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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.
Adenoid cystic carcinoma is a slow-growing cancer of the salivary glands that spreads along nerves and can come back years after treatment, often in the lungs. Surgery with radiotherapy is the treatment that cures it, chemotherapy has little effect, and the tablets lenvatinib and axitinib can hold spreading disease still for months rather than shrink it.
Adenoid cystic carcinoma arises in the minor salivary glands of the palate and sinonasal tract, the submandibular gland and the parotid, and occasionally in the lacrimal gland, trachea or breast. It is defined by a MYB-NFIB (or MYBL1) fusion in most cases, grows in cribriform, tubular or solid patterns, and invades nerves, so facial numbness or pain is a common first symptom. A subset with activating NOTCH1 mutations and solid histology behaves aggressively and spreads to bone and liver; the rest progress slowly, with lung metastases that may be watched for years.
Cure depends on surgery with the widest margins the anatomy allows, often sacrificing nerves, followed by radiotherapy, which reduces local recurrence but cannot be shown to improve survival in a disease that recurs so late. Unresectable tumours are treated with radiotherapy alone, and heavy-particle therapy has a particular place: the Heidelberg COSMIC trial of intensity-modulated radiotherapy with a carbon-ion boost and the older fast-neutron series reported better local control than photons, and proton and carbon-ion therapy are offered where available.
| Setting | Approach | Guideline |
|---|---|---|
| Localised, resectable | Wide resection including involved nerves where needed, with neck dissection for node-positive disease, followed by postoperative radiotherapy to the bed and nerve pathways. | NCCN Category 2A |
| Unresectable or inoperable | Definitive radiotherapy; carbon-ion or proton therapy where available (COSMIC, Heidelberg; fast-neutron series). | not mapped |
| Slow-growing metastatic disease | Observation with scans every few months; stereotactic radiotherapy or resection for isolated symptomatic metastases. | NCCN Category 2A |
| Progressive metastatic disease | Lenvatinib or axitinib (phase 2 evidence); clinical trials preferred. | NCCN Category 2A |
| Chemotherapy | Cisplatin with doxorubicin and cyclophosphamide, or single agents, for symptomatic disease after kinase inhibitors; responses are uncommon. | NCCN Category 2B |
| Trials | MYB-directed REM-422, HG146 and NOTCH inhibitors for NOTCH1-mutant disease. | not mapped |