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.
Overview
Alveolar soft part sarcoma is defined by the unbalanced translocation der(17)t(X;17) that fuses ASPSCR1 to the transcription factor TFE3, driving a programme of angiogenesis and metabolic genes; the same TFE3 fusion partner occurs in translocation renal cell carcinoma. The tumour is richly vascular, grows slowly in deep muscle of the limbs of young adults or in the orbit and tongue of children, and metastasises early to the lungs, bone and, unusually for a sarcoma, the brain. Because it is indolent, patients with metastases often survive many years, and the disease is followed with regular brain imaging.
Localised tumours are treated with wide resection, with radiotherapy for close margins, and isolated metastases with surgery or stereotactic radiotherapy. Conventional chemotherapy is ineffective. VEGFR-directed kinase inhibitors are active: sunitinib produced responses in Italian series, cediranib improved progression-free survival against placebo in the randomised CASPS trial, pazopanib is used in Europe, and anlotinib, approved in China for soft tissue sarcoma, showed benefit in the alveolar soft part sarcoma cohort of the APROMISS phase 3 trial.
Despite a low mutational burden, the tumour responds to PD-L1 blockade: the NCI-sponsored phase 2 study of atezolizumab produced durable objective responses in a substantial minority of patients, leading to the first FDA approval for any drug in this disease in December 2023 for unresectable or metastatic alveolar soft part sarcoma in patients aged two and over. Combinations of checkpoint inhibitors with anti-angiogenic drugs, including benmelstobart plus anlotinib, are the main line of current trials.
State of the art
- Atezolizumab is the first approved therapy, with durable responses despite a low mutational burden.
- Anti-angiogenic kinase inhibitors remain active alternatives and partners for immunotherapy.
Show survival figures (1)
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
- The slow course means long survival with metastases and a need for decades of surveillance.
Red cards
From the labels and guidelines behind the standard of care. Your team's thresholds win.- Emergency services nowBowel perforation
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
- Emergency services nowFainting or palpitations
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
- Emergency services nowHypophysitis or adrenal crisis
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
- Check before combiningSunitinib with Pazopanib: major interaction
QT: both Sunitinib and Pazopanib 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.
- Check before combiningFood and drink: Pazopanib
Take on an empty stomach (1 hour before or 2 hours after food).
- Check before combiningFood and drink: Sunitinib
Avoid grapefruit.
See all on the product pages:AnlotinibAtezolizumabPazopanibSunitinib·Printable cards in the navigator
Anatomy and lymph node drainage
- Metaphysis, near the growth plate (osteosarcoma)
- Shaft (Ewing sarcoma)
- Deep soft tissue compartment
- Skeletal muscle (rhabdomyosarcoma)
- Neurovascular bundle (limb salvage decision)
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
- Metaphysis, near the growth plate (osteosarcoma)
- Shaft (Ewing sarcoma)Metastatic disease to lung, bone and brain (indolent course)
- Deep soft tissue compartmentAlveolar soft part sarcoma of the deep soft tissue of the limbs (adolescents and young adults) · Head and neck (orbit, tongue) alveolar soft part sarcoma in children · Metastatic disease to lung, bone and brain (indolent course)
- Skeletal muscle (rhabdomyosarcoma)Alveolar soft part sarcoma of the deep soft tissue of the limbs (adolescents and young adults) · Head and neck (orbit, tongue) alveolar soft part sarcoma in children
- Neurovascular bundle (limb salvage decision)
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Retroperitoneal sarcoma, Soft tissue sarcoma of the extremity (localised and advanced)
Under one percent of soft tissue sarcomas, arising in adolescents and young adults, usually in the thigh or buttock and in children in the head and neck; it grows slowly but has often already spread to the lungs, bone or brain at diagnosis, and patients live for years with metastatic disease.
- MRIStandard of care
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
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.
Subtypes & biomarkers
top- Alveolar soft part sarcoma of the deep soft tissue of the limbs (adolescents and young adults)
- Head and neck (orbit, tongue) alveolar soft part sarcoma in children
- Metastatic disease to lung, bone and brain (indolent course)
- ASPSCR1-TFE3 fusion (FISH or RNA sequencing; TFE3 immunohistochemistry)
- PAS-positive, diastase-resistant crystals
- Brain imaging surveillance for metastases
How often this target appears
- 1952Christopherson, Foote and Stewart name alveolar soft part sarcoma
- 2001ASPSCR1-TFE3 fusion identified as the defining alteration
- 2009Sunitinib shows activity in alveolar soft part sarcoma
- 2019CASPS: cediranib improves progression-free survival against placebo
- 2023Atezolizumab approved by the FDA, the first drug for the disease
Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 6 changes by month →- 2026-09-17This recordAlveolar soft part sarcomaFacts on this page last checked
When this page itself was last checked or edited.
