Granuloma inguinale

Disease Description (Causes and Symptoms)

• Definition and pathophysiology: Uterine sarcoma is a rare group of malignant tumors originating from the muscles of the uterus (smooth muscle) or connective tissue (stroma). Major subtypes include uterine leiomyosarcoma, endometrial stromal sarcoma (ESS: low-grade and high-grade subtypes), carcinosarcoma (mixed type), and undifferentiated sarcoma. Growth rate, hormone receptor status, and patterns of metastasis differ according to the subtype.
• Causes and risk factors: The exact disease mechanism is unclear. Reported associated factors include older age, obesity, nulliparity (having never given birth), and a history of prior pelvic radiation. Rarely, it may be associated with hormone sensitivity (ESS) or genetic abnormalities.
• Main symptoms:
  • Abnormal vaginal bleeding (including postmenopausal bleeding)
  • Pelvic pressure or a feeling of a mass in the lower abdomen
  • Pelvic or abdominal pain
  • Rapid enlargement of the uterus before or after menopause (distinction from fibroids is necessary)
• Test findings: CA-125 may be elevated on blood testing (nonspecific). Imaging (pelvic ultrasound and MRI) may show an intrauterine or myometrial mass, and definitive diagnosis is made by tissue examination (endometrial curettage or pathology after resection).

Treatment Methods

• Surgical treatment
  • Standard: Total hysterectomy is the basic treatment. Bilateral salpingo-oophorectomy is generally considered (determined according to age and subtype), while pelvic lymph node dissection is performed selectively depending on the subtype and clinical presentation.
  • In advanced-stage disease or when there is a large mass, resection or partial resection to reduce the tumor size may be considered, but complete radical resection takes priority.
  • Caution: If the tumor is mistaken for a uterine fibroid, electrical or mechanical morcellation should be avoided because it may cause dissemination of a potentially malignant tumor within the abdominal cavity.
• Adjuvant systemic therapy and radiation
  • Radiation therapy: Pelvic radiation therapy may be used to reduce the risk of local recurrence, but its effect on overall survival varies by subtype and stage.
  • Chemotherapy: Used in metastatic or high-risk disease. Traditionally, doxorubicin, ifosfamide, and the gemcitabine + docetaxel combination are used. Response rates and recommendations differ by subtype.
  • Hormone therapy: In low-grade ESS, when estrogen and progesterone receptors are positive, patients may respond to progestins (such as medroxyprogesterone) or aromatase inhibitors (in postmenopausal patients).
  • Targeted therapy/immunotherapy: In advanced or recurrent disease, some targeted therapies such as pazopanib or investigational drugs in clinical trials may be considered.
• Factors in treatment decisions: Treatment is determined through a multidisciplinary approach, taking into account the stage (localized vs. metastatic), histologic subtype, the patient’s age, menopausal status, desire for pregnancy, and general condition.
• Lifestyle management and precautions
  • Bleeding and pain control, nutritional and weight management, infection prevention (after surgery), and monitoring for side effects during chemotherapy (such as vomiting and bone marrow suppression) are necessary.
  • Control of smoking and alcohol use before and after surgery and optimization of chronic conditions (diabetes and hypertension) are recommended.

Checklist Before an Outpatient Visit (Including Preparations and Example Questions)

• Items to bring to the visit
  • A list of current medications (including prescription drugs, over-the-counter medicines, and supplements)
  • Past medical history and surgical records (especially any uterine surgery or radiation therapy)
  • Copies or CDs of previous test results (pelvic ultrasound, MRI, CT, blood tests, pathology reports, etc.)
  • Symptom records: bleeding (onset date, pattern, frequency), pain (location, severity, changes), weight, shortness of breath, etc.
• Important information to provide to the doctor during the visit
  • Recent menstrual cycles and changes, and whether menopause has occurred
  • Whether pregnancy is desired (the treatment plan differs if fertility preservation is desired)
  • Family history (cancer and hereditary diseases)
• Pre-visit checklist (brief)
  • Record the amount and duration of bleeding
  • Keep a pain diary (pain severity and triggers)
  • Recent weight changes, shortness of breath, and cough (if pulmonary metastasis is suspected)
  • Check whether previous pathology slides can be reviewed again

Examples of General Questions to Ask the Doctor

• "What is the histologic subtype of my lesion (for example, leiomyosarcoma or endometrial stromal sarcoma)?"
• "What is the stage, and what is the prognosis?"
• "What standard treatment do you recommend for me, and what side effects can be expected?"
• "Can my ovaries be preserved during surgery? (Regarding fertility preservation)"
• "What follow-up tests should I have to prevent recurrence and metastasis, when should they begin, and how often should they be performed?"

Examples of Disease-Specific Questions

• Regarding uterine leiomyosarcoma:
  • "How is the risk of morcellation evaluated when I was diagnosed with a fibroid during or before surgery? How is the surgical method determined?"
• Regarding low-grade endometrial stromal sarcoma (low-grade ESS):
  • "What are the results of the hormone receptor tests (ER/PR)? Is hormone therapy (a progestin or aromatase inhibitor) possible?"
• Regarding metastatic or recurrent disease:
  • "Could you explain the specific chemotherapy or targeted therapy options and whether participation in a clinical trial is possible?"

Additional Notes

• Diagnostic approach: Pelvic ultrasound and MRI are used to evaluate the characteristics of the mass, while CT is used to assess metastasis (especially to the lungs). Because diagnosis may be difficult with endometrial curettage or tissue biopsy, final confirmation is often made through postoperative pathology.
• Prognosis summary (summary of clinical evidence): Even when the stage is low, the recurrence rate is high (clinical data report recurrence in approximately 50% of stage I cases), and recurrence and metastasis rates are higher in advanced stages (increased recurrence rates in stages II–IV). The most common sites of metastasis are the abdominal cavity (peritoneum) and lungs.

(The information above summarizes general information based on the latest recommendations and clinical data. Please consult your treating medical team regarding individual care.)