Disease Description (Causes and Symptoms)
- Definition summary: Pheochromocytoma is a catecholamine (adrenaline, noradrenaline, etc.)-secreting tumor that primarily arises in the adrenal medulla and, rarely, also occurs in sympathetic ganglia (around the aorta, bladder, etc.).
- Causes and heredity: Approximately 10–30% are associated with hereditary conditions (e.g., MEN2/RET, VHL, NF1, SDHx), which may involve multiple tumors and various endocrine organs.
- Pathophysiology: Catecholamines secreted by the tumor cause vasoconstriction and increased heart rate, resulting in hypertension (persistent or paroxysmal), abnormal heart rhythms, hyperglycemia, and other effects.
- Main symptoms: Sweating, palpitations, headache, dizziness, paroxysmal or persistent hypertension, weight loss, anxiety, nausea/vomiting, chest pressure, and numbness or cold sensations in the hands and feet. Some patients may be asymptomatic.
Treatment Methods and Precautions
- Basic principle: When possible, complete surgical removal of the tumor is the first-line treatment.
- Preoperative preparation: Alpha-blocker therapy for at least 10–14 days before surgery to stabilize blood pressure is recommended. If necessary, a beta-blocker may subsequently be added to control the heart rate (however, beta-blockers must be used only after alpha-blockade). Fluid replacement is used to correct blood volume.
- Surgical approach: Laparoscopic or robot-assisted surgery is commonly used, but open surgery may be necessary depending on the tumor size and degree of invasion. Intensive monitoring for bleeding and hemodynamic instability during and after surgery (hypertensive crisis, postoperative hypotension, and shock) is necessary.
- Local nonsurgical treatment: In metastatic or inoperable cases, radionuclide therapy (e.g., MIBG therapy) or targeted/anticancer therapy may be considered. In metastatic malignant cases, chemotherapy (such as CVD) may be used.
- Lifestyle management and precautions:
- Because hypertensive attacks (dangerous sudden rises in blood pressure) may increase the risk of stroke, myocardial infarction, and arrhythmia, specialized treatment and monitoring are essential.
- Certain medications before and after diagnosis or surgery (including some antidepressants, sympathomimetic drugs, and some contrast agents and injectable medications) may trigger attacks or blood pressure fluctuations, so consult the attending physician.
- Because recurrence is possible even after surgery, long-term follow-up (blood pressure, catecholamine-related tests, and imaging) is necessary.
- Preparation for emergencies: Immediate emergency treatment is necessary if severe headache, chest pain, altered consciousness, or similar symptoms occur. Carrying a medical alert card or wearing a medical alert necklace is recommended.
Outpatient Visit Checklist (Items to Bring, Patient Preparation, and Sample Questions)
- Recommended items to bring:
- A list of all medications and supplements currently being taken (including over-the-counter medications and herbal medicines)
- Copies of previous test results (blood tests, 24-hour urine catecholamine/metabolite tests, and CT/MRI/nuclear medicine test results)
- Blood pressure and heart rate records (including measurements during attacks, if possible) and a symptom diary (attack frequency, triggers, and duration)
- Information about family history (especially hereditary endocrine disorders)
- Possibility of pregnancy or breastfeeding status
- Information to tell the attending physician in advance during the outpatient visit: Recent acute symptoms (severe headache, fainting, or chest pain), scheduled tests or procedures (e.g., procedures involving contrast agents), and allergy history.
- Medications that may be recommended for discontinuation before testing (as directed by a physician): Some medications and supplements that may affect catecholamine testing may need to be discontinued, so be sure to ask before the test.
Examples of General Questions to Ask the Doctor
- "What tests (blood, urine, and imaging) are recommended for my condition?"
- "Which medications should I take before surgery and for how long? What are the side effects?"
- "What are the major risks and complications that may occur during and after surgery?"
- "When would treatment other than surgery (radionuclide therapy, anticancer treatment, etc.) be necessary?"
- "What is the risk of recurrence after surgery, and how often should follow-up tests be performed?"
Examples of Disease-Specific Questions
- "Do I need testing for hereditary conditions (e.g., MEN2, VHL, or SDH mutations)? What are the criteria for recommending family testing?"
- "What are the levels of the hormones secreted by my tumor (such as metanephrines), and what do these levels mean for treatment and prognosis?"