The first 60 days: Uterine sarcoma
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. Below, week by week, is what OnCo's record of Uterine sarcoma says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- PathologistNamed in the standard of care for: Fusion-driven sarcoma.
- SurgeonNamed in the standard of care for: Localised, any subtype, Low-grade endometrial stromal sarcoma.
- Medical oncologistNamed in the standard of care for: Localised, any subtype, Advanced or recurrent leiomyosarcoma, Low-grade endometrial stromal sarcoma, Fusion-driven sarcoma.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised, any subtype.
- Palliative and supportive care teamNamed in the standard of care for: Advanced or recurrent leiomyosarcoma.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
- 1.Localised, any subtypeNCCN category Category 2A, NCCN Uterine Neoplasms; ESGO-EURACAN-GCIG guideline
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.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Histological subtype and mitotic count, Estrogen and progesterone receptor status, Fusion testing: JAZF1, YWHAE, BCOR, NTRK, ALK, Homologous recombination deficiency and BRCA alterations, Stage and whether the tumour was morcellated), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Uterine leiomyosarcoma, Low-grade endometrial stromal sarcoma, High-grade endometrial stromal sarcoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised, any subtype
- For my situation (localised, any subtype), which of the standard options do you recommend and why?Guideline options include: 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.
Advanced or recurrent leiomyosarcoma
- For my situation (advanced or recurrent leiomyosarcoma), which of the standard options do you recommend and why?Guideline options include: Doxorubicin alone or with trabectedin (LMS-04); gemcitabine-docetaxel; trabectedin, pazopanib or eribulin in later lines.
- Am I a candidate for Doxorubicin, Trabectedin, Gemcitabine or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Low-grade endometrial stromal sarcoma
- For my situation (low-grade endometrial stromal sarcoma), which of the standard options do you recommend and why?Guideline options include: Aromatase inhibitor (letrozole) or progestin; avoid estrogen and tamoxifen; surgery for resectable recurrence.
- Am I a candidate for Letrozole (and other aromatase inhibitors), Progestins (megestrol acetate, medroxyprogesterone, levonorgestrel IUD), and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Fusion-driven sarcoma
- For my situation (fusion-driven sarcoma), which of the standard options do you recommend and why?Guideline options include: Larotrectinib or entrectinib for NTRK fusions, crizotinib or alectinib for ALK, imatinib for COL1A1-PDGFB.
- Am I a candidate for Larotrectinib, Entrectinib, Crizotinib or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Any stage
- Are there clinical trials I could join, for example of Trabectedin, Larotrectinib, Pazopanib?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “Preoperative distinction of leiomyosarcoma from fibroids: MRI and LDH criteria and imaging AI are under study”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Advanced LMS remains chemoresistant after two lines: PARP inhibitors for the HRD subset and antibody-drug conjugates are in trials”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- Study of Lurbinectedin in Combination With Doxorubicin Versus Doxorubicin Alone as First-line Treatment in Participants With Metastatic Leiomyosarcoma (SaLuDo)Phase 3 · active · NCT06088290Randomized, Controlled, Open-label, Phase III Study of Lurbinectedin in Combination With Doxorubicin Versus Doxorubicin Alone as First-line Treatment in Patients With Metastatic Leiomyosarcoma
- A Study to Evaluate Tabelecleucel in Participants With Epstein Barr Virus (EBV) Associated DiseasesPhase 2 · active · NCT04554914An Open-label, Single-arm, Multicohort, Phase 2 Study to Assess the Efficacy and Safety of Tabelecleucel in Subjects With Epstein Barr Virus Associated Diseases (EBVision)
- MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue SarcomaPhase 2 · recruiting · NCT06277154A Phase II Study Evaluating the Safety and Efficacy of MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment in Patients With Advanced Soft Tissue Sarcoma
- STC-15 as a Part of Combination Therapy With Toripalimab in Selected Advanced Cancers and as Monotherapy in Participants With Selected SarcomasPhase 1/2 · recruiting · NCT06975293Open-label, Non-randomized, Multi-cohort, Phase 1b/2 Trial Investigating the Safety, Tolerability, and Antitumor Activity of STC-15 (a METTL3 Inhibitor) as a Part of Combination Therapy With Toripalimab in Participants With Selected Advanced Cancers and as Monotherapy in Participants With Selected Sarcomas
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Uterine sarcoma: the full pageUterine 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.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.
- Hysterectomy: Removing the uterus (womb), often with the cervix, tubes and ovaries.
- Rare cancers: Rare cancers are those with fewer than about 6 new cases per 100,000 people per year.
Every term links to the glossary.