Adenoid cystic carcinoma is a rare cancer that grows from tiny glands that make fluid, most often the salivary glands around the mouth and face. It usually grows slowly but can travel along nerves and come back after many years. It may spread to the lungs or bones later in life. Doctors often see it as a painless lump at first, but nerve pain or numbness can happen because the tumor likes to move along nerve paths. Because of these behaviors, careful imaging and a tissue biopsy are needed to diagnose it correctly. NCBICleveland Clinicar.iiarjournals.org
Adenoid cystic carcinoma (ACC) is a rare cancer that often starts in the salivary glands in the head and neck. It can also begin in the lacrimal gland (tear gland), trachea and bronchi, breast, skin (rare), or other secretory glands. The tumor grows slowly, but it can spread along nerves (called perineural invasion). It may come back years later and can spread to the lungs, bones, liver, or brain. Pathologists see three main patterns: cribriform (sieve-like holes), tubular (small tubes), and solid (sheets of cells). Solid areas behave more aggressively. ACC cells often carry gene fusions such as MYB-NFIB or MYBL1; these help confirm the diagnosis in a lab.
Other names
Adenoid cystic carcinoma is also called AdCC, adenocystic carcinoma (older spelling), and historically cylindroma because its tumor cells can form round, “cylindrical” spaces under the microscope. You may also see “salivary gland–type AdCC” when it starts in places like the sinuses, trachea, or breast but looks the same as salivary AdCC. In reports, pathologists may write “cribriform–tubular–solid AdCC” to describe its three common growth patterns. These are all descriptions of the same disease. PMCMeridian
Types
By microscopic pattern. Pathologists describe three patterns: cribriform, tubular, and solid. Tumors with a large “solid” portion tend to act more aggressively than the others. Meridian
By anatomic site. Most AdCC begins in minor salivary glands (palate and other mouth sites) or major salivary glands (parotid, submandibular). Less often it starts in the sinonasal tract, trachea/bronchi, lacrimal gland, skin, breast, or female genital tract, but the biology is similar. NCBIPMC
By clinical extent. Doctors often group cases as localized (confined to the original gland), locally advanced (into nearby soft tissues/along nerves/skull base), or metastatic (commonly to lung). This matters for planning surgery, radiation, and follow-up imaging. Cancer.gov
Causes
No single lifestyle cause has been proven. Most cases happen sporadically. Modern research shows that changes in certain genes seem to drive this cancer; the best studied are MYB and MYBL1 gene rearrangements, often formed by a fusion with NFIB. Below is a practical list of possible factors and biologic drivers people ask about. Items 1–6 have stronger research links (molecular drivers); others are general salivary-gland cancer risks or clinical observations with weaker evidence for AdCC specifically.
MYB–NFIB fusion (oncogenic driver found in many tumors). PubMed
MYB alterations (split/loss/overexpression) even without the NFIB fusion. PubMed
MYBL1 rearrangements (MYB-like driver in a subset). PubMed
Overexpression of MYB/MYBL1 proteins (seen in most tumors). PubMedMDPI
NFIB partner gene involvement (molecular hallmark that helps confirm diagnosis). oooojournal.net
Other cooperating genetic changes (being mapped; help explain behavior and prognosis). MDPI
Prior head-and-neck radiation (general salivary gland cancer risk; evidence specific to AdCC is limited). Cancer.gov
Older age (AdCC is more common in mid-to-late adulthood). NCBI
Female sex slight predominance (descriptive epidemiology; cause unknown). PMC
Occupational exposures linked to salivary cancers in general (e.g., certain industrial dusts/chemicals; AdCC-specific proof is limited). Kaiser Permanente
Chronic inflammation/scar in glands (theory in gland tumors generally; not proven causation). NCBI
Immune microenvironment changes that let tumor cells evade defenses (emerging science). PMC
Perineural niche signaling that favors growth along nerves (explains spread pattern more than “cause”). clinicalradiologyonline.net
Hormonal influence (no strong evidence; investigated and largely inconclusive). NCBI
Family history of salivary gland tumor (rare; no clear hereditary AdCC syndrome). NCBI
Viral causes (e.g., HPV/EBV): not established for AdCC. NCBI
Lifestyle factors (tobacco/alcohol): not clearly tied to AdCC behavior. NCBI
Long natural history with time for mutations to accumulate (explains late recurrences). ar.iiarjournals.org
Site-specific microenvironments (sinonasal vs tracheal vs lacrimal glands) that may shape how tumors start. PMC
Random DNA replication errors (background risk in many cancers). NCBI
Plain-language note: doctors speak of risk factors and drivers, not proven day-to-day “causes,” for AdCC. The MYB/MYBL1 pathway is the most consistent signal in modern studies. PubMed
Symptoms and signs
Each person’s symptoms depend on where the tumor starts and where it travels.
