Initial Treatment for Stromal Sarcoma
The standard initial treatment for endometrial stromal sarcoma is total abdominal hysterectomy with bilateral salpingo-oophorectomy. 1
Surgical Management Based on Stromal Sarcoma Type
Low-Grade Endometrial Stromal Sarcoma (ESS)
- Total abdominal hysterectomy with bilateral salpingo-oophorectomy is the standard surgical approach, especially due to the hormonal sensitivity of these tumors 1
- Lymphadenectomy is not routinely indicated as lymph node involvement is less than 5% 1
- Patients should not receive post-operative hormone replacement therapy containing estrogens 1
High-Grade ESS and Undifferentiated Endometrial Sarcoma
- Total abdominal hysterectomy is the standard treatment 1
- Bilateral salpingo-oophorectomy may be considered, particularly if there is macroscopic involvement 1
- Lymphadenectomy is not routinely indicated in the absence of macroscopic involvement 1
Adjuvant Therapy Considerations
Radiation Therapy
- Adjuvant pelvic radiotherapy has not been shown to improve survival and is not routinely indicated in FIGO stage I and II disease 1
- May be considered for selected high-risk cases with well-defined areas at risk for local recurrence 1
Hormonal Therapy
- Use of adjuvant estrogen deprivation therapy is not indicated for early-stage disease 1
- For advanced/metastatic ESS, estrogen deprivation therapy with an aromatase inhibitor or progestogen is recommended 1
- Tamoxifen is contraindicated as its action may be pro-estrogenic 1
- Approximately 50% of uterine leiomyosarcomas express estrogen and progesterone receptors, making hormone receptor testing important 2
Chemotherapy
- For high-grade ESS and undifferentiated endometrial sarcoma, systemic treatment parallels that for adult-type soft tissue sarcomas 1
- Doxorubicin-based regimens are considered standard first-line treatment for high-risk patients 3, 4
- For advanced disease, options include doxorubicin/epidoxorubicin plus ifosfamide 4
Special Considerations
Uterine Adenosarcoma
- For early-stage disease, standard surgical treatment is total hysterectomy and bilateral salpingo-oophorectomy 1
- There is no proven role for adjuvant therapy due to lack of evidence 1
- For metastatic disease with predominantly epithelial component, endocrine therapy is an option 1
- For sarcomatous overgrowth (>25% of tumor volume), systemic treatment along a uterine leiomyosarcoma paradigm is advised 1
Follow-up Protocol
- High-grade sarcoma patients should be followed every 3-4 months in the first 2-3 years 2
- Then twice a year up to the fifth year and once a year thereafter 2
- Regular imaging should be performed to detect pulmonary metastases 2
Common Pitfalls and Caveats
- Preoperative differential diagnosis of stromal sarcomas from benign gynecologic diseases is often difficult 5
- Recurrence rates for low-grade ESS can be as high as 25%, despite their generally indolent nature 5
- Hormone replacement therapy containing estrogens is contraindicated in patients with endometrial stromal sarcoma 1
- Treatment decisions should be made in centers with expertise in sarcoma management 3
By following this treatment algorithm, clinicians can provide optimal care for patients with stromal sarcoma, prioritizing interventions that have been shown to improve morbidity, mortality, and quality of life outcomes.