Disease Description (Causes and Symptoms)
- Definition and pathophysiology: A malignant tumor arising in the adrenal cortex that can metastasize to surrounding tissues (kidneys, liver, lungs, bones, etc.) through blood vessels and lymphatic vessels. If the tumor excessively secretes hormones, it can cause systemic metabolic and endocrine abnormalities, while nonfunctioning tumors are often discovered incidentally.
- Main causes/risk factors: Most cases are sporadic (low genetic association), but some are associated with genetic syndromes (Li-Fraumeni, Beckwith-Wiedemann, MEN-1, familial adenomatous polyposis, etc.). Other reported risk factors include a high-fat diet, smoking, lack of physical activity, and exposure to carcinogens.
- Main symptoms:
- Functioning tumors (approximately 60%): Cushing-like symptoms due to excessive corticosteroid secretion — moon face, central (abdominal) obesity, muscle atrophy (proximal muscle weakness), edema, acne, hypertension, diabetes, irregular menstruation and hirsutism (in women), etc.
- Nonfunctioning tumors (approximately 40%): May be discovered without symptoms, or may cause abdominal discomfort, bloating, pain, indigestion, etc., as the tumor increases in size.
- With metastasis: Lymph node enlargement or specific symptoms depending on the metastatic organ (bone pain, shortness of breath, etc.).
- General symptoms: Loss of appetite, weight loss, anemia.
Treatment Methods
- General principles: When possible, curative surgery (complete resection) is best. If there is local invasion or metastasis, the extent and method of surgery may be limited and systemic treatment is required.
- Surgery: Adrenalectomy (open, laparoscopic, or robotic; transperitoneal or retroperitoneal approach) — simultaneous resection of surrounding organs (kidneys, liver, spleen, etc.) and metastatic sites may be considered. Complete resection improves prognosis.
- Drug treatment:
- Mitotane — a cytotoxic drug specific to adrenocortical cells, used as adjuvant therapy and for advanced disease.
- Chemotherapy — combination regimens such as EDP (etoposide + doxorubicin + cisplatin) may be used as the standard treatment in advanced/metastatic cases.
- Radiation therapy: Mainly for adjuvant treatment, pain relief, or local control (such as bone metastases).
- Endocrine management: Before and after removal of a functioning tumor, correction of excessively secreted hormones is needed, as well as glucocorticoid (steroid) supplementation due to temporary relative adrenal insufficiency (adrenal insufficiency) after surgery.
- Supportive care/lifestyle management: Control of blood pressure, blood glucose, and electrolytes; infection and nutritional management; rehabilitation (recovery of muscle strength), etc.
- Follow-up: Regular imaging (CT/MRI), blood and urine hormone tests, and PET/MIBG when necessary.
Precautions for This Disease
- Surgery-related complications: Bleeding, damage to surrounding organs (liver, spleen, kidneys, colon, blood vessels, etc.), pneumothorax, infection, etc.
- Risks related to hormonal changes: Delayed recovery of the opposite normal adrenal gland after resection of a functioning tumor may cause temporary or persistent adrenal insufficiency (hypocortisolism) and electrolyte imbalance, hypotension, and shock (adrenal crisis) → perioperative steroid replacement is necessary.
- In the presence of systemic metastasis, treatment with local surgery alone is limited → systemic chemotherapy and symptomatic treatment are required.
- Long-term follow-up is necessary (monitoring for recurrence and metastasis).
- Self-management: Take prescribed steroids and anticancer drugs accurately, contact a doctor immediately if symptoms of infection occur, and record changes in blood pressure, blood glucose, weight, and symptoms.
- Complications: Postoperative intestinal adhesions and intestinal obstruction, pneumonia and atelectasis, wound infection, bleeding, etc.
Checklist Before an Outpatient Visit (Including Items to Bring and Example Questions)
- Items to bring (for appointments and tests):
- List of current medications, supplements, and herbal medicines (names and doses)
- Copies of records of previous surgeries and hospitalizations and recent test results (CT/MRI, blood and urine hormone tests, etc.), or information for CD/PACS access
- Notes on changes in symptoms (when they started, factors that worsen or improve them, changes in meals and weight, etc.)
- Blood pressure and blood glucose records (if possible)
- Pre-test check: If you have recently taken steroids, be sure to report it (this can affect test results and the interpretation of endocrine tests).
- Symptoms to tell the doctor about first during an outpatient visit: Dizziness or fainting, severe abdominal pain, severe fatigue, high fever, shortness of breath, rapid weight gain (edema), or weight loss.
- General example questions to ask the doctor during an outpatient visit:
- "What tests and testing frequency are recommended for my current condition?"
- "Is the goal of treatment cure or symptom relief?"
- "What are the main symptoms of side effects that may be expected from the tests or medications?"
- Disease-specific example questions to ask the doctor during an outpatient visit:
- "A genetic predisposition is suspected. Do you recommend genetic counseling or genetic testing?"
- "Is mitotane treatment appropriate in my case? What benefits can be expected from long-term use, and what side effects should I watch for?"
Examples of General Questions to Ask the Doctor
- What side effects are expected from this medication (or treatment), and how can they be managed?
- What will the expected recovery process and rehabilitation plan be after surgery (or chemotherapy)?
- What is my prognosis (chance of cure, risk of recurrence)?
- Are there any particular things I should avoid in daily life, diet, or exercise?
Examples of Disease-Specific Questions
- How will steroid replacement therapy be administered before and after surgery? Can it be stopped suddenly?
- Should we check for an association with hereditary diseases (e.g., Li-Fraumeni, MEN-1, etc.)? Is family testing recommended?
(Note: The diagnostic and treatment plan varies depending on the patient's condition, disease stage, and comorbidities. Please discuss individual decisions with your treating physician.)