochromocytomas typically produce paroxysmal episodes that may include any of the following:

  • Hypertension (80%)
  • Diaphoresis (71%)
  • Palpitation with or without tachycardia (64%)
  • Pallor (40%)
  • Nausea with or without vomiting (42%)
  • Tremor (31%)
  • Weakness or exhaustion (28%)
  • Nervousness or anxiety (22%)
  • Epigastric pain (22%)
  • Chest pain (19%)
  • Dyspnea (19%)
  • Flushing or warmth (18%)
  • Numbness or paresthesia (11%)
  • Blurred vision (11%)
  • Tightness of throat
  • Dizziness
  • Convulsion
  • Pain in the neck, shoulder, extremities, or flank
  • Tinnitus
  • Dysarthria
  • Unsteadiness

These paroxysms occur at varying intervals, from several times a day to once every month or more; however, in children, hypertension is most often sustained. All patients with pheochromocytoma experience hypertension at some point.

Hypertension appears to be uniformly present and is sustained in 80-90% of affected children at the time of diagnosis. Occasionally, children with sustained hypertension also have paroxysmal episodes. The paroxysms are occasionally precipitated by excitement or a particular physical activity, such as bending over or lifting a heavy object. Convulsions secondary to hypertensive encephalopathy may occur.

Wide fluctuations in blood pressure are characteristic, and marked increases may be followed by hypotension and syncope. When the blood pressure is elevated, postural hypotension may also be present.

Other associated cardiovascular complications seen mainly in adults include serious ventricular arrhythmias or conduction disturbances, reversible dilated or hypertrophic cardiomyopathy, and Takotsubo cardiomyopathy (also known as stress-induced cardiomyopathy).[11]

Headache is the most frequent symptom in children (75%), followed by sweating in two thirds of patients and nausea and vomiting in half of patients. These headaches are usually described as pounding.

Pallor is usually present because of the intense alpha-receptor–mediated peripheral vasoconstriction, which causes cool, moist hands and feet, and facial pallor.

Palpitations, mediated by beta1 receptors, reflect increased cardiac output and heart rate.

Hyperthermia or flushing secondary to decreased heat loss and increased metabolism leads to reflex sweating.

Poor weight gain or severe cachexia may develop because of hypermetabolism. The child may have a good appetite but, because of hypermetabolism, does not gain weight.

Polyuria and polydipsia may result from increased glycolysis and alpha-receptor–mediated inhibition of insulin release. This insulin inhibition causes an increase in blood sugar levels and glucose intolerance. As a result, patients may present with diabetes mellitus or glucose intolerance, most commonly during paroxysms.

Hypercalcemia is an uncommon but well-recognized complication that may reflect associated hyperparathyroidism, particularly in familial cases.

A syndrome consisting of watery diarrhea, hypokalemia, and achlorhydria secondary to the ectopic production of vasoactive intestinal peptide has been described. This syndrome, along with other laboratory markers of dehydration, such as elevation of the blood urea (BUN) level and hematocrit, usually resolves when the tumor is removed.

The clinical course of pheochromocytoma may be adversely affected by drugs or diagnostic studies that affect catecholamine metabolism, such as opiates, cold medicine, decongestants, and some contrast dyes.

In severe cases, precordial pain may radiate into the arms. Pulmonary edema and cardiac and hepatic enlargement may also develop.

Affected children are often emotionally labile and have an anxious expression. Occasionally, these children are labeled hyperactive with an attention deficit disorder.

Nocturnal enuresis that does not respond to fluid restriction and voiding before bedtime may develop.

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Physical Examination

Patients with pheochromocytomas usually have a thin body habitus. The presence of obesity does not rule out pheochromocytomas, however.

Hypertension may be present in both arms and legs. During a paroxysm, the blood pressure may range from 180-260 mm Hg systolic and from 120-210 mm Hg diastolic. Upon cardiovascular examination, tachycardia with forceful heartbeat is often found and is easily palpable. Postural hypotension may be present

Patients may feel warm and have pallor of the face and chest. Body perspiration and cool, moist hands and feet may also be found.

A mass may be palpable in the neck or in deep palpation of the abdomen. Deep palpation of the abdomen may produce a typical paroxysm.

Hypertensive retinopathy and cardiomyopathy are often present. Ophthalmoscopic examination may reveal papilledema, hemorrhages, exudates, and arterial constriction.

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Contributor Information and Disclosures
Author

Patricia Myriam Vuguin, MD, MSc  Associate Professor of Pediatrics, Department of Pediatric Endocrinology, Albert Einstein College of Medicine; Consulting Staff, Children's Hospital at Montefiore

Patricia Myriam Vuguin, MD, MSc is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephan A Grupp, MD, PhD  Director, Stem Cell Biology Program, Department of Pediatrics, Division of Oncology, Children's Hospital of Philadelphia; Associate Professor of Pediatrics, University of Pennsylvania School of Medicine

Stephan A Grupp, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD  Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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Axial, T2-weighted MRI scan showing large left suprarenal mass of high signal intensity on a T2-weighted image. The mass is a pheochromocytoma.
Abdominal CT scan demonstrating left suprarenal mass of soft tissue attenuation representing a paraganglioma.
 
 
 
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