Introduction
Pheochromocytoma is a rare, usually benign tumor that develops in the adrenal glands. These glands, located on top of each kidney, produce hormones that regulate various bodily functions. Pheochromocytomas often cause the adrenal glands to release excessive amounts of hormones called catecholamines, including adrenaline and noradrenaline, which can lead to significant health issues.
Symptoms
The symptoms of pheochromocytoma can vary, but they typically result from the excessive production of catecholamines. Common symptoms include:
- High Blood Pressure: This can be persistent or episodic, often leading to headaches, sweating, and palpitations.
- Tachycardia: Rapid heart rate that may be accompanied by chest pain or discomfort.
- Headaches: Often severe and throbbing, usually associated with high blood pressure.
- Excessive Sweating: Profuse sweating, particularly during episodes of high blood pressure.
- Weight Loss: Unintentional weight loss due to the metabolic effects of excess catecholamines.
- Flushing: Reddening of the face and skin, often occurring with other symptoms.
- Anxiety or Panic Attacks: Feelings of anxiety, fear, or panic, sometimes mistaken for psychiatric issues.
Symptom spells
Most often, the symptoms of pheochromocytoma come and go. When they start suddenly and keep coming back, they’re known as spells or attacks. These spells may or may not have a trigger that can be found.
Certain activities or conditions can lead to a spell, such as:
- Physical hard work.
- Anxiety or stress.
- Changes in body position, such as bending over, or going from sitting or lying down to standing.
- Labor and delivery.
- Surgery and a medicine that causes you to be in a sleep-like state during surgery, called an anesthetic.
Foods high in tyramine, a substance that affects blood pressure, also can trigger spells. Tyramine is common in foods that are fermented, aged, pickled, cured, overripe or spoiled. These foods include:
- Some cheeses.
- Some beers and wines.
- Soybeans or products made with soy.
- Chocolate.
- Dried or smoked meats.
Certain medicines and drugs that can trigger spells include:
- Depression medicines called tricyclic antidepressants. Some examples of tricyclic antidepressants are amitriptyline and desipramine (Norpramin).
- Depression medicines called monoamine oxidase inhibitors (MAOIs), such as phenelzine (Nardil), tranylcypromine (Parnate) and isocarboxazid (Marplan). The risk of spells is even higher if these medicines are taken with foods or drinks high in tyramine.
- Stimulants such as caffeine, amphetamines or cocaine.
Causes and Risk Factors
The exact cause of pheochromocytoma is not well understood, but certain genetic factors and conditions can increase the risk. Some of the known risk factors include:
- Genetic Mutations: Inherited genetic syndromes such as Multiple Endocrine Neoplasia type 2 (MEN 2), Von Hippel-Lindau disease, and Neurofibromatosis type 1 can predispose individuals to pheochromocytoma.
- Family History: A family history of pheochromocytoma or related genetic syndromes can increase the risk.
- Age: Pheochromocytomas can occur at any age but are most commonly diagnosed in adults between the ages of 30 and 50.
Diagnosis
Diagnosing pheochromocytoma involves a combination of clinical evaluation, laboratory tests, and imaging studies. Key diagnostic steps include:
- Blood and Urine Tests: Measuring levels of catecholamines and their metabolites (metanephrines) in the blood and urine can help confirm the presence of a pheochromocytoma.
- Blood catecholamine tests: These tests measure the level of catecholamines in your blood. Substances that result from the breakdown of these hormones are also measured. Higher-than-normal levels of certain catecholamines in your blood may be a sign of pheochromocytoma.
- Imaging Studies: Imaging techniques such as computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans can help locate the tumor and determine its size.
- Genetic Testing: Genetic testing may be recommended for individuals with a family history of pheochromocytoma or related genetic syndromes.
Treatment
The primary treatment for pheochromocytoma is surgical removal of the tumor. Additional treatments may include:
- Surgery: Laparoscopic adrenalectomy is a minimally invasive procedure commonly used to remove pheochromocytomas. In some cases, open surgery may be necessary, especially for larger tumors.
- Medications: Alpha-blockers and beta-blockers are often prescribed before surgery to control blood pressure and heart rate by blocking the effects of excess catecholamines.
- Radiation Therapy: In cases where the tumor is malignant or cannot be completely removed, radiation therapy may be used to target and destroy cancerous cells.
- Follow-up Care: Regular follow-up visits and monitoring are essential to detect any recurrence of the tumor or related health issues.
Complications
A pheochromocytoma can lead to other health problems. The high blood pressure linked with a pheochromocytoma can damage organs, especially tissues of the heart and blood vessel system, brain and kidneys. This damage can cause dangerous conditions, including:
- Heart disease.
- Stroke.
- Kidney failure.
- Vision loss.
Nursing Care of a Patient with Pheochromocytoma
Pheochromocytoma is a rare but serious condition characterized by a tumor in the adrenal glands, which can lead to excessive production of catecholamines. This can cause severe hypertension and other cardiovascular complications. Nursing care plays a critical role in managing patients with pheochromocytoma, ensuring their safety, comfort, and recovery.
Preoperative Care
Patient Assessment
- Monitor vital signs, with a particular emphasis on blood pressure and heart rate.
- Assess for symptoms such as headaches, sweating, palpitations, and anxiety.
- Review patient history for any previous episodes of hypertension or related health issues.
Medication Management
- Administer prescribed alpha-blockers and beta-blockers to control blood pressure and heart rate.
- Educate the patient about the purpose and potential side effects of these medications.
- Monitor the patient for any adverse reactions to the medications.
Education and Preparation
- Inform the patient and their family about the surgical procedure and expected outcomes.
- Discuss the importance of preoperative medication adherence.
- Provide emotional support to alleviate anxiety and stress related to the upcoming surgery.
Postoperative Care
Monitoring and Assessment
- Continuously monitor vital signs, focusing on blood pressure, heart rate, and respiratory status.
- Assess for signs of complications such as hemorrhage, infection, or adrenal insufficiency.
- Monitor fluid and electrolyte balance and administer intravenous fluids as prescribed.
Pain Management
- Administer prescribed analgesics to manage postoperative pain.
- Assess pain levels regularly and adjust pain management strategies as needed.
- Encourage non-pharmacological pain relief methods, such as deep breathing exercises and relaxation techniques.
Patient Education
- Educate the patient about the signs and symptoms of potential complications.
- Discuss the importance of follow-up appointments and ongoing monitoring.
- Provide guidance on lifestyle modifications to manage blood pressure and overall health.
Long-Term Care
Follow-Up Visits
- Schedule regular follow-up visits to monitor the patient’s recovery and detect any recurrence of the tumor.
- Conduct routine blood tests and imaging studies as recommended by the healthcare provider.
- Assess the patient’s adherence to prescribed medications and lifestyle changes.
Support and Counselling
- Encourage the patient to maintain a healthy lifestyle with a balanced diet, regular exercise, and stress management techniques.
- Offer emotional support and counseling to help the patient cope with the diagnosis and treatment.
- Provide resources for support groups or mental health professionals if needed.
REFERENCES
- Myo Clinic,Pheochromocytoma ,https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/symptoms-causes/syc-20355367
- Pheochromocytoma. Merck Manual Professional Version. https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma.
- Ferri FF. Pheochromocytoma. In: Ferri’s Clinical Advisor 2024. Elsevier; 2024. https://www.clinicalkey.com.
- What is pheochromocytoma (adrenal medulla tumor)? Urology Care Foundation. https://www.urologyhealth.org/urology-a-z/p/pheochromocytoma-(adrenal-medulla-tumor).
- Young WF. Clinical presentation and diagnosis of pheochromocytoma. https://www.uptodate.com/contents/search.
- Types of blood pressure medications. American Heart Association. https://www.heart.org/en/health-topics/high-blood-pressure/changes-you-can-make-to-manage-high-blood-pressure/types-of-blood-pressure-medications.
- Pheochromocytoma. National Cancer Institute Center for Cancer Research. https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-endocrine-tumor/pheochromocytoma.
- Overview of adrenal function. Merck Manual Professional Version. https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/overview-of-adrenal-function.
- Robertson RP, ed. Pheochromocytoma. In: DeGroot’s Endocrinology: Basic Science and Clinical Practice. 8th ed. Elsevier; 2023. https://www.clinicalkey.com.
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