Pheochromocytoma is a type of tumor that arises from specialized cells known as chromaffin cells. These cells are responsible for producing essential hormones within the body and are predominantly located in the adrenal glands.

Pheochromocytomas are typically non-cancerous (benign) tumors originating from chromaffin tissue. They are most commonly found within the adrenal glands but can also occur in sympathetic ganglia, which are nerve clusters along the spinal column. These tumors are characterized by their secretion of catecholamines, such as noradrenaline (norepinephrine) and adrenaline (epinephrine).

Clinical Presentation

Individuals with pheochromocytoma may experience a range of symptoms, including:

• Headaches: Often severe and episodic.

• Heavy sweating (hyperhidrosis): Excessive perspiration, sometimes occurring in bouts.

• A rapid heartbeat (tachycardia): An increased heart rate, potentially feeling like palpitations.

• High blood pressure (hypertension): Blood pressure that is consistently elevated, or spikes suddenly.

• A pale face (pallor): Unusual paleness of the skin, particularly on the face.

• Feeling of being sick (nausea): A sensation of unease in the stomach, sometimes leading to vomiting.

• Feeling anxious or panicky: Unexplained feelings of worry, nervousness, or sudden fear.

• Shakiness (tremor): Involuntary trembling or shaking, often noticeable in the hands.

• Paroxysmal hypertension: Sudden, unpredictable episodes of very high blood pressure.

• Flustering (agitation): A state of being visibly worried or tense.

• Chest discomfort: Pain or tightness in the chest area.

Diagnosis

The diagnosis of pheochromocytoma is often considered based on a combination of clinical features, sometimes referred to as the “5 H’s”:

• Hypertension: Elevated blood pressure.

• Headache: Often severe and episodic.

• Hyperhidrosis: Excessive sweating.

• Hypermetabolism: Increased metabolic rate, even at rest.

• Hyperglycemia: Elevated blood sugar levels.

Key diagnostic tests include:

• Urinary catecholamine tests: Measuring the levels of catecholamines and their breakdown products in the urine collected over a 24-hour period. This is a direct and informative test for diagnosing the condition.

• Serum epinephrine and norepinephrine levels: Blood tests to measure the levels of these specific hormones in the bloodstream. Elevated levels are indicative of the tumor.

  • It’s important to avoid certain substances for 24 hours before a 24-hour urine collection to prevent false results. These include coffee, tea, bananas, chocolate, vanilla, aspirin (ASA), nicotine, amphetamines, and decongestants.

• Clonidine suppression test: Clonidine is a medication that typically suppresses the release of catecholamines in individuals without pheochromocytoma. Failure of catecholamine levels to decrease after clonidine administration suggests the presence of an autonomously secreting tumor.

• Imaging studies: Techniques used to locate the tumor.

  • CT Scan (Computed Tomography): Uses X-rays to create detailed cross-sectional images of the body.

  • MRI (Magnetic Resonance Imaging): Uses magnetic fields and radio waves to create detailed images of the organs and tissues.

How the Tumour Affects the Adrenal Glands

The adrenal glands are small organs located above the kidneys. They are responsible for producing vital hormones, including adrenaline (epinephrine) and noradrenaline (norepinephrine). These hormones are released into the bloodstream as needed to regulate various bodily functions. They play a critical role in controlling:

• Heart rate: How fast the heart beats.

• Blood pressure: The force of blood against the artery walls.

• Metabolism: The chemical processes that the body uses to convert food and oxygen into energy to keep organs functioning properly.

A pheochromocytoma disrupts this normal hormonal balance. The tumor causes the adrenal glands (or other chromaffin tissue) to produce an excessive amount of adrenaline and noradrenaline. This hormonal overproduction leads to the characteristic symptoms, particularly the fluctuations in heart rate and the development of high blood pressure.

Management

The primary goal in managing pheochromocytoma is to address the excess hormone production and remove the tumor.

• Surgery to remove the phaeochromocytoma: This is the preferred treatment method as most of these tumors are benign and surgical removal is typically successful in resolving the condition.

• Alpha blockers (and in some cases beta blockers): These medications are typically prescribed for several weeks leading up to the surgery. They work by counteracting the effects of the excessive adrenaline and noradrenaline on the body. Specifically, they help to stabilize heart rate and blood pressure, reducing the risks associated with surgery.

• The operation to remove the tumor can be performed using different surgical techniques:

  • “Keyhole” (laparoscopic) surgery: This minimally invasive approach involves making several small incisions. Specialized instruments are then inserted through these incisions to carefully remove the tumor. This is the most common surgical method for pheochromocytomas due to its smaller incisions, less pain, and faster recovery.

  • Open surgery: This traditional method involves making a single, larger incision to directly access and remove the tumor. The incision is usually made in the abdomen (tummy).

• If phaeochromocytoma is cancerous: In rare cases where the tumor is malignant (cancerous), treatment may involve a combination of approaches. In addition to surgery, chemotherapy (using drugs to kill cancer cells) or radiotherapy (using high-energy radiation to destroy cancer cells) may be necessary.

Nursing Care

Nursing care for patients with pheochromocytoma is tailored to the specific treatment received and their individual needs. This includes:

• Providing standard post-surgical care for patients recovering from either laparoscopic or open surgery.

• Managing any complications arising from surgery or the medical management.

• Monitoring vital signs closely, particularly blood pressure and heart rate.

• Administering prescribed medications, such as alpha and beta blockers.

• Providing education and support to the patient and their family regarding the condition, treatment, and follow-up care.