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Adenoid cystic carcinoma: the decisions you may face

6 treatment settings, 4 with more than one named option. Each section lays out the options the standard of care names, what each is for, the trials behind them with their recorded results, the side effects and cautions on record, and questions to ask. Built from the cancer page's standard-of-care rows; nothing here is advice for your case.

Early / localised

Localised, resectable

2 options

Wide resection including involved nerves where needed, with neck dissection for node-positive disease, followed by postoperative radiotherapy to the bed and nerve pathways.

The options, in plain words

IMRT and IGRT shape the radiation beam to the tumour's outline from multiple angles and check the patient's position with a scan before every session, so surrounding organs receive less dose. Fewer, larger doses are now standard in breast and prostate cancer, but a low-dose bath still spreads across normal tissue.

  • Conformal dose, fewer side effects
  • Hypofractionation saves visits
Proton therapyEstablished

Radiation using protons, which stop inside the tumour instead of passing through, so tissue behind it gets no dose.

  • No exit dose; lower integral dose
  • Reduced second cancers in children
The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record
  • Low-dose bath to normal tissue
  • Motion management
  • Cost
  • Range uncertainty
  • Limited randomised evidence in adults
Questions to ask about this decision
  1. Between IMRT / IGRT (modern external beam) and Proton therapy, which do you recommend for me, and what about my case would make you choose differently?
    Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
  2. What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
    Why: The aim shapes how much side effect and disruption is worth accepting.
  3. What happens if I delay, or decline this step for now? Is the decision reversible?
    Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
  4. Does your recommendation follow the current guideline (NCCN Guidelines: Head and Neck Cancers), and if it departs from it, why?
    Why: Departures from guidelines are sometimes right for an individual; they should be explained.
  5. For my situation (localised, resectable), which of the standard options do you recommend and why?
    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.

Add these to your appointment list, or take the full question set for this cancer.

Locally advanced

Unresectable or inoperable

3 options

Definitive radiotherapy; carbon-ion or proton therapy where available (COSMIC, Heidelberg; fast-neutron series).

The options, in plain words

Heavier charged particles that kill even radiation-resistant tumours, available at only a handful of centres worldwide.

  • Effective in radioresistant tumours
Proton therapyEstablished

Radiation using protons, which stop inside the tumour instead of passing through, so tissue behind it gets no dose.

  • No exit dose; lower integral dose
  • Reduced second cancers in children

IMRT and IGRT shape the radiation beam to the tumour's outline from multiple angles and check the patient's position with a scan before every session, so surrounding organs receive less dose. Fewer, larger doses are now standard in breast and prostate cancer, but a low-dose bath still spreads across normal tissue.

  • Conformal dose, fewer side effects
  • Hypofractionation saves visits
The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record
  • Very few facilities
  • Cost
  • Cost
  • Range uncertainty
  • Limited randomised evidence in adults
  • Low-dose bath to normal tissue
  • Motion management
Questions to ask about this decision
  1. Between Carbon-ion therapy, Proton therapy and IMRT / IGRT (modern external beam), which do you recommend for me, and what about my case would make you choose differently?
    Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
  2. What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
    Why: The aim shapes how much side effect and disruption is worth accepting.
  3. What happens if I delay, or decline this step for now? Is the decision reversible?
    Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
  4. Does your recommendation follow the current guideline (ESMO salivary gland cancer guideline 2022), and if it departs from it, why?
    Why: Departures from guidelines are sometimes right for an individual; they should be explained.
  5. For my situation (unresectable or inoperable), which of the standard options do you recommend and why?
    Why: Guideline options include: Definitive radiotherapy; carbon-ion or proton therapy where available (COSMIC, Heidelberg; fast-neutron series).

Add these to your appointment list, or take the full question set for this cancer.

Advanced, first line

Slow-growing metastatic disease

One path named

Observation with scans every few months; stereotactic radiotherapy or resection for isolated symptomatic metastases.

The path, in plain words

Stereotactic body radiotherapy converges multiple beams with sub-millimetre accuracy to deliver tumour-destroying doses in one to five outpatient sessions, doing the job of surgery for inoperable early lung cancer and for metastases in liver, spine and brain. Tumour size and location limit its use, and late toxicity is a concern near the central airways.

  • Ablative doses with minimal recovery
  • Outpatient
The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record
  • Size and location limits
  • Late toxicity near central airways
Questions to ask about this decision
  1. Is SBRT / SABR (stereotactic radiotherapy) the only reasonable path for me, or is there a trial, a different sequence or a wait-and-see option?
    Why: A single standard does not mean a single choice; timing and trials are decisions too.
  2. What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
    Why: The aim shapes how much side effect and disruption is worth accepting.
  3. What happens if I delay, or decline this step for now? Is the decision reversible?
    Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
  4. Does your recommendation follow the current guideline (NCCN Guidelines: Head and Neck Cancers), and if it departs from it, why?
    Why: Departures from guidelines are sometimes right for an individual; they should be explained.
  5. For my situation (slow-growing metastatic disease), which of the standard options do you recommend and why?
    Why: Guideline options include: Observation with scans every few months; stereotactic radiotherapy or resection for isolated symptomatic metastases.

Add these to your appointment list, or take the full question set for this cancer.

Second line

Progressive metastatic disease

Lenvatinib or axitinib (phase 2 evidence); clinical trials preferred.

