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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.
Cytotoxic chemotherapy rarely produces responses. Multikinase inhibitors that block VEGF receptors are the drugs with evidence: lenvatinib produced responses in 15.6 percent of 32 patients with median progression-free survival of 17.5 months in a Memorial Sloan Kettering phase 2, and a randomised phase 2 of axitinib against observation in progressive disease found median progression-free survival of 10.8 against 2.8 months, so both appear in the NCCN guideline for progressive disease while indolent metastases are observed. Immune checkpoint inhibitors have little activity; NOTCH inhibitors gave modest responses in NOTCH1-mutant disease; and the MYB messenger RNA degrader REM-422 and the drug HG146 are in early 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.
Site decides cause and behaviour: HPV drives oropharyngeal cancer, EBV drives nasopharyngeal cancer, tobacco drives oral and laryngeal cancer; all drain into the neck node levels that surgeons and radiotherapists map.
Same organ: Acinic cell carcinoma of the salivary glands, Carcinoma ex pleomorphic adenoma, Multiple endocrine neoplasia type 1 (MEN1), Multiple endocrine neoplasia type 2 (MEN2A and MEN2B), Hyperparathyroidism-jaw tumour syndrome (CDC73-related parathyroid carcinoma), Oropharyngeal cancer (tonsil and base of tongue), Laryngeal and hypopharyngeal cancer, Oral cavity cancer (mouth and tongue), Head and neck squamous cell carcinoma, Nasopharyngeal carcinoma, Salivary gland cancers, Papillary thyroid cancer, Follicular thyroid cancer, Medullary thyroid cancer, Anaplastic thyroid cancer, Thyroid cancer, Nasal cavity and paranasal sinus cancers (including esthesioneuroblastoma), NUT carcinoma (midline carcinoma with NUTM1 rearrangement), Parathyroid carcinoma, Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4), HPV-positive oropharyngeal cancer, HPV-negative head and neck squamous cell carcinoma (including HPV-negative oropharyngeal cancer), Recurrent or metastatic head and neck squamous cell carcinoma, Hypopharyngeal cancer, Salivary duct carcinoma, Mucoepidermoid carcinoma, Oral tongue and floor of mouth cancer, Buccal mucosa and gingivobuccal cancer (oral cancer in India), Lip cancer, Locoregionally advanced nasopharyngeal carcinoma (stage III to IVA), Recurrent and metastatic nasopharyngeal carcinoma, Esthesioneuroblastoma (olfactory neuroblastoma), Sinonasal undifferentiated carcinoma (SNUC) and SWI/SNF-deficient sinonasal carcinoma
Wide resection including involved nerves where needed, with neck dissection for node-positive disease, followed by postoperative radiotherapy to the bed and nerve pathways.
Definitive radiotherapy; carbon-ion or proton therapy where available (COSMIC, Heidelberg; fast-neutron series).
Observation with scans every few months; stereotactic radiotherapy or resection for isolated symptomatic metastases.
Lenvatinib or axitinib (phase 2 evidence); clinical trials preferred.
Cisplatin with doxorubicin and cyclophosphamide, or single agents, for symptomatic disease after kinase inhibitors; responses are uncommon.
MYB-directed REM-422, HG146 and NOTCH inhibitors for NOTCH1-mutant disease.
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Lenvatinib (like axitinib) is a guideline option for progressing adenoid cystic carcinoma; responses are uncommon, so it is reserved for documented progression rather than indolent disease.
MYB immunostaining and MYB-NFIB fusion testing are now diagnostic tools for adenoid cystic carcinoma, and MYB is the principal target of therapeutic research in the disease.
The observation-first approach for indolent metastases and the move to biomarker-directed therapy (HER2, androgen receptor, NTRK) on the salivary cancer pages follow this review's conclusions.
Query for this cancer: (TITLE:"Adenoid cystic carcinoma" OR ABSTRACT:"Adenoid cystic carcinoma" OR TITLE:"ACC" OR ABSTRACT:"ACC" OR TITLE:"Cylindroma historical" OR ABSTRACT:"Cylindroma historical" OR TITLE:"Adenoid cystic carcinoma of the salivary glands" OR ABSTRACT:"Adenoid cystic carcinoma of the salivary glands") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Adenoid cystic carcinoma, 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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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
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.
Avoid grapefruit.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
See all on the product pages:AxitinibCisplatinCyclophosphamideDoxorubicinLenvatinib·Printable cards in the navigator
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