It is recommended that Uterine sarcomas be discussed at a tumour board with pathology review.
Diagnosis of uterine sarcoma is made at the time of uterine biopsy:
- Operable cases: A total abdominal hysterectomy +/- bilateral salpingo-oophorectomy is recommended for all histological subtypes. Lymphnode metastatses are uncommon in sarcomas. When disease is outside of the uterus surgical staging and cytoreduction can be performed provided that the surgeon believes the disease to be resectable.
- Non surgical candidates: Can be offered systemic therapy +/- radiation therapy for local control.
Diagnosis of uterine sarcoma is made after hysterectomy
- Patients with uterine sarcomas should be referred post diagnosis to BC Cancer for staging and an opinion regarding adjuvant treatment.
- Further surgical intervention is determined based on the histology, stage and type of primary surgery.
Pelvic and abdominal recurrence develops in one-third to one-half of patients. The interval to recurrence can vary with a mean of three years. Distant metastases are uncommon.
- Stage I: surveillance vs. endocrine therapy. No role for radiation therapy.
- Stage II/IV: endocrine therapy +/- radiation therapy.
- Data to support administration of adjuvant endocrine therapy for stage II to IV ESS are limited. The decision to pursue adjuvant systemic treatment, drug choice, and duration of treatment is case dependent.
- Stage I: observation vs adjuvant chemo
- Stage II/III resected to zero residual disease: consideration for adjuvant chemo+/- radiation therapy
- The role for adjuvant systemic therapy for adenosarcoma, leiomyosarcoma, HGESS, or UUS is controversial and should not be considered standard of care, given there is no overall survival benefit reflected in prospective adjuvant trials.
- Stage III/IV suboptimally debulked, or nonoperable
- Systemic therapy should be considered. Treatment choice and duration of treatment is case dependent.
There is a limited role for surgical resection.
Radiation therapy, outside of oligometastic, disease is reserved for symptomatic disease. Mainstay of treatment is systemic therapy.
- Low grade stromal sarcoma
- For ER/PR positive disease, hormonal therapy should be considered. Tamoxifen should be avoided as there is some evidence to suggest increased risk of recurrence with use.
- Gemcitabine/docetaxel or doxorubin are reasonable choices for patients with good functional status.
- Aromatase inhibitors have response rates <10%. However, for some patients with ER/PR positive disease, low disease burden or indolent pace, or poor functional status, this strategy may be employed.
- High grade/ undifferentiated stromal sarcoma and adenosarcomas
- Should be treated like Leiomyosarcoma