Painless, firm lump in the palate, cheek, or under the jaw. This is common at first. NCBI
Pain or burning that spreads along the face or mouth because the tumor follows nerves. ar.iiarjournals.org
Numbness or tingling in lips, cheek, teeth, or palate. Cleveland Clinic
Weakness of facial muscles (smile asymmetry, eye not closing) if the facial nerve is involved. NCBI
Trouble opening the mouth (trismus) if muscles of chewing are affected. NCBI
Tooth loosening or pain when the palate/alveolus is involved. NCBI
Nasal blockage or nosebleeds in sinonasal AdCC. PMC
Eye pain, bulging, or double vision in lacrimal gland AdCC. NCBI
Hoarseness or noisy breathing in laryngo-tracheal AdCC. ScienceDirect
Cough or coughing blood if airway or lung is involved. ScienceDirect
Sore throat or trouble swallowing for base-of-tongue or pharyngeal sites. NCBI
Ear pain referred from throat or jaw areas. NCBI
Non-healing mouth ulcer over a hard lump. NCBI
Shortness of breath from lung spread (often years later). Cleveland Clinic
Unplanned weight loss or fatigue in advanced disease. NCBI
Diagnostic tests
A) Physical examination
Head-and-neck exam. The clinician looks and feels for lumps in the mouth, palate, tongue, cheek, and jawline, checks mouth opening, and inspects the skin. Purpose: find the main tumor and any satellite masses. Why it helps: AdCC often presents as a firm, painless lump. NCBI
Neck lymph-node exam. The neck is palpated for enlarged nodes. Purpose: staging. Why it helps: nodal spread is less common than in some other head-and-neck cancers, but it can happen. Cancer.gov
Cranial nerve exam. Sensation, facial movement, taste, and eye movements are checked. Purpose: detect perineural spread. Why it helps: subtle numbness or weakness may be the first clue. Cleveland Clinic
In-office endoscopy of nose/throat (flexible scope). The doctor directly sees the sinonasal cavity and throat. Purpose: map tumor extent and biopsy safely. PMC
B) Manual/bedside tests
Bimanual palpation of salivary glands and floor of mouth. One hand inside, one outside to feel deep masses. Purpose: define size and fixation. NCBI
Sensory mapping of the face and palate. Light touch/pinprick lines are drawn to see which nerve branches are affected. Purpose: guides imaging fields along nerve pathways. clinicalradiologyonline.net
Tinel-like tapping over foramina or along nerve paths. Gentle tapping may reproduce tingling if a nerve is involved. Purpose: bedside clue to perineural irritation. clinicalradiologyonline.net
Lacrimal/ocular surface checks (e.g., tear film tests) when the lacrimal gland is involved. Purpose: symptom relief planning and baseline function. NCBI
C) Laboratory & pathological tests
Fine-needle aspiration (FNA). A thin needle removes cells for cytology. Purpose: early, low-risk tissue diagnosis. Explanation: can suggest AdCC, but core biopsy may be needed to see patterns. NCBI
Core needle or incisional biopsy. A small cylinder or piece of tissue is taken. Purpose: confirm architecture (cribriform/tubular/solid) and perineural features. Meridian
Definitive histopathology. Under the microscope, the pathologist looks for cribriform “holes,” tubular glands, or solid sheets; checks margins and nerves. Purpose: grading and planning surgery/radiation. Meridian
Immunohistochemistry (IHC). Stains like MYB, SOX10, and CD117 (c-KIT) support the diagnosis. Purpose: strengthen accuracy, especially on small biopsies. Meridian
FISH for MYB or MYB–NFIB rearrangements. Purpose: detect the classic genetic hallmark; helpful when morphology is tricky. PubMedoooojournal.net
RT-PCR for fusion transcripts. Purpose: confirm MYB–NFIB or MYBL1 rearrangements at the RNA level. ScienceDirect
Next-generation sequencing (NGS) panel. Purpose: look for driver events (MYB/MYBL1/NFIB and others) and potential trial options; can inform prognosis. MDPI
Baseline blood tests (CBC, chemistry). Purpose: general fitness for anesthesia and treatment; they do not diagnose AdCC. Cancer.gov
D) Electrodiagnostic tests
