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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Uterine sarcomas are rare cancers of the muscle and supporting tissue of the womb, distinct from the far commoner endometrial cancer. Removing the uterus intact is the main treatment and is followed by observation for stage I disease; low-grade stromal sarcomas respond to hormone-blocking pills, while advanced leiomyosarcoma is treated with doxorubicin and trabectedin.
Uterine sarcomas comprise leiomyosarcoma (LMS, the majority), low-grade and high-grade endometrial stromal sarcoma (ESS), undifferentiated uterine sarcoma, adenosarcoma, and a growing set of molecularly defined entities: JAZF1-SUZ12 fusions in low-grade ESS, YWHAE-NUTM2 and BCOR alterations in high-grade ESS, and rare NTRK-, ALK- or COL1A1-PDGFB-rearranged uterine sarcomas that have matched targeted drugs. LMS is often diagnosed after hysterectomy or myomectomy for presumed fibroids; power morcellation of an unsuspected LMS disseminates tumour and worsens outcome, which led the FDA to restrict the practice in 2014.
Treatment of localised disease is total hysterectomy with intact removal; oophorectomy is standard for ESS (hormone-sensitive) but optional in premenopausal LMS. Adjuvant chemotherapy did not improve outcomes in the randomised GOG-0277 trial (gemcitabine-docetaxel followed by doxorubicin versus observation, closed early) and adjuvant radiotherapy did not improve survival in EORTC 55874, so observation is standard after complete resection of stage I LMS. For advanced LMS, doxorubicin-based therapy is first line (GeDDiS showed gemcitabine-docetaxel was not superior to doxorubicin; LMS-04 showed doxorubicin plus trabectedin improved progression-free survival over doxorubicin alone), followed by trabectedin, gemcitabine-docetaxel or pazopanib. Low-grade ESS is treated with aromatase inhibitors or progestins, not chemotherapy, and estrogen must be avoided.
| Setting | Approach | Guideline |
|---|---|---|
| Localised, any subtype | Total hysterectomy with intact removal (no morcellation); bilateral salpingo-oophorectomy for ESS; observation after complete resection of stage I LMS because adjuvant chemotherapy (GOG-0277) and radiotherapy (EORTC 55874) did not improve survival. | NCCN Category 2A |
| Advanced or recurrent leiomyosarcoma | Doxorubicin alone or with trabectedin (LMS-04); gemcitabine-docetaxel; trabectedin, pazopanib or eribulin in later lines. | NCCN Category 2A |
| Low-grade endometrial stromal sarcoma | Aromatase inhibitor (letrozole) or progestin; avoid estrogen and tamoxifen; surgery for resectable recurrence. | not mapped |
| Fusion-driven sarcoma | Larotrectinib or entrectinib for NTRK fusions, crizotinib or alectinib for ALK, imatinib for COL1A1-PDGFB. | not mapped |