The first 60 days: Alveolar soft part sarcoma
Alveolar soft part sarcoma is a rare, slow-growing cancer of young adults driven by a single fusion gene, ASPSCR1-TFE3, that switches on blood vessel growth. Chemotherapy does not work, but drugs that block the tumour's blood supply shrink it, and in 2023 the immunotherapy atezolizumab became the first drug approved for it. Below, week by week, is what OnCo's record of Alveolar soft part 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: Localised, Advanced, first line.
- RadiologistNamed in the standard of care for: Localised.
- SurgeonNamed in the standard of care for: Localised, Oligometastatic.
- Medical oncologistNamed in the standard of care for: Localised, Advanced, first line, Oligometastatic.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised, Oligometastatic.
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.
Wide resection with radiotherapy for close or positive margins; surveillance of lungs and brain.
Atezolizumab (FDA approved 2023) or a VEGFR kinase inhibitor (sunitinib, pazopanib; anlotinib in China); conventional chemotherapy is not used.
Metastasectomy or stereotactic radiotherapy to lung and brain lesions in slowly progressing disease.
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 ASPSCR1-TFE3 fusion, PAS-positive, diastase-resistant crystals, Brain imaging surveillance for metastases), 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 Alveolar soft part sarcoma of the deep soft tissue of the limbs, Head and neckalveolar soft part sarcoma in children, Metastatic disease to lung, bone and brain.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised
- For my situation (localised), which of the standard options do you recommend and why?Guideline options include: Wide resection with radiotherapy for close or positive margins; surveillance of lungs and brain.
Advanced, first line
- For my situation (advanced, first line), which of the standard options do you recommend and why?Guideline options include: Atezolizumab (FDA approved 2023) or a VEGFR kinase inhibitor (sunitinib, pazopanib; anlotinib in China); conventional chemotherapy is not used.
- Am I a candidate for Atezolizumab, Sunitinib, Pazopanib or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of Phase III Trial of Anlotinib, Catequentinib in Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma, Synovial Sarcoma (APROMISS) apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Oligometastatic
- For my situation (oligometastatic), which of the standard options do you recommend and why?Guideline options include: Metastasectomy or stereotactic radiotherapy to lung and brain lesions in slowly progressing disease.
Any stage
- Are there clinical trials I could join, for example of Atezolizumab, Anlotinib, Clinical Trials of Benmelstobart Injection Combined With Anlotinib Hydrochloride Capsules in the Treatment of Advanced or Unresectable Alveolar Soft Part Sarcoma, Phase III Trial of Anlotinib, Catequentinib in Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma, Synovial Sarcoma (APROMISS)?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 “Why a low-mutation fusion sarcoma responds to immunotherapy is not understood”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “Brain metastases are common and hard to treat”. 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.
- Phase III Trial of Anlotinib, Catequentinib in Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma, Synovial Sarcoma (APROMISS)Phase 3 · recruiting · NCT03016819A Phase III Study of AL3818 (Anlotinib, Catequentinib) Hydrochloride Monotherapy in Subjects With Metastatic or Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma and Synovial Sarcoma
- Clinical Trials of Benmelstobart Injection Combined With Anlotinib Hydrochloride Capsules in the Treatment of Advanced or Unresectable Alveolar Soft Part SarcomaPhase 2 · recruiting · NCT07548177To Evaluate the Single-arm, Multi-center Phase II Clinical Trial of Benmelstobart Injection Combined With Anlotinib Hydrochloride Capsules in the Treatment of Advanced or Unresectable Alveolar Soft Part Sarcoma
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Alveolar soft part sarcoma: the full pageAlveolar soft part sarcoma is a rare, slow-growing cancer of young adults driven by a single fusion gene, ASPSCR1-TFE3, that switches on blood vessel growth. Chemotherapy does not work, but drugs that block the tumour's blood supply shrink it, and in 2023 the immunotherapy atezolizumab became the first drug approved for it.
- 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.