Granulosa cell tumor

Disease Description (Causes and Symptoms)

• Definition and pathophysiology: Granulosa cell tumor (GCT), which originates in the gonad (gonadal stroma) of the ovary, is a tumor that develops in the granulosa cells (follicular epithelial cells) that form ovarian follicles. Because it often produces gonadal hormones, mainly estrogen, hormone-related symptoms may occur. It is classified into adult and juvenile types, and the FOXL2 C134W genetic variant is commonly observed in the adult type (rare in the juvenile type).
• Cause: Although the exact mechanism of development is unknown, genetic mutations (e.g., FOXL2) and abnormalities at the cellular level are involved.
• Main symptoms:
  • Excessive estrogen effects: abnormal uterine bleeding (postmenopausal bleeding), menstrual abnormalities (irregular menstruation or amenorrhea), and possible accompanying endometrial hyperplasia or endometrial cancer
  • In children, precocious puberty (early breast development, pubic hair development, and spontaneous uterine bleeding)
  • Abdominal bloating, a palpable abdominal mass, pain, and ascites (when the tumor is large)
  • Most tumors are unilateral (in most cases, affecting one ovary), while bilateral tumors are rare (approximately 2–8%)

Treatment Methods

• Basic principle: Surgery is the primary treatment
  • Surgical approach according to stage and desire for future pregnancy
    • If fertility preservation is desired and the tumor is confined to one ovary: unilateral salpingo-oophorectomy (selected when preservation is feasible)
    • If the patient no longer desires pregnancy or depending on the extent of disease: total hysterectomy and bilateral salpingo-oophorectomy (removal of the uterus and ovaries)
  • Recommended staging during surgery: peritoneal washing, abdominal inspection (peritoneal and omental biopsy or partial resection), and lymph node evaluation when necessary
• Adjuvant therapy (depending on stage and recurrence)
  • Chemotherapy: Used for advanced disease (when the disease has spread beyond the local area) or recurrent lesions. Commonly used regimens include:
    • Platinum- and taxane-based regimens (e.g., carboplatin + paclitaxel) are frequently used
    • BEP (bleomycin, etoposide, cisplatin) is one option, although practice varies by institution and clinical situation
  • Radiation therapy: Limited role (may be used for some localized recurrences)
  • Hormone therapy: Cases have been reported using aromatase inhibitors (such as anastrozole), progestins, or GnRH agonists for low-volume or recurrent disease (the level of evidence varies)
• Follow-up
  • Granulosa cell tumors may recur late (on average after 5–6 years, and sometimes decades later), so long-term follow-up (for years to decades) is necessary
  • Regular examinations including serum markers (inhibin B, AMH (or MIS), and estradiol) and pelvic ultrasound are recommended
• Lifestyle management and precautions
  • Management of hormonal changes and menopausal symptoms after surgery (consult a specialist if necessary)
  • Prompt evaluation of abnormal bleeding associated with endometrial abnormalities (endometrial examination is recommended)
  • If fertility preservation is desired, consultation with a reproductive medicine specialist before treatment is recommended

Outpatient Visit Checklist (Including Example Questions)

• Items to prepare (recommended to bring to the appointment)
  • A list of all currently used medications (including hormonal medications)
  • Past medical history (especially ovarian or uterine surgery and cancer history) and family history (cancer in parents or siblings)
  • Recent test results (pelvic ultrasound, CT/MRI, blood test results, and tumor marker levels)
  • Menstrual history and bleeding patterns (start and end dates and records of abnormal bleeding)
  • Reproductive-system-related symptoms (breast changes, symptoms of precocious puberty, etc.) and records of pain or a palpable mass
  • Fertility plans (whether pregnancy is desired)
• Examinations and preparation
  • Pregnancy testing when necessary (for those of potentially childbearing age)
  • Bringing recent imaging materials (CD or report) can help with treatment and surgical planning
• Photos/notes to show the doctor during the appointment
  • Photos showing changes in the mass (abdominal enlargement) over time
  • A menstrual cycle chart or notes on abnormal bleeding patterns

General Example Questions to Ask the Doctor

• "What is the stage of this tumor, and how does the treatment plan vary according to the stage?"
• "Can it be cured with surgery? What is the risk of recurrence?"
• "What are the main side effects of surgery and chemotherapy, and how long is the recovery period?"
• "How will treatment affect fertility (pregnancy), and are there alternatives for preserving fertility?"

Examples of Disease-Specific Questions

• "The tumor is confined to one ovary. Is it safe to undergo unilateral salpingo-oophorectomy to preserve fertility? What are the recurrence rate after fertility-sparing surgery and the follow-up testing plan?"
• "My serum inhibin B/AMH levels were high. Can these levels be used to determine treatment or monitor recurrence? At what intervals and with which tests should I be followed?"

(If necessary, consult a gynecologic oncologist to establish a treatment and follow-up plan tailored to your individual circumstances.)