The first 60 days: Adenoid cystic carcinoma
Adenoid cystic carcinoma is a slow but relentless cancer of the salivary glands that creeps along nerves and comes back years later, often in the lungs. Surgery with radiotherapy is the only cure, chemotherapy barely works, and the tablets lenvatinib and axitinib can hold spreading disease still for months rather than shrink it. Below, week by week, is what OnCo's record of Adenoid cystic carcinoma says about the first two months: the order is typical, the timing is yours to ask about. Sections appear only where the record has something to say. Orientation, not medical advice.
What happens now
Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.
Ask which of these were tested and what the results were; see the report reader for what each value means.
These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.
- SurgeonNamed in the standard of care for: Localised, resectable, Slow-growing metastatic disease.
- Medical oncologistNamed in the standard of care for: Localised, resectable, Unresectable or inoperable, Slow-growing metastatic disease, Progressive metastatic disease and 2 more.
- Clinical oncologist (radiotherapy)Named in the standard of care for: Localised, resectable, Unresectable or inoperable, Slow-growing metastatic disease.
- Transplant and cell therapy teamNamed in the standard of care for: Chemotherapy.
- Palliative and supportive care teamNamed in the standard of care for: Chemotherapy.
A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.
Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.
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.
- 6.Trials
MYB-directed REM-422, HG146 and NOTCH inhibitors for NOTCH1-mutant disease.
Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example MYB immunohistochemistry or MYB-NFIB fusion by FISH or sequencing, NOTCH1 mutation, Perineural invasion and margin status, Solid component and grade, Ki-67), and what were the results?These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Recognised subtypes for this cancer include Cribriform and tubular pattern, Solid pattern or high-grade transformation, NOTCH1-mutant adenoid cystic carcinoma.
- Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.
Localised, resectable
- For my situation (localised, resectable), which of the standard options do you recommend and why?Guideline options include: Wide resection including involved nerves where needed, with neck dissection for node-positive disease, followed by postoperative radiotherapy to the bed and nerve pathways.
Unresectable or inoperable
- For my situation (unresectable or inoperable), which of the standard options do you recommend and why?Guideline options include: Definitive radiotherapy; carbon-ion or proton therapy where available (COSMIC, Heidelberg; fast-neutron series).
Slow-growing metastatic disease
- For my situation (slow-growing metastatic disease), which of the standard options do you recommend and why?Guideline options include: Observation with scans every few months; stereotactic radiotherapy or resection for isolated symptomatic metastases.
Progressive metastatic disease
- For my situation (progressive metastatic disease), which of the standard options do you recommend and why?Guideline options include: Lenvatinib or axitinib (phase 2 evidence); clinical trials preferred.
- Am I a candidate for Lenvatinib, Axitinib, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Chemotherapy
- For my situation (chemotherapy), which of the standard options do you recommend and why?Guideline options include: Cisplatin with doxorubicin and cyclophosphamide, or single agents, for symptomatic disease after kinase inhibitors; responses are uncommon.
- Am I a candidate for Cisplatin, Doxorubicin, Cyclophosphamide, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
Trials
- For my situation (trials), which of the standard options do you recommend and why?Guideline options include: MYB-directed REM-422, HG146 and NOTCH inhibitors for NOTCH1-mutant disease.
- Am I a candidate for REM-422, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of Study of REM-422 in Patients With Recurrent, Metastatic, or Unresectable Adenoid Cystic Carcinoma and Clinical Trial of HG146 Administered to Participants with Adenoid Cystic Carcinoma apply to someone like me?Trial populations differ from individual patients; ask how closely you match.
Any stage
- Are there clinical trials I could join, for example of REM-422, Study of REM-422 in Patients With Recurrent, Metastatic, or Unresectable Adenoid Cystic Carcinoma, Clinical Trial of HG146 Administered to Participants with Adenoid Cystic Carcinoma, Lenvatinib?Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Supportive care improves quality of life and helps patients complete treatment.
- I read that “No drug shrinks the disease reliably; kinase inhibitors only slow it”. How does that affect my plan?Open problems are where trials and second opinions matter most.
- I read that “When to start treatment for indolent lung metastases”. How does that affect my plan?Open problems are where trials and second opinions matter most.
Trials open now for this cancer in OnCo, largest phase first. Joining a trial is a decision like any other: ask what the comparison arm is, whether a placebo is used, and what happens if you leave. The cancer page searches ClinicalTrials.gov live for more.
- Clinical Trial of HG146 Administered to Participants with Adenoid Cystic CarcinomaPhase 2 · recruiting · NCT06781567A Phase Il Clinical Study to Evaluate the Efficacy and Safety of HG146 Capsules in Participants with Recurrent or Metastatic Adenoid Cystic Carcinoma.
- Study of REM-422 in Patients With Recurrent, Metastatic, or Unresectable Adenoid Cystic CarcinomaPhase 1/2 · recruiting · NCT06118086A Phase 1/2, Multicenter, Open-label Study of REM-422, a MYB mRNA Degrader, in Patients With Recurrent, Metastatic, or Unresectable Adenoid Cystic Carcinoma
Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.
- Adenoid cystic carcinoma: the full pageAdenoid cystic carcinoma is a slow but relentless cancer of the salivary glands that creeps along nerves and comes back years later, often in the lungs. Surgery with radiotherapy is the only cure, chemotherapy barely works, and the tablets lenvatinib and axitinib can hold spreading disease still for months rather than shrink it.
- One-page appointment sheetYour questions, the words you may hear, what to bring, and space for the answers. Print it.
- Treatment sequencingWhich treatment tends to follow which, line by line.
- NavigatorStandard of care for your stage, what you have tried, and trials near you.