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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.
The frontier is molecular: fusion-directed therapy for NTRK and ALK cases, and trials of PARP inhibitors and immunotherapy in the subset of LMS with homologous recombination deficiency or high mutation burden.
About 3 to 4 percent of uterine cancers; leiomyosarcoma is the most common type, typically diagnosed in women in their fifties, often unexpectedly after surgery for presumed fibroids.
Most high-grade ovarian cancers begin at the tip of the fallopian tube; endometrial cancer lines the uterus, cervical cancer starts at the transformation zone; each drains to a different node group.
Same organ: Adenosquamous carcinoma of the cervix, Small cell neuroendocrine carcinoma of the cervix, Bartholin gland carcinoma, Vulvar melanoma, Vaginal melanoma, High-grade serous ovarian cancer, Low-grade serous ovarian cancer, Clear cell ovarian cancer, Mucinous ovarian cancer, Adult granulosa cell tumour of the ovary, Ovarian cancer, Endometrial cancer, Cervical cancer, Vulvar cancer, Gestational trophoblastic neoplasia, Vaginal cancer, POLE-ultramutated endometrial cancer, Mismatch-repair-deficient endometrial cancer, p53-abnormal endometrial cancer, including uterine serous carcinoma, Endometrial cancer with no specific molecular profile, Advanced or recurrent endometrial cancer, Uterine carcinosarcoma, Early cervical cancer and fertility-sparing surgery, Locally advanced cervical cancer, Recurrent or metastatic cervical cancer, Platinum-sensitive ovarian cancer, Platinum-resistant ovarian cancer, HPV-associated vulvar squamous cell carcinoma, HPV-independent vulvar squamous cell carcinoma (p53-mutant), Vaginal squamous cell carcinoma (HPV-associated), Vaginal adenocarcinoma (including DES-associated clear cell adenocarcinoma), Low-risk gestational trophoblastic neoplasia (FIGO score 0 to 6), High-risk gestational trophoblastic neoplasia (FIGO score 7 or more, including ultra-high-risk), Placental-site trophoblastic tumour and epithelioid trophoblastic tumour
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
Doxorubicin alone or with trabectedin (LMS-04); gemcitabine-docetaxel; trabectedin, pazopanib or eribulin in later lines.
Aromatase inhibitor (letrozole) or progestin; avoid estrogen and tamoxifen; surgery for resectable recurrence.
Larotrectinib or entrectinib for NTRK fusions, crizotinib or alectinib for ALK, imatinib for COL1A1-PDGFB.
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Query for this cancer: (TITLE:"Uterine sarcoma" OR ABSTRACT:"Uterine sarcoma" OR TITLE:"Leiomyosarcoma of the uterus" OR ABSTRACT:"Leiomyosarcoma of the uterus" OR TITLE:"Endometrial stromal sarcoma" OR ABSTRACT:"Endometrial stromal sarcoma" OR TITLE:"Undifferentiated uterine sarcoma" OR ABSTRACT:"Undifferentiated uterine sarcoma" OR TITLE:"Uterine LMS" OR ABSTRACT:"Uterine LMS" OR TITLE:"ESS" OR ABSTRACT:"ESS") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Uterine sarcoma, not a curated reading list.
Lee and colleagues; earlier Koontz 2001 for JAZF1.
Unsuspected uterine sarcoma disseminated by morcellation during fibroid surgery.
Seddon and colleagues, Lancet Oncol; doxorubicin retained as first line.
Adjuvant chemotherapy for stage I LMS did not improve outcomes; observation is standard.
Pautier and colleagues, Lancet Oncol.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
A swollen painful calf, or sudden breathlessness with chest pain; the tamoxifen boxed warning covers pulmonary embolism and stroke.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
QT: both Entrectinib and Crizotinib prolong the QT interval (known and known risk).. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
QT: both Pazopanib and Crizotinib prolong the QT interval (known and known risk).. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
See all on the product pages:CrizotinibDocetaxelDoxorubicinEntrectinibEribulinGemcitabineImatinibLetrozole (and other aromatase inhibitors)PazopanibProgestins (megestrol acetate, medroxyprogesterone, levonorgestrel IUD)Trabectedin·Printable cards in the navigator
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