- 2023MilestoneAtezolizumabAtezolizumab approved by the FDA, the first drug for the disease
A milestone in how this cancer is treated.
- 2019MilestoneAlveolar soft part sarcomaCASPS: cediranib improves progression-free survival against placebo
A milestone in how this cancer is treated.
- 2009MilestoneSunitinibSunitinib shows activity in alveolar soft part sarcoma
A milestone in how this cancer is treated.
- 2001MilestoneAlveolar soft part sarcomaASPSCR1-TFE3 fusion identified as the defining alteration
A milestone in how this cancer is treated.
- 1952MilestoneAlveolar soft part sarcomaChristopherson, Foote and Stewart name alveolar soft part sarcoma
A milestone in how this cancer is treated.
What is in development for Alveolar soft part sarcoma, drawn from the whole corpus: 2 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Trials under way · 2
- Clinical Trials of Benmelstobart Injection Combined With Anlotinib Hydrochloride Capsules in the Treatment of Advanced or Unresectable Alveolar Soft Part Sarcoma · phase 2 · Chia Tai Tianqing Pharmaceutical Group Co., Ltd.
- Phase III Trial of Anlotinib, Catequentinib in Advanced Alveolar Soft Part Sarcoma, Leiomyosarcoma, Synovial Sarcoma (APROMISS) · phase 3 · Advenchen Laboratories, LLC
Open problems and what is being done
Why a low-mutation fusion sarcoma responds to immunotherapy is not understood.
Brain metastases are common and hard to treat.
and how the field plans to fix it →What is being done about thisAdvanced and metastatic diseaseAvailable now- IMRT / IGRT (modern external beam)Standard of care
- MRIStandard of care
- SBRT / SABR (stereotactic radiotherapy)Standard of care
In trialsNothing recorded yet.
Ideas and roadmapsNothing recorded yet.
Also on OnCo: Atlas of advanced disease · Invasion and metastasis.
No cure for metastatic disease despite long survival.
and how the field plans to fix it →What is being done about thisAdvanced and metastatic diseaseAvailable now- IMRT / IGRT (modern external beam)Standard of care
- MRIStandard of care
- SBRT / SABR (stereotactic radiotherapy)Standard of care
In trialsNothing recorded yet.
Ideas and roadmapsNothing recorded yet.
Also on OnCo: Atlas of advanced disease · Invasion and metastasis.
Trials
topTrials recruiting now
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Landmark trials
Expert centres
topExpert centres
Stanford · university | United States | 0 | 3,000 | 50,162 | #30 | ||
Amsterdam · cancer center | Netherlands | none recorded | 0 | 1,451 | 25,873 | #45 | |
Wuhan · hospital | China | none recorded | 0 | 2,478 | 31,527 | - | |
Dallas, TX · cancer center | United States | 0 | 1,744 | 19,757 | - | ||
Utrecht · cancer center | Netherlands | none recorded | 0 | 1,422 | 20,323 | - | |
Beijing · cancer center | China | none recorded | 0 | 1,344 | 18,195 | - | |
Leiden · university | Netherlands | none recorded | 0 | 1,245 | 16,125 | - | |
Tianjin · cancer center | China | none recorded | 0 | 1,219 | 11,455 | - | |
Hangzhou · cancer center | China | none recorded | 0 | 1,219 | 17,635 | - | |
Lyon · cancer center | France | none recorded | 0 | 1,071 | 14,301 | - | |
Rozzano (Milan) · hospital | Italy | none recorded | 0 | 1,031 | 10,720 | - | |
Zhengzhou · cancer center | China | none recorded | 0 | 970 | 12,409 | - | |
Padua · cancer center | Italy | none recorded | 0 | 962 | 12,326 | - | |
Naples · cancer center | Italy | none recorded | 0 | 961 | 15,028 | - | |
Changsha · cancer center | China | none recorded | 0 | 930 | 14,477 | - |
These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Alveolar soft part sarcoma but is thinly recorded here will look small.
Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.
Questions to ask
topQuestions to ask your oncologist about Alveolar soft part sarcoma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: 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?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: 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?Why: Inherited variants can change treatment and matter for relatives.
Localised
- For my situation (localised), which of the standard options do you recommend and why?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?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?Why: 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?Why: 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?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)?Why: 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?Why: 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?Why: 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?Why: 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?Why: Open problems are where trials and second opinions matter most.
Newly diagnosed? Read the first 60 days with Alveolar soft part sarcoma, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
8targets
4drugs
4companies
5trials
2Latest papers
topQuery for this cancer: (TITLE:"Alveolar soft part sarcoma" OR ABSTRACT:"Alveolar soft part sarcoma" OR TITLE:"ASPS" OR ABSTRACT:"ASPS" OR TITLE:"ASPSCR1-TFE3 sarcoma" OR ABSTRACT:"ASPSCR1-TFE3 sarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Alveolar soft part sarcoma, not a curated reading list.
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