Nerve-conduction studies (NCS). Surface electrodes test how fast facial or trigeminal signals travel. Purpose: document nerve injury when symptoms are unclear; helps track perineural disease impact. clinicalradiologyonline.net
Electromyography (EMG). A tiny needle checks muscle electrical activity (e.g., facial muscles) to see if a nerve is damaged. Purpose: baseline function and recovery monitoring. clinicalradiologyonline.net
E) Imaging tests
MRI with contrast of the primary site and nerve pathways. Purpose: best test to map perineural spread into skull base; more sensitive than CT for this question. Explanation: radiologists follow the affected nerve back to foramina and brainstem. PMCScienceDirectclinicalradiologyonline.net
CT scan with contrast of head/neck. Purpose: shows bone erosion, calcification, and surgical anatomy; complements MRI. clinicalradiologyonline.net
Ultrasound of salivary glands/neck. Purpose: characterizes superficial masses and guides FNA/core biopsy. NCBI
Chest CT (baseline and follow-up). Purpose: look for lung metastases, which can appear late and be slow-growing. Cleveland Clinic
Whole-body PET-CT (selected cases). Purpose: survey for distant disease or clarify uncertain findings; sensitivity varies with slow tumors. Cancer.gov
Targeted MRI of skull base or orbit/sinonasal tract (when symptoms point there). Purpose: define complex routes of spread for surgery or radiation planning. PMC
Non-pharmacological treatments
(15 physiotherapy & rehabilitation; plus mind-body, “gene-informed” education, and practical self-management). Each item explains description, purpose, mechanism, and benefits in plain words.)
1) Shoulder and neck range-of-motion therapy (post-surgery/RT)
Description: Gentle, guided stretches for neck, shoulder, and jaw after parotid, submandibular, or palate surgery and after radiation.
Purpose: Prevent stiffness and frozen shoulder; restore daily movement.
Mechanism: Repeated slow stretching lengthens tight capsules and fascia, reduces scar adhesion, and improves synovial glide.
Benefits: Less pain, better reach and head turn, easier hair care, driving, and sleep.
2) Jaw opening (trismus) therapy
Description: Daily mouth-opening exercises using stacked tongue depressors or a jaw device, plus massage of the chewing muscles.
Purpose: Prevent or reverse trismus from surgery or radiation.
Mechanism: Low-load prolonged stretch remodels muscle and connective tissue; neuromuscular retraining lowers spasm.
Benefits: Easier eating, speech, dental care; less jaw pain.
3) Swallowing rehabilitation with a speech-language pathologist (SLP)
Description: Individual swallowing drills, safe-swallow strategies, and food texture planning.
Purpose: Maintain nutrition and prevent aspiration.
Mechanism: Repetitive practice strengthens tongue, pharynx, and larynx; compensations (chin-tuck, effortful swallow) improve airway protection.
Benefits: Safer eating, fewer chest infections, better energy.
4) Head-and-neck lymphedema therapy
Description: Manual lymph drainage, compression garments, and self-massage training.
Purpose: Control swelling after lymph node removal or radiation.
Mechanism: Gentle strokes redirect lymph fluid to open channels; compression improves tissue pump.
Benefits: Smaller neck/face swelling, better appearance, less heaviness.
5) Scar management and desensitization
Description: Scar massage, silicone sheets, and friction desensitization around incisions.
Purpose: Keep scars supple; reduce nerve sensitivity.
Mechanism: Mechanical input breaks minor adhesions; silicone optimizes hydration; graded touch calms over-active nerve endings.
Benefits: Softer scars, less itch, easier neck turning.
6) Postural retraining
Description: Ergonomic coaching and exercises to align head, shoulders, and spine.
Purpose: Reduce muscle overload and headaches.
Mechanism: Strengthening deep neck flexors and scapular stabilizers redistributes loads; posture cues recalibrate proprioception.
Benefits: Less fatigue and pain; improved breathing and voice.
7) Breathing exercises (including diaphragmatic breathing)
Description: Daily slow nasal breaths with belly rise; paced breathing.
Purpose: Ease anxiety, improve ventilation, and support speech.
Mechanism: Activates parasympathetic tone; improves tidal volume and CO₂ regulation.
Benefits: Calmer mood, better stamina, steadier voice.
8) Airway clearance techniques (for tracheal/bronchial ACC)
Description: Active cycle of breathing, huff cough, oscillating positive expiratory pressure devices.
Purpose: Move mucus and reduce infections.
Mechanism: Oscillations and back-pressure keep airways open; airflow shears mucus off walls.
Benefits: Less cough, easier breathing, fewer flares.
9) Neuromobilization for perineural pain
Description: Gentle nerve glide exercises supervised by a therapist.
Purpose: Reduce tingling, shooting pain, and tightness from nerve involvement.
Mechanism: Sliding nerves within tunnels reduces mechanosensitivity and edema.
Benefits: Less neuropathic pain; better function.
10) TENS (transcutaneous electrical nerve stimulation)
Description: Small skin electrodes deliver mild pulses near painful areas.
Purpose: Non-drug pain relief.
Mechanism: Activates A-beta fibers to gate pain signals; may trigger endorphin release.
Benefits: Lower pain scores; less need for pills.
11) Balance and fall-prevention training
Description: Progressive balance drills and safe-home advice.
Purpose: Offset weakness, neuropathy, or vision changes.
Mechanism: Challenges vestibular and proprioceptive systems; builds strength.
Benefits: Fewer stumbles; more confidence.
12) Progressive resistance training (2–3 days/week)
Description: Light weights or bands for major muscle groups.
Purpose: Preserve lean mass and bone; reduce fatigue.
Mechanism: Muscle protein synthesis increases with load; improves insulin sensitivity.
Benefits: Stronger body, better mood, improved treatment tolerance.
13) Moderate aerobic activity (most days)
Description: Brisk walking or cycling 20–30 minutes as tolerated.
Purpose: Boost stamina and heart-lung health.
Mechanism: Improves mitochondrial function and oxygen use; reduces inflammation.
Benefits: More energy, better sleep, less anxiety.
14) Fatigue management and energy conservation
Description: Plan tasks, pace activities, schedule rests, and prioritize essentials.
Purpose: Control cancer-related fatigue.
Mechanism: Matches energy supply with demand; reduces “crash-and-burn” cycles.
Benefits: More predictable days; better quality of life.
15) Pelvic and core conditioning for posture & breath support
Description: Gentle core stability exercises and pelvic alignment work.
Purpose: Improve trunk stability that supports neck and breathing.
Mechanism: Engages diaphragm-core synergy; lowers compensatory neck strain.
Benefits: Less neck ache; smoother speech and breath.
16) Mindfulness-Based Stress Reduction (MBSR)
Description: Daily 10–20 minutes of guided mindfulness practice.
Purpose: Lower stress, fear of recurrence, and pain intensity.
Mechanism: Trains attention and reduces amygdala reactivity; shifts pain perception.
Benefits: Calmer mood, better sleep, improved coping.
17) Cognitive Behavioral Therapy (CBT) for cancer-related anxiety
Description: Structured short-term therapy reframing worry and catastrophic thoughts.
Purpose: Ease anxiety and depression.
Mechanism: Changes thought-behavior cycles; builds skills for uncertainty.
Benefits: Better mood, clearer decisions, higher adherence.
18) Guided imagery and relaxation
Description: Audio scripts that pair breathing with soothing mental images.
Purpose: Reduce tension and anticipatory nausea.
Mechanism: Lowers sympathetic arousal; conditions calmer responses.
Benefits: Less distress during scans and treatments.
19) CBT-I (insomnia program) and sleep hygiene
Description: Regular sleep schedule, screen limits, and stimulus control.
Purpose: Fix sleep disturbance from pain or steroids.
Mechanism: Re-associates bed with sleep; strengthens circadian cues.
Benefits: Deeper sleep; better daytime energy.
20) Acceptance and Commitment Therapy (ACT)
Description: Values-based actions despite uncertainty.
Purpose: Improve quality of life even when disease is chronic.
Mechanism: Psychological flexibility reduces struggle with unchangeable facts.
Benefits: More engagement in meaningful activities.
21) Treatment education & shared decision-making (“gene-informed”)
Description: Clear teaching about stage, surgery, radiation, systemic options, and clinical trials (e.g., MYB/NOTCH-targeted research).
Purpose: Empower informed choices.
Mechanism: Replaces fear with knowledge; aligns care with goals.
Benefits: Greater satisfaction and adherence.
22) Nutrition counseling for dry mouth and swallowing
Description: Soft, moist, high-protein meal plan; safe liquid thickness if needed.
Purpose: Maintain weight and healing.
Mechanism: Texture modification reduces choking; protein and calories support repair.
Benefits: Steady weight, fewer hospital visits.
23) Xerostomia (dry mouth) self-care education
Description: Frequent sips, sugar-free xylitol gum, saliva substitutes, humidifier.
Purpose: Ease dryness after radiation.
Mechanism: Stimulates residual glands; coats mucosa.
Benefits: Better speech, taste, and oral comfort.
24) Speech and communication strategies
Description: Voice therapy, articulation drills, and assistive tools if needed.
Purpose: Maintain clear speech after palate or tongue therapy.
Mechanism: Strengthens remaining muscles; optimizes resonance.
Benefits: Clearer communication and confidence.
25) Vocational and social support planning
Description: Stepwise return-to-work plan, disclosure tips, and benefits counseling.
Purpose: Protect income and roles.
Mechanism: Breaks big goals into safe stages; reduces stress.
Benefits: Smoother return to normal life.
Drug treatments
1) Cisplatin (Platinum chemotherapy)
Class: Alkylating-like, DNA cross-linker.
Typical dose/time: Often 70–100 mg/m² IV every 3 weeks (varies).
Purpose: Cytotoxic control in advanced or recurrent disease; sometimes with 5-FU or radiation.
Mechanism: Cross-links DNA so cancer cells cannot divide.
Side effects: Nausea, kidney injury, hearing loss, neuropathy, low counts.
2) Carboplatin
Class: Platinum analog.
Typical dose: AUC 5–6 IV every 3–4 weeks.
Purpose: Alternative to cisplatin; often paired with paclitaxel.
Mechanism: DNA cross-links.
Side effects: Low counts, fatigue, less kidney/ear toxicity than cisplatin.
3) Paclitaxel
Class: Taxane.
Typical dose: 175 mg/m² IV every 3 weeks (or weekly low-dose regimens).
Purpose: Additive activity with platinum in salivary cancers.
Mechanism: Stabilizes microtubules, stops cell division.
Side effects: Neuropathy, low counts, hair loss.
4) Docetaxel
Class: Taxane.
Typical dose: 60–75 mg/m² IV every 3 weeks.
Purpose: Similar role when paclitaxel not suitable.
Mechanism: Microtubule stabilization.
Side effects: Low counts, fluid retention, mouth sores.
5) 5-Fluorouracil (5-FU) or Capecitabine
Class: Antimetabolite (capecitabine is oral prodrug of 5-FU).
Typical dose: 5-FU continuous IV infusion; capecitabine 1000–1250 mg/m² orally twice daily, 14 days on/7 off.
Purpose: Combine with platinum; palliative control.
Mechanism: Thymidylate synthase inhibition; faulty RNA/DNA.
Side effects: Mouth sores, diarrhea, hand-foot syndrome, low counts.
6) Lenvatinib
Class: Oral multi-kinase inhibitor (VEGFR, FGFR, etc.).
Typical dose: 20–24 mg orally once daily (adjust as needed).
Purpose: Targeted option with documented activity in ACC.
Mechanism: Blocks tumor blood vessel signaling and growth pathways.
Side effects: High blood pressure, fatigue, diarrhea, hand-foot skin reaction, proteinuria.
7) Axitinib
Class: VEGFR tyrosine kinase inhibitor.
Typical dose: 5 mg orally twice daily (titrate).
Purpose: Anti-angiogenic therapy for progressing disease.
Mechanism: Inhibits VEGF receptors to starve tumor vessels.
Side effects: Hypertension, diarrhea, fatigue, voice change.
8) Sorafenib or Sunitinib (selected cases)
Class: Multi-kinase inhibitors.
Typical dose: Sorafenib 400 mg twice daily; Sunitinib 37.5–50 mg daily (cyclic).
Purpose: Off-label use in some centers for ACC; consider trials.
Mechanism: Inhibits angiogenic and proliferative kinases.
Side effects: Hand-foot reaction, fatigue, hypertension, low counts.
9) Pembrolizumab (or Nivolumab)
Class: PD-1 checkpoint inhibitors.
Typical dose: Pembrolizumab 200 mg IV every 3 weeks (or 400 mg q6w).
Purpose: Immunotherapy; responses are uncommon but can occur, especially if PD-L1 positive or TMB high (rare in ACC).
Mechanism: Releases immune brakes on T-cells.
Side effects: Immune inflammation (thyroiditis, colitis, pneumonitis), fatigue.
10) Cetuximab (selected cases)
Class: EGFR monoclonal antibody.
Typical dose: 400 mg/m² loading, then 250 mg/m² weekly (varies).
Purpose: EGFR-targeted therapy; mixed evidence in ACC.
Mechanism: Blocks EGFR signaling.
Side effects: Acne-like rash, infusion reactions, magnesium loss.
11) Gabapentin (neuropathic pain)
Class: Anticonvulsant/neuropathic analgesic.
Typical dose: Start 100–300 mg at night; titrate to effect (e.g., 300–600 mg TID).
Purpose: Manage nerve-related pain from perineural invasion or surgery.
Mechanism: Modulates calcium channels and neurotransmitter release.
Side effects: Sleepiness, dizziness, swelling.
12) Duloxetine (neuropathic pain & mood)
Class: SNRI antidepressant.
Typical dose: 30–60 mg orally daily.
Purpose: Neuropathy relief and anxiety/depression support.
Mechanism: Boosts serotonin and norepinephrine in pain pathways.
Side effects: Nausea, dry mouth, sweating, insomnia.
13) Pilocarpine (dry mouth)
Class: Muscarinic agonist.
Typical dose: 5 mg orally three times daily.
Purpose: Stimulate saliva after radiation.
Mechanism: Activates M3 receptors in salivary glands.
Side effects: Sweating, flushing, urination frequency.
14) Amifostine (selected with RT/chemo)
Class: Cytoprotective agent.
Typical dose: IV before radiation/chemotherapy per protocol.
Purpose: Reduce dry mouth and tissue damage.
Mechanism: Free-radical scavenger in normal tissues.
Side effects: Low blood pressure, nausea.
15) Standard antiemetics (e.g., Ondansetron)
Class: 5-HT3 antagonist.
Typical dose: 8 mg orally/IV before chemo, then as needed.
Purpose: Prevent nausea and vomiting from therapy.
Mechanism: Blocks serotonin receptors in gut/brain.
Side effects: Headache, constipation, QT prolongation (rare).
Important: Some targeted pills interact with foods and supplements (e.g., grapefruit). Always clear every pill—prescription or herbal—with your oncology pharmacist.
Dietary molecular supplements
Discuss all supplements with your doctor; some interact with chemotherapy or TKIs.
1) Omega-3 fatty acids (EPA/DHA)
Dose: 1–2 g/day combined EPA+DHA with meals (if approved).
Function/mechanism: Anti-inflammatory lipid mediators; may support weight and reduce cachexia.
Note: Can increase bleeding risk with anticoagulants.
2) Vitamin D3
Dose: 1000–2000 IU/day (or as guided by blood levels).
Function: Supports bone, muscle, and immune regulation.
Mechanism: Nuclear receptor signaling; may improve mood and strength.
Caution: Avoid excess; monitor 25-OH vitamin D.
3) Curcumin (turmeric extract with piperine unless on TKIs)
Dose: 500–1000 mg/day standardized curcumin.
Function: Anti-inflammatory/antioxidant.
Mechanism: NF-κB modulation.
Caution: Drug interactions (especially with targeted agents); ask pharmacy.
4) Green tea extract (EGCG)
Dose: 200–300 mg EGCG/day.
Function: Antioxidant; may support fatigue reduction.
Mechanism: Polyphenol signaling.
Caution: Liver safety—use reputable products; avoid with bortezomib-like drugs.
5) Selenium
Dose: 100–200 mcg/day (if dietary intake is low).
Function: Antioxidant enzymes (glutathione peroxidase).
Mechanism: Redox balance supporting mucosal health.
Caution: Narrow safety window—avoid excess.
6) Zinc
Dose: 10–25 mg elemental zinc/day for limited periods.
Function: Taste recovery, wound healing.
Mechanism: Enzyme cofactor in epithelial repair.
Caution: Too much lowers copper—limit duration.
7) Probiotics (evidence varies)
Dose: Per label (e.g., Lactobacillus/Bifidobacterium).
Function: Gut comfort during therapy.
Mechanism: Microbiome modulation.
Caution: Avoