# Adenoid cystic carcinoma

Source: https://onco.cc/cancers/adenoid-cystic-carcinoma/  
OnCo record `adenoid-cystic-carcinoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Summary

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: ACC; Cylindroma (historical); Adenoid cystic carcinoma of the salivary glands
- Tags: subtype-page; head-and-neck
- Group: head and neck
- Burden: Rare, but the second commonest salivary gland malignancy; it grows slowly along nerves, can recur years after treatment and can spread to the lungs, which is why follow-up runs well past the usual five years and why five-year survival overstates the long-term picture.
- Subtypes: Cribriform and tubular pattern (classical, indolent); Solid pattern or high-grade transformation (aggressive); NOTCH1-mutant adenoid cystic carcinoma (bone and liver spread, shorter survival); MYB-NFIB or MYBL1 fusion-positive disease (the majority); Adenoid cystic carcinoma of the minor salivary glands, palate and sinonasal tract; Adenoid cystic carcinoma outside the head and neck (lacrimal gland, trachea, breast)
- Biomarkers: MYB immunohistochemistry or MYB-NFIB fusion by FISH or sequencing; NOTCH1 mutation (aggressive subset, NOTCH inhibitor trials); Perineural invasion and margin status; Solid component and grade; Ki-67; PD-L1 (usually absent)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/adenoid-cystic-carcinoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/adenoid-cystic-carcinoma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/adenoid-cystic-carcinoma/#what-it-is [6 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/adenoid-cystic-carcinoma/#finding-it [6 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/adenoid-cystic-carcinoma/#treating-it [6 settings, 4 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/adenoid-cystic-carcinoma/#evidence [2 trials, 3 key papers, 5 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/adenoid-cystic-carcinoma/#science [7 targets]
- Countries and centres (own page): 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. https://onco.cc/cancers/adenoid-cystic-carcinoma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/adenoid-cystic-carcinoma/#living-with-it [19 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/adenoid-cystic-carcinoma/coming/ [6 medicines, 2 trials, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/adenoid-cystic-carcinoma/data/ [34 connected records]

## Standard of care

- 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. ([IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/))
- Unresectable or inoperable: Definitive radiotherapy; carbon-ion or proton therapy where available (COSMIC, Heidelberg; fast-neutron series). ([Carbon-ion therapy](https://onco.cc/technologies/carbon-ion/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/))
- Slow-growing metastatic disease: Observation with scans every few months; stereotactic radiotherapy or resection for isolated symptomatic metastases. ([SBRT / SABR (stereotactic radiotherapy)](https://onco.cc/technologies/sbrt/))
- Progressive metastatic disease: Lenvatinib or axitinib (phase 2 evidence); clinical trials preferred. ([Lenvatinib](https://onco.cc/drugs/lenvatinib/), [Axitinib](https://onco.cc/drugs/axitinib/), [Small-molecule kinase inhibitors](https://onco.cc/technologies/kinase-inhibitors/))
- Chemotherapy: Cisplatin with doxorubicin and cyclophosphamide, or single agents, for symptomatic disease after kinase inhibitors; responses are uncommon. ([Cisplatin](https://onco.cc/drugs/cisplatin/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/))
- Trials: MYB-directed REM-422, HG146 and NOTCH inhibitors for NOTCH1-mutant disease. ([REM-422](https://onco.cc/drugs/rem-422/), [Study of REM-422 in Patients With Recurrent, Metastatic, or Unresectable Adenoid Cystic Carcinoma](https://onco.cc/trials/nct06118086/), [Clinical Trial of HG146 Administered to Participants with Adenoid Cystic Carcinoma](https://onco.cc/trials/nct06781567/))

## State of the art

- Surgery and radiotherapy cure a minority; most patients live with slowly growing metastases for years.
- Lenvatinib and axitinib are the first drugs with randomised or reproducible phase 2 evidence of delaying progression.
- MYB and NOTCH1 biology has produced the first rational targets and the first trials designed for this disease alone.

## Open problems

- No drug shrinks the disease reliably; kinase inhibitors only slow it.
- When to start treatment for indolent lung metastases.
- Trials are small because the disease is rare and slow.
- Perineural spread makes clear margins impossible at the skull base.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Adenoid_cystic_carcinoma
- Wikipedia: https://en.wikipedia.org/wiki/Adenoid_cystic_carcinoma

## Connected records

- cancers: [Acinic cell carcinoma of the salivary glands](https://onco.cc/cancers/acinic-cell-carcinoma-salivary/), [Adenoid cystic carcinoma of the breast](https://onco.cc/cancers/adenoid-cystic-carcinoma-breast/), [Bartholin gland carcinoma](https://onco.cc/cancers/bartholin-gland-carcinoma/), [Carcinoma ex pleomorphic adenoma](https://onco.cc/cancers/carcinoma-ex-pleomorphic-adenoma/), [Mucoepidermoid carcinoma](https://onco.cc/cancers/mucoepidermoid-carcinoma/), [Salivary duct carcinoma](https://onco.cc/cancers/salivary-duct-carcinoma/), [Salivary gland cancers](https://onco.cc/cancers/salivary-gland/)
- key papers: [Phase 2 study of lenvatinib in progressive, recurrent or metastatic adenoid cystic carcinoma](https://onco.cc/key-papers/paper-tchekmedyian-lenvatinib-adenoid-cystic-jco-2019/), [Recurrent fusion of MYB and NFIB transcription factor genes in adenoid cystic carcinoma](https://onco.cc/key-papers/paper-persson-myb-nfib-pnas-2009/), [Systemic therapy in the palliative management of advanced salivary gland cancers (review)](https://onco.cc/key-papers/paper-laurie-licitra-salivary-systemic-jco-2006/)
- drugs: [Axitinib](https://onco.cc/drugs/axitinib/), [Cisplatin](https://onco.cc/drugs/cisplatin/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Lenvatinib](https://onco.cc/drugs/lenvatinib/), [REM-422](https://onco.cc/drugs/rem-422/)
- trials: [Clinical Trial of HG146 Administered to Participants with Adenoid Cystic Carcinoma](https://onco.cc/trials/nct06781567/), [Study of REM-422 in Patients With Recurrent, Metastatic, or Unresectable Adenoid Cystic Carcinoma](https://onco.cc/trials/nct06118086/)
- technologies: [Carbon-ion therapy](https://onco.cc/technologies/carbon-ion/), [IMRT / IGRT (modern external beam)](https://onco.cc/technologies/imrt-igrt/), [Proton therapy](https://onco.cc/technologies/proton-therapy/), [SBRT / SABR (stereotactic radiotherapy)](https://onco.cc/technologies/sbrt/), [Small-molecule kinase inhibitors](https://onco.cc/technologies/kinase-inhibitors/)
- targets: [CRTC1](https://onco.cc/targets/crtc1/), [ESRRA](https://onco.cc/targets/esrra/), [NFIB](https://onco.cc/targets/nfib/)

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JSON: https://onco.cc/api/v1/entities/adenoid-cystic-carcinoma.json