Adrenal cancer

Disease Description (Causes and Symptoms)

• Definition and pathophysiology: Adrenal cancer (primarily adrenocortical carcinoma) is a malignant tumor arising in the adrenal gland. The tumor may invade blood vessels and the capsule and metastasize to surrounding or distant organs. Some tumors secrete excessive hormones (functional), while others do not secrete hormones (nonfunctional).
• Causes and risk factors:
  • Most cases are sporadic (not hereditary). In children and some adults, it may be associated with hereditary syndromes, including Li-Fraumeni, Beckwith-Wiedemann, MEN-1, and familial adenomatous polyposis (FAP).
  • Lifestyle and environmental factors: A high-fat diet, smoking, insufficient physical activity, and exposure to carcinogens may increase risk.
• Main symptoms:
  • Functional tumors (approximately 60%): Symptoms vary depending on the hormone secreted in excess.
    • Excessive glucocorticoid secretion → Cushingoid features (round face, central obesity, abdominal fat, skin acne and acneiform changes, muscle atrophy, hypertension, diabetes, etc.)
    • Androgen excess (especially in women) → hirsutism, menstrual irregularities, sexual dysfunction, and masculinizing changes
    • Excess aldosterone → hypokalemic hypertension (when there is excessive secretion)
  • Nonfunctional tumors (approximately 40%): They are often asymptomatic initially and discovered incidentally during health checkups. As the tumor enlarges, abdominal discomfort, abdominal distension, pain, loss of appetite, weight loss, generalized weakness, and anemia may occur. If metastasis occurs, symptoms specific to the affected organs (such as the liver or lungs) may develop.
  • Common sites of metastasis: liver, lungs, bones, peritoneum, and lymph nodes

Treatment Methods (Standard Treatment, Medications, Procedures, Lifestyle Management, and Precautions)

• Important points before and during diagnosis:
  • Functional status (hormone secretion) must be assessed before surgery: tests may include cortisol (e.g., 1 mg overnight dexamethasone suppression test or 24-hour urine cortisol), electrolytes, aldosterone/renin ratio, and catecholamines (plasma/urine metanephrines)—pheochromocytoma must be ruled out in particular to prevent acute complications during surgery.
  • Imaging tests: abdominal CT (the primary test), MRI, PET, MIBG scan (when necessary), and bone scan (to assess bone metastasis)
  • Biopsy: Percutaneous needle biopsy is generally not recommended (exceptions include cases in which a preoperative definitive diagnosis is necessary or differentiation of a primary cancer from metastasis of another organ is needed).
• Localized disease (resectable):
  • Complete surgical resection (radical adrenalectomy) is the primary treatment. Depending on tumor size and extent of invasion, open surgery (generally for large or invasive tumors) or laparoscopic surgery (for small, noninvasive tumors) may be selected.
  • Extensive resection including surrounding organs (kidney, liver, spleen, colon, etc.) or lymph nodes may be necessary.
  • If the opposite adrenal gland does not immediately recover function because of suppression of hormonal activity after surgery, temporary or long-term glucocorticoid replacement therapy may be necessary.
• Unresectable locally advanced or metastatic disease:
  • Systemic chemotherapy: Mitotane is a drug used specifically for adrenocortical carcinoma. It is used alone or in combination (e.g., EDP: etoposide, doxorubicin, cisplatin + mitotane) as standard therapy.
  • Radiation therapy: Used as adjuvant or palliative treatment to relieve symptoms such as recurrence-related pain, local pain, or pain from bone metastases.
  • Targeted therapy/immunotherapy: Currently in the research and clinical trial stages. Clinical trials should be considered when appropriate.
• Lifestyle management and precautions:
  • Management of blood pressure, blood glucose, and electrolytes (especially low potassium) is important in hormone-secreting tumors.
  • Discuss the perioperative steroid management plan (replacement therapy and dose adjustment) with the treating medical team in advance.
  • Mitotane may cause fatigue, gastrointestinal symptoms, neurotoxicity, changes in liver enzyme levels, and changes in thyroid function and blood lipids, so regular monitoring is necessary.
  • Risk of surgical complications: bleeding, injury to surrounding organs (liver, spleen, kidney, colon, inferior vena cava, etc.), infection, intestinal adhesions and bowel obstruction, and pneumothorax.
  • Long-term follow-up is necessary because recurrence and metastasis are frequent.

Checklist Before an Outpatient Visit (Including Items to Bring and Example Questions)

• Items to bring (bringing these can facilitate the consultation):
  • List of medications currently being taken (including prescriptions, OTC medications, and supplements)
  • Previous test results and imaging (CT/MRI reports and, if possible, image files on a CD/USB)
  • Previous surgeries, medical history, and family history (especially family history related to hereditary cancer syndromes involving the brain, breast, pancreas, or parathyroid glands)
  • Records of symptom changes (when symptoms began and how they have progressed, weight changes, blood pressure and blood glucose levels, menstrual changes, etc.)
  • Information about allergies (including medications and anesthesia)
• Useful information to provide to the doctor during the outpatient visit:
  • Results of recent hormone-related tests (24-hour urine cortisol, blood metanephrines, etc.)
  • Dates of recent imaging studies and whether any changes were observed
  • Changes in exercise and dietary patterns, and smoking and alcohol use

General Example Questions to Ask the Doctor

• What is the stage and prognosis (survival rate) of this disease? What is the goal of treatment (curative vs. palliative)?
• If surgery is possible, which method is recommended—open surgery or laparoscopic surgery? Why?
• What are the expected benefits and major side effects of anticancer drugs such as mitotane? How will testing and monitoring be performed?
• Will steroid (adrenal hormone) replacement be necessary after surgery or during treatment? If so, for how long?
• What follow-up tests (interval and type) do you recommend to reduce the risk of recurrence or metastasis?

Examples of Disease-Specific Questions

• Considering my medical history (or family history), do I need testing for hereditary cancer syndromes (Li-Fraumeni, MEN1, etc.)? If genetic testing is recommended, which tests should I undergo and when?
• If my current tumor is functional (hormone-secreting), how will steroids (or other hormones) be managed before and after surgery? What emergency measures are needed in an emergency (vomiting, fever, low blood pressure, etc.)?

Note: Specific treatment decisions, medication administration, and monitoring plans are determined by the treating doctor after considering the patient's overall clinical situation, including tumor size, stage, hormone secretion status, and comorbidities. Please consult your doctor about any additional tests that may be needed and whether genetic counseling is appropriate.