Parathyroid cancer

Disease Description (Causes and Symptoms)

• Definition and pathophysiology: A malignant tumor arising from the adrenal cortex (adrenocortical carcinoma, adrenal cancer) may invade blood vessels and lymphatic vessels, causing local invasion and distant metastasis. Some cases are associated with genetic syndromes (e.g., Li‑Fraumeni, Beckwith‑Wiedemann, MEN1, FAP).
• Causes/risk factors: Most cases occur sporadically, but some are associated with genetic predisposition. Smoking, a high-fat diet, lack of physical activity, and exposure to carcinogens may increase the risk.
• Main symptoms:
  • Hormone-secreting type (approximately 40–60%): Excessive corticosteroid secretion may cause Cushing syndrome (moon face, central obesity, proximal muscle wasting, hypertension, diabetes, etc.); sex hormone secretion may cause amenorrhea, hirsutism, or virilization in women, or sexual dysfunction or feminization in men.
  • Nonfunctioning type: Often discovered incidentally without symptoms in the early stages.
  • Mass effect/metastasis: Abdominal discomfort, bloating, pain, palpable lymph nodes, and symptoms involving the affected organs due to metastasis to the liver, lungs, or bones (jaundice, shortness of breath, bone pain, etc.).
• Tests used for diagnosis (summary): Abdominal CT/MRI (initial imaging), hormone tests (blood/24-hour urine), PET/MIBG/bone scan (assessment of metastasis), and biopsy and pathology (definitive diagnosis at surgery).

Treatment Methods and Precautions

• Localized lesions (when complete resection is possible): Complete surgical resection (R0) is the only curative treatment. Depending on tumor size and degree of invasion, open surgery (recommended for large or invasive tumors) or selected laparoscopic/robotic surgery may be performed. Extensive resection together with adjacent organs (kidney, liver, spleen, etc.) may be required (en bloc resection).
• Adjuvant/systemic treatment:
  • Mitotane: An adrenotoxic drug that is widely used as adjuvant therapy (after surgery in high-risk patients), for local recurrence, or for metastatic disease. Blood levels must be monitored and toxicity managed during treatment.
  • Chemotherapy: Combination regimens such as EDP‑M (etoposide, doxorubicin, cisplatin + mitotane) are recommended for systemic progressive disease or unresectable cases.
  • Radiation therapy: May be used as an adjuvant or palliative treatment (for local pain or suppression of local recurrence) rather than with curative intent.
• Hormone-related treatment and management: When excessive hormone symptoms are present (such as Cushing syndrome), endocrine control is necessary. After surgery, temporary or persistent adrenal insufficiency may occur due to reduced function of the remaining adrenal gland, requiring steroid replacement therapy (including continued therapy and perioperative stress doses) and monitoring of electrolytes and blood glucose.
• Complications and precautions:
  • Surgery-related: Bleeding, injury to adjacent organs (liver, spleen, kidney, colon, inferior vena cava, etc.), infection, and pulmonary complications. In the event of major bleeding during laparoscopic surgery, conversion to open surgery may be necessary.
  • Drug-related: Mitotane may cause gastrointestinal disturbances, neurotoxicity, and skin/endocrine abnormalities (thyroid and sex hormone changes); as a strong CYP inducer, it has many drug interactions.
  • Systemic complications: Adrenal insufficiency, organ failure due to metastasis, and postoperative aspiration pneumonia, intestinal obstruction, etc.
• Lifestyle management: Management of blood pressure and blood glucose, infection prevention, smoking cessation, a healthy diet, and regular exercise are recommended. Contraception is recommended while taking mitotane (due to possible effects on fertility).

Outpatient Visit Checklist (Items to Bring and Sample Questions)

• Items/materials to bring:
  • A list of all medications and supplements currently being taken (including dosage and administration times)
  • Previous test results (CT/MRI, ultrasound, blood and urine hormone test results) or examination CDs/image files
  • A chronological record of recent symptom changes (weight changes, blood pressure and blood glucose levels, menstrual or sexual function changes, etc.)
  • Family history (particularly cancers occurring at a young age, brain tumors, endocrine tumors, or other conditions related to hereditary syndromes)
  • Records of blood pressure, weight, and blood glucose levels (if possible, from the past several weeks)
• Items patients may find useful to write down at the outpatient visit: Whether surgery or anticancer treatment is desired, pregnancy plans, records of drug allergies, and limitations in daily life

Examples of General Questions to Ask the Doctor

• What is the goal of treatment for this disease (treatment, cure, or palliation)?
• Can my lesion be completely removed by surgery? Which method do you recommend, open surgery or laparoscopic surgery?
• Is mitotane treatment necessary? What are its side effects, and how can they be prevented and managed?
• Do I need steroid supplementation before or after surgery? (When and how much?)
• What is the schedule for follow-up tests (imaging and hormone tests)? What factors affect the prognosis (risk of recurrence)?

Examples of Disease-Specific Questions

• What blood and urine tests should I undergo to determine whether my tumor secretes hormones (whether it is functioning)? (For example, 24-hour urine cortisol, serum ACTH, androgen/estrogen levels, etc.)
• Is adjuvant mitotane treatment recommended after surgical resection? If so, what is the expected treatment duration and plan for managing the target blood concentration?

(Test and treatment plans vary depending on the individual’s condition. Consult your treating medical team regarding the final treatment plan and decisions related to medications and surgery.)