The options, in plain words

An oral anti-angiogenic pill that matched sorafenib in liver cancer with higher response rates, and partners with pembrolizumab in kidney and endometrial cancer.

Axitinib is a selective VEGF-receptor pill, now given mainly with pembrolizumab or avelumab as first-line kidney cancer treatment.

Pills that block the specific enzyme a cancer relies on. Imatinib in 2001 proved a cancer could be switched off by design.

  • Oral, outpatient
  • Dramatic responses in oncogene-addicted cancers
The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record
  • Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
  • Reduce to 14 mg (thyroid) or 10 mg (RCC) in severe impairment.
  • Reduce in severe renal impairment.
  • Avoid grapefruit.
  • Halve the starting dose in moderate impairment.
  • Near-universal resistance in metastatic disease
  • Off-target toxicities
Questions to ask about this decision
  1. Between Lenvatinib, Axitinib and Small-molecule kinase inhibitors, which do you recommend for me, and what about my case would make you choose differently?
    Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
  2. What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
    Why: The aim shapes how much side effect and disruption is worth accepting.
  3. What happens if I delay, or decline this step for now? Is the decision reversible?
    Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
  4. Does your recommendation follow the current guideline (NCCN Guidelines: Head and Neck Cancers), and if it departs from it, why?
    Why: Departures from guidelines are sometimes right for an individual; they should be explained.
  5. For my situation (progressive metastatic disease), which of the standard options do you recommend and why?
    Why: Guideline options include: Lenvatinib or axitinib (phase 2 evidence); clinical trials preferred.
  6. Am I a candidate for Lenvatinib, Axitinib, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Add these to your appointment list, or take the full question set for this cancer.

Cisplatin with doxorubicin and cyclophosphamide, or single agents, for symptomatic disease after kinase inhibitors; responses are uncommon.

The options, in plain words

Cisplatin is the original platinum chemotherapy, discovered by accident in 1965; it cures testicular cancer and makes radiation work better in cervical and head and neck cancer.

The red chemotherapy drug from a soil bacterium that is still the backbone of treatment for sarcoma, lymphoma and breast cancer, limited by cumulative heart damage.

Cyclophosphamide is an alkylating chemotherapy that damages DNA in dividing cells. It is part of CHOP for lymphoma, AC for breast cancer and VAC for childhood sarcomas, clears lymphocytes before CAR-T and prevents graft-versus-host disease after transplant; bladder bleeding, infertility and secondary leukaemia are its harms.

The evidence behind it

No trial record is attached to this row yet. The trials tab lists what is recruiting and the landmark trials for this cancer.

The main trade-offs on record
  • Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
  • Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
  • Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
Questions to ask about this decision
  1. Between Cisplatin, Doxorubicin and Cyclophosphamide, which do you recommend for me, and what about my case would make you choose differently?
    Why: Guidelines list several reasonable options; the choice turns on details of your tumour, your health and your priorities.
  2. What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
    Why: The aim shapes how much side effect and disruption is worth accepting.
  3. What happens if I delay, or decline this step for now? Is the decision reversible?
    Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
  4. Does your recommendation follow the current guideline (NCCN Guidelines: Head and Neck Cancers), and if it departs from it, why?
    Why: Departures from guidelines are sometimes right for an individual; they should be explained.
  5. For my situation (chemotherapy), which of the standard options do you recommend and why?
    Why: Guideline options include: Cisplatin with doxorubicin and cyclophosphamide, or single agents, for symptomatic disease after kinase inhibitors; responses are uncommon.
  6. Am I a candidate for Cisplatin, Doxorubicin, Cyclophosphamide, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Add these to your appointment list, or take the full question set for this cancer.

One path named

MYB-directed REM-422, HG146 and NOTCH inhibitors for NOTCH1-mutant disease.

The path, in plain words

REM-422 is a small-molecule inhibitor from Remix Therapeutics, in registered phase 2 trials for salivary gland cancers.

The evidence behind it
The main trade-offs on record

No side-effect rates or interaction flags are recorded for these options yet. The side-effect lookup and interaction checker cover the products that have them.

Questions to ask about this decision
  1. Is REM-422 the only reasonable path for me, or is there a trial, a different sequence or a wait-and-see option?
    Why: A single standard does not mean a single choice; timing and trials are decisions too.
  2. What is each option trying to achieve: cure, long control, or relief of symptoms, and over what time?
    Why: The aim shapes how much side effect and disruption is worth accepting.
  3. How closely do I match the people in 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, and does that change what the results mean for me?
    Why: Trial populations are selected; age, fitness, prior treatment and biomarkers all affect how far results carry.
  4. What happens if I delay, or decline this step for now? Is the decision reversible?
    Why: Some decisions can wait for a second opinion or a trial slot; others cannot. Knowing which is part of the choice.
  5. For my situation (trials), which of the standard options do you recommend and why?
    Why: Guideline options include: MYB-directed REM-422, HG146 and NOTCH inhibitors for NOTCH1-mutant disease.
  6. Am I a candidate for REM-422, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
  7. 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?
    Why: Trial populations differ from individual patients; ask how closely you match.

Add these to your appointment list, or take the full question set for this cancer.

How to read this page. Options and results come from OnCo records with their sources; the settings are the standard-of-care rows on the cancer page, and the lines of therapy are on the sequencing grid. Where a setting names one path, the choice is usually about timing, trials and where to be treated: see expert centres. OnCo is orientation, not medical advice.