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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.
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.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
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.
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.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, 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)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
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Atezolizumab is the first-line systemic treatment for advanced alveolar soft part sarcoma, a rare, slow-growing but ultimately metastatic sarcoma of young adults for which chemotherapy never worked.
TFE3 immunostaining and ASPSCR1-TFE3 fusion testing confirm the diagnosis of alveolar soft part sarcoma and link it to a family of TFE3-rearranged tumours including a type of renal cell carcinoma.
Query 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.
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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Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
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.
Take on an empty stomach (1 hour before or 2 hours after food).
Avoid grapefruit.
See all on the product pages:AnlotinibAtezolizumabPazopanibSunitinib·Printable cards in the navigator
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