Pheochromocytoma & Paraganglioma

Specialized Care for Pheochromocytoma & Paraganglioma

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that can cause the body to produce excess stress hormones, leading to serious symptoms such as high blood pressure, headaches, sweating, and heart palpitations. While uncommon, these tumors can have significant health consequences if left untreated.

At Providence Saint John’s Health Center, our Endocrine Tumor Program provides specialized, coordinated care for patients with adrenal and extra-adrenal neuroendocrine tumors.

We are focused on delivering personalized care that improves outcomes and supports long-term health.

What Are Pheochromocytoma & Paraganglioma?

Pheochromocytomas and paragangliomas are tumors that arise from chromaffin cells of the neuroendocrine system. These cells normally help regulate the body’s response to stress by producing catecholamines such as adrenaline and noradrenaline. When tumors develop, excess hormone release can occur—often in unpredictable episodes.

Pheochromocytoma

Pheochromocytomas develop in the adrenal glands, which sit above each kidney and play a key role in hormone regulation. These tumors are more likely to produce hormones and frequently cause sudden spikes in blood pressure and other symptoms.

Paraganglioma

Paragangliomas form outside the adrenal glands, commonly along the spine or in the neck, chest, abdomen, or pelvis. Some paragangliomas produce hormones, while others do not. Because of their varied locations and behavior, diagnosis and treatment often require specialized imaging and multidisciplinary expertise.

Signs and Symptoms

Symptoms vary depending on whether the tumor produces hormones and may occur in episodes triggered by stress, physical activity, or certain foods.

Causes and Risk Factors

Many pheochromocytomas and paragangliomas occur sporadically, with no known family history. However, up to 30–40% are linked to inherited genetic mutations.

Hereditary conditions associated with PPGLs include:

• Multiple Endocrine Neoplasia type 2 (MEN2)
• Von Hippel-Lindau (VHL) syndrome
• Hereditary paraganglioma-pheochromocytoma syndromes
• Neurofibromatosis type 1 (NF1)

Because of this strong genetic component, genetic counseling and testing are recommended for all patients diagnosed with PPGLs. Results help guide treatment decisions and long-term surveillance for patients and their families.

Diagnosis of Pheochromocytoma & Paraganglioma

Accurate diagnosis requires a step-by-step approach combining hormone testing, advanced imaging, and genetic evaluation.

Biochemical Testing

Initial testing determines whether excess hormones are present and may include:

• Plasma free metanephrines
• 24-hour urine metanephrines
• Catecholamine testing

These tests confirm whether the tumor is hormonally active.

Imaging Studies

Once hormone excess is identified, imaging is used to locate and characterize the tumor. Studies may include:

• CT or MRI of the abdomen and adrenal glands
• MRI of the chest, spine, or pelvis
• Functional imaging, such as MIBG scans or PET-CT for complex or metastatic disease

Genetic Testing

Given the high rate of hereditary involvement, genetic counseling is a critical part of care. Genetic findings influence treatment planning and determine appropriate long-term monitoring strategies.