Pheochromocytoma Treatment in Mumbai, India

Pheochromocytoma is a rare non-cancerous (benign) tumor that develops in the adrenal gland. It usually develops in the center (medulla) of one or both adrenal glands. In patients with pheochromocytoma, the tumor releases hormones that may cause high blood pressure, headache, sweating, and symptoms of a panic attack. If a pheochromocytoma is left untreated, it can cause severe or life-threatening damage to other organs.

Associated Anatomy

The adrenal medulla

Alternate Name

Adrenal medullary tumor

Pheochromocytomas Symptoms

• Rapid heartbeat
• Headache
• Shortness of breath
• Heavy sweating
• High blood pressure
• Panic attack
• Stomach upset
• Paleness in the face
• Weight loss
• Tremors

Pheochromocytoma Causes

In most cases, the exact cause of pheochromocytoma is unknown. Most cases occur randomly, mostly genetic factors play a role in some cases.

• Genetic mutation: Pheochromocytomas result from genetic disruptions or changes (mutations) to specific genes in approximately 35% of cases.
• Role of hormones: Pheochromocytoma causes an excessive release of adrenal gland hormones, leading to an unwarranted increase in blood pressure and heart rate.

Pheochromocytoma Alternate Types

Paraganglioma: Paragangliomas are usually non-cancerous (benign). However, some paragangliomas can become cancerous and spread (metastasize) to other parts of the body. The tumor commonly secretes hormones known as catecholamines, including adrenaline, which can cause episodes of high blood pressure, rapid heartbeat, sweating, headache and tremors.

Pheochromocytoma Stages

There is no such particular standard for the staging of pheochromocytoma or paraganglioma. Each tumor will be described as localised, regional, or metastatic:

• Localized pheochromocytoma. It is the early stage, tumor has been detected in the adrenal medulla of the adrenal glands.
• Regional pheochromocytoma or paraganglioma. Cancer has spread from where the tumor began to nearby surrounding tissues and/or lymph nodes.
• Metastatic pheochromocytoma or paraganglioma. In this stage, cancer spreads from the origin to the distant part of the body.
• Localized paraganglioma. This is an early stage, the tumor is detected in one area of the body only.

Pheochromocytoma Tests

• Laboratory tests of patients are the primary means to detect a pheochromocytoma.
• Blood or urine tests: These tests show if the person has high adrenaline or noradrenaline levels in his/her body.
• Imaging tests: A CT or MRI scan of the adrenal glands may confirm the presence of a tumor.
• Genetic tests: If investigations confirm the presence of a tumor, the doctor may recommend a suitable genetic test that can show if the person has a genetic mutation that increases the risk of this and/or another tumor type.

Pheochromocytoma Treatment

• Medications: Drugs are often administered to control high blood pressure and block the effect of excessive hormones.
• Surgery: You will most likely need surgery to remove the tumor. This tumor of the adrenal gland can be removed by laparoscopic surgery or open surgery, wherein the healthy portions of the adrenal glands are kept intact.
• Radiation therapy and chemotherapy: Radiation therapy and chemotherapy treatments are used when pheochromocytoma has metastasized.

Pheochromocytoma Risk Factors

If you have pheochromocytoma, you may have other genetic conditions that increase your chance of developing more aggressive forms of pheochromocytoma. These genetic conditions consist of:

• Multiple endocrine neoplasias, type II (MEN2), a form of thyroid cancer that develops in conjunction with pheochromocytoma.
• Von Hippel-Lindau (VHL) syndrome, a condition where cysts and tumors grow in the central nervous system, kidneys, adrenal glands, or other areas of the body, most are non-cancerous.
• Neurofibromatosis type 1 (NF1) results in tumors on the skin and optic nerves.
• Hereditary paraganglioma syndrome.

Pheochromocytoma Complications

If you don't undergo treatment, pheochromocytoma can lead to life-threatening conditions, including:

• Stroke
• Pulmonary oedema
• The disease of your heart muscle (cardiomyopathy)
• Kidney failure
• Heart attack
• Heart muscle inflammation (myocarditis)
• Bleeding in the brain
• Nerve damage in the eyes

Pheochromocytoma Preventions

Primary Prevention

There are no specific ways by which you can prevent the occurrence of pheochromocytoma. The only known risk factor is hereditary and one’s genetic makeup cannot be altered.

Secondary Prevention

The best choice for treating pheochromocytoma is the removal of the tumor through surgery, which eventually also treats hypertension.

• Preoperative blockade of adrenal hormones to prevent cardiovascular complications is recommended.
• Blood pressure and heart rate are normalised with the help of preoperative medical treatment.
• Provide volume expansion with an isotonic sodium chloride solution.
• To prevent severe hypotension after surgery, salt intake should be encouraged.
• Immediately after surgery, a few parameters like glucose levels, heart rate and blood pressure should be monitored.

Pheochromocytoma Diagnosis

Pheochromocytoma must be differentiated from others, including alcohol withdrawal, labile essential hypertension, hyperventilation, multiple pharmacologic agents, migraine, autonomic neuropathy (baroreflex failure), stroke, toxemia of pregnancy, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, Acute intermittent porphyria, cardiogenic pulmonary oedema, and renovascular hypertension.

Epidemiology

• Pheochromocytomas are rare, reportedly occurring in 0.05–0.2% of individuals with hypertension. This accounts for only a small portion of cases because many patients may be asymptomatic.
• In 50% of patients with pheochromocytoma, the diagnosis is usually made at autopsy. Approximately 10% of pheochromocytomas are discovered incidentally.
• Black populations tend to have lower levels of Pheochromocytomas compared to other races.
• Pheochromocytomas may occur in individuals of any age, but the peak incidence is from the third to the fifth decades of life.
• It has been reported that children contribute approximately 10% of the cases. Around 50% of pheochromocytoma cases involving children are solitary intra-adrenal lesions, 25% are present bilaterally, and 25% are extra-adrenal.

Prognosis

Expected Prognosis

Patients reporting less or lower pheochromocytoma with limited spread have about a 95% of survival rate of five-year. Patients with pheochromocytomas that have grown back (recurred) or metastasised have a five-year survival rate ranging between 34% and 60%.

Natural Progression

If left untreated, an emergency could be caused because of hyperglycemia and hypertension, leading to heart failure and cerebrovascular strokes. If it is not treated properly, it can metastasise to the lungs, liver, bones, and lymph nodes. If left untreated, it can cause life-threatening complications.

Pathophysiology

Pheochromocytoma is effectuated by the secretion of catecholamines and ensuing stimulation of adrenergic receptors. It appears from the chromaffin cells of the adrenal medulla and sympathetic ganglia. The pathophysiology is not dependent on the histological subtypes. The histological and biochemical properties are the same and shared by both malignant and benign pheochromocytomas.