Pheochromocytoma Treatment in Delhi, India

A neuroendocrine tumour originating from chromaffin cells is called pheochromocytoma. The chromaffin cells produce hormones of the adrenal gland - adrenaline and noradrenaline. Adrenal glands are small endocrine glands placed above the kidneys. About 80-85% of pheochromocytomas arise from the adrenal medulla, while 15-20% grow extra-adrenally and are termed paragangliomas.

The majority of tumours are benign and don't spread to other parts of the body. However, the tumour releases hormones that may increase blood pressure and cause headaches, sweating, and symptoms similar to a panic attack. If a pheochromocytoma is left untreated, it can be fatal. Most pheochromocytomas affect people aged 20 to 50 years. With the surgical removal of pheochromocytomas, the symptoms also disappear.

Symptoms of Pheochromocytoma

Some symptoms of pheochromocytoma are:

• High blood pressure or hypertension
• Headache
• Tremours
• Paleness in the face
• Shortness of breath
• Heavy sweating
• Rapid heartbeat
• Constipation
• Panic attack-like symptoms
• Anxiety
• Weight loss

Causes of Pheochromocytoma

The exact cause of pheochromocytoma is not known. However, the tumour develops in chromaffin cells situated at the center of the adrenal gland. Chromaffin cells release adrenaline and noradrenaline, hormones controlling heart rate, blood pressure, and blood sugar.

Diagnosis of Pheochromocytoma

The doctors perform a physical exam and ask about medical history and symptoms. The tests they may advise include:

• Abdominal CT scan
• Catecholamines blood test (serum catecholamines)
• Glucose test
• 24-hour urine test for catecholamines and metanephrines.
• Metanephrine blood test (serum metanephrine)
• An imaging test called an MIBG scintiscan
• MRI of the abdomen
• PET scan of the abdomen
• Genetic tests: Doctors sometimes recommend genetic testing to assess if the pheochromocytoma is associated with an inherited disorder. Genetic testing can be useful because:

Some inherited disorders can cause multiple conditions, indicating the need to evaluate and test for other medical disorders.

Some diseases are more likely to be recurrent or cancerous. The test results may impact treatment decisions or long-term plans to monitor health.

The tests may encourage other family members to get checked for pheochromocytoma or other conditions.

Treatment of Pheochromocytoma

The first line of treatment for pheochromocytoma is the surgical removal of the tumour. However, before the surgery, doctors try controlling blood pressure with medications that obstruct the actions of the high-adrenaline hormones. This reduces the risk of developing dangerously high blood pressure during the surgery.

Preoperative preparation

Doctors recommend taking two drugs for seven to ten days before the surgery to control blood pressure, such as alpha-blockers and beta-blockers.

Additionally, by increasing the intake of salt, the blood pressure will not drop to life-threatening levels post-operatively.

• Alpha-blockers- These drugs relax the blood vessels, improve blood flow and thus lower blood pressure. Side effects may include dizziness, fatigue, irregular heartbeat, vision problems, sexual dysfunction in men, and swelling in the limbs.
• Beta-blockers- These drugs lower heart rate and slow the pumping of the heart by relaxing the heart muscles. Additionally, they help relax the blood vessels. This drug is prescribed about seven to ten days before the scheduled date of surgery. The side effects of these drugs are fatigue, dizziness, constipation, diarrhoea, upset stomach, headache, irregular heartbeat, difficulty breathing, and swelling in the limbs.
• High-salt diet: Alpha and beta-blockers broaden the blood vessels, lowering the amount of fluid within the blood vessels. This may lead to a drop in blood pressure to dangerous levels. Salt has a tendency to attract fluids. Hence, a high-salt diet will draw in more fluid within the blood vessels. This prevents the lowering of blood pressure to dangerous levels during and after surgery.

Surgery

In the majority of cases, the surgeons remove the entire adrenal gland with pheochromocytoma. This can be achieved with minimally invasive surgery, depending on the tumour size.

The other adrenal gland, or any remaining adrenal gland, is left to perform its usual functions. As a result, the blood pressure returns to normal within a few weeks of surgery.

Cancer treatments

Only a handful of pheochromocytomas are malignant. The studies about the treatment of malignant pheochromocytomas are thus limited. Treatment options for malignant tumours and metastasized (spread to other body parts) related to a pheochromocytoma are:

• MIBG: This is a form of radiation therapy that combines MIBG, a compound that attaches to adrenal tumours, with a type of radioactive iodine. The therapy aims to provide radiation therapy to a specific site and eliminate malignant cells.
• Peptide receptor radionuclide therapy (PRRT): This therapy combines a drug that targets cancer cells and a small amount of a radioactive substance. Radiation is delivered directly to the abnormal cancer cells.
• Chemotherapy: Chemotherapy is the most efficient type of treatment. Administration of potent drugs during chemotherapy kills the rapidly proliferating cancer cells.
• Radiation therapy: Radiation therapy helps with the symptomatic treatment of tumours that have spread to other organs.
• Targeted cancer therapies: These medicines hamper the function of innately occurring molecules that stimulate the growth and spread of cancerous cells.

Complications of Pheochromocytoma

Hypertension caused by pheochromocytoma can damage multiple organs, such as the cardiovascular system, brain, and kidneys. This damage can cause numerous critical conditions, such as:

• Stroke
• Heart disease
• Renal failure
• Problems with the nerves of the eye

Risk Factors of Pheochromocytoma

People with rare inherited disorders carry an increased possibility of having pheochromocytoma or paraganglioma. Tumours associated with these disorders have a high chance of being cancerous. These genetic disorders may include:

• Multiple endocrine neoplasias, type 2 (MEN 2)
• Von Hippel-Lindau disease
• Neurofibromatosis 1 (NF1)
• Hereditary paraganglioma syndromes

Prognosis of Pheochromocytoma

Most people with benign tumours removed with surgery can live for about five years. However, recurrence has been seen in some cases. After the tumour is surgically removed, the levels of hormones return to normal.

Blood pressure may remain high even after the surgery. However, this can be controlled with medicines and making certain lifestyle changes.

People successfully treated for pheochromocytoma should get regularly tested to ensure that the tumour has not recurred. Family members can also benefit from testing because some cases may show prominent inheritance.

Review

Reviewed by Dr. Anshu Alok , Senior Consultant - Endocrinology & Diabetes.