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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Localised | Wide resection with radiotherapy for close or positive margins; surveillance of lungs and brain. | not mapped |
| Advanced, first line | Atezolizumab (FDA approved 2023) or a VEGFR kinase inhibitor (sunitinib, pazopanib; anlotinib in China); conventional chemotherapy is not used. | not mapped |
| Oligometastatic | Metastasectomy or stereotactic radiotherapy to lung and brain lesions in slowly progressing disease. | not mapped |