Pheochromocytoma in Children: How to Spot the Warning Signs

Quick Takeaways

• Sudden, severe headaches or abdominal pain in a child may signal pheochromocytoma.
• Unexplained high blood pressure, especially with sweating or rapid heartbeat, is a red flag.
• Episodes of panic‑like symptoms (palpitations, trembling, pale skin) often come in bursts.
• Blood or urine tests for catecholamine metabolites confirm the diagnosis.
• Early surgical removal of the tumor usually cures the condition.

When a kid shows odd spikes in blood pressure or weird panic attacks, most parents think it’s stress or a cold. But a rare adrenal tumor called pheochromocytoma is a catecholamine‑producing tumor that arises from the adrenal medulla can be the hidden cause. Spotting the clues early can prevent dangerous spikes in blood pressure and save a child from a life‑threatening crisis.

What is Pheochromocytoma?

Pheochromocytoma is a tumor that usually forms in the adrenal gland, the small organ perched atop each kidney. The tumor cells release excess catecholamines-mainly adrenaline and noradrenaline-into the bloodstream. In children, this excess creates sudden bursts of symptoms that mimic anxiety, asthma, or a heart problem.

Why It Matters for Kids

Adults with pheochromocytoma often get diagnosed after a routine check‑up for high blood pressure. Kids, however, rarely have regular BP readings, so the tumor can stay hidden until a dramatic episode occurs. Uncontrolled hypertension can damage the brain, kidneys, and heart, leading to permanent issues. Early recognition not only prevents organ damage but also opens the door to a curative surgery.

Typical Signs and Symptoms

The classic triad-headache, sweating, and rapid heartbeat-looks the same in children, but the presentation can be more subtle or intermittent. Below is a quick reference of what to watch for.

Notice that many of these signs come in bursts-minutes to hours-and then disappear. That on‑and‑off pattern is a tell‑tale sign that catecholamines are being released episodically.

When to suspect a Genetic Syndrome

About 30% of pediatric pheochromocytomas are linked to inherited conditions such as von Hippel‑Lindau disease, neurofibromatosis type1, or multiple endocrine neoplasia type2 (MEN‑2). Children with a family history of these syndromes, or who have other related tumors (e.g., retinal angiomas, neurofibromas), should be evaluated early. Genetic testing can pinpoint the mutation and guide both the child’s treatment and family counseling.

How Doctors Confirm the Diagnosis

1. Blood or urine testing - The most reliable markers are plasma free metanephrines or 24‑hour urinary fractionated metanephrines. Elevated levels (>2‑fold the upper limit) strongly suggest pheochromocytoma.

2. Imaging - Once biochemical proof is in hand, a CT scan or MRI of the abdomen pinpoints the tumor’s size and exact location. MRI is preferred for children to reduce radiation exposure.

3. MIBG scan (metaiodobenzylguanidine) - This nuclear medicine test helps locate extra‑adrenal or metastatic disease if the CT/MRI is inconclusive.

4. Genetic panel - If a hereditary syndrome is suspected, a targeted gene panel confirms the underlying mutation.

What to Do if You Spot the Warning Signs

Don’t wait for the next episode. Schedule an appointment with a pediatrician who can refer you to a pediatric endocrinologist or a pediatric surgeon. Bring a written log of blood pressure readings (if you have a home cuff), symptom timing, and any family history of tumors or genetic conditions.

Treatment Overview

Surgical removal of the tumor is the definitive cure. Before surgery, doctors use alpha‑blockade (e.g., phenoxybenzamine) for 10‑14 days to stabilize blood pressure and prevent intra‑operative spikes. Some centers add a beta‑blocker after the alpha block is effective to control heart rate.

Minimally invasive laparoscopic adrenalectomy has become the standard for most pediatric cases, offering quicker recovery and less pain. In rare cases where the tumor is malignant or spreads, additional chemotherapy or targeted therapy may be needed.

Long‑Term Follow‑Up

Even after a successful operation, about 10‑15% of children develop a new tumor later in life, especially those with a genetic syndrome. Annual blood pressure checks and periodic plasma metanephrine tests are advised for at least a decade after surgery.

Key Points for Parents and Caregivers

• Track any sudden episodes of high blood pressure, headaches, or sweating.
• Keep a symptom diary with dates, times, and triggers (like stress or certain foods).
• Ask the doctor about screening for catecholamine levels if the episodes are unexplained.
• If a tumor is found, surgery is usually curative; the pre‑op medication plan is crucial for safety.
• Stay vigilant for recurrence, especially if there’s a family history of related genetic conditions.