What is the treatment for adenosarcoma of the uterus with sarcomatous overgrowth?

Treatment of Adenosarcoma of the Uterus with Sarcomatous Overgrowth

For uterine adenosarcoma with sarcomatous overgrowth, the standard treatment is total hysterectomy with bilateral salpingo-oophorectomy followed by systemic treatment along a uterine leiomyosarcoma paradigm, with some evidence supporting the use of trabectedin. 1

Surgical Management

• Total abdominal hysterectomy (TAH) with bilateral salpingo-oophorectomy (BSO) is the standard surgical approach for patients with uterine adenosarcoma with sarcomatous overgrowth 1
• Routine lymphadenectomy is not indicated as lymph node involvement is less than 5% in uterine sarcomas 1
• Complete surgical debulking should be attempted for optimal outcomes 2
• Surgery should be performed with the goal of achieving negative margins, as this is a significant prognostic factor 3

Prognostic Factors

• Sarcomatous overgrowth (defined as >25% of tumor volume) is a major poor prognostic factor 1, 3
• Myometrial invasion is another significant poor prognostic indicator 3
• Patients with sarcomatous overgrowth have significantly worse outcomes, with 2-year progression-free and overall survival rates of only 20% compared to 100% for adenosarcoma without sarcomatous overgrowth 4
• The median survival for patients with adenosarcoma with sarcomatous overgrowth has been reported as approximately 13 months 2

Adjuvant Therapy

• For patients with sarcomatous overgrowth (>25% of tumor volume), systemic treatment following a uterine leiomyosarcoma paradigm is recommended 1
• There is some evidence supporting the use of trabectedin in these cases 1
• Common chemotherapy regimens include:
  • Ifosfamide and liposomal doxorubicin 5
  • Gemcitabine-docetaxel followed by doxorubicin 1
• Adjuvant pelvic radiotherapy has not been shown to improve survival and is not routinely indicated in FIGO stage I and II disease 1
• However, radiotherapy could be considered for selected high-risk cases, particularly those with sarcomatous overgrowth 1

Management of Advanced/Metastatic Disease

• For metastatic disease, systemic chemotherapy is the mainstay of treatment 6
• If the tumor has a predominantly epithelial component, endocrine therapy may be considered 1
• For tumors with sarcomatous overgrowth, treatment should follow protocols used for high-grade uterine sarcomas 1, 6
• Consider surgical resection of isolated metastases when feasible 6

Follow-up Recommendations

• High-grade sarcoma patients should be followed every 3-4 months in the first 2-3 years 6
• Follow-up should then continue twice a year up to the fifth year and once a year thereafter 6
• Regular chest imaging is recommended to detect pulmonary metastases, which are common in high-grade sarcomas 6

Special Considerations

• Due to the rarity and aggressive nature of this disease, treatment at centers with expertise in sarcoma management is strongly recommended 7, 6
• Early diagnosis and surgical intervention are critical, as delayed diagnosis can lead to more advanced disease and poorer outcomes 5, 8
• The aggressive nature of adenosarcoma with sarcomatous overgrowth should not be underestimated, as it behaves more like a high-grade sarcoma than typical adenosarcoma 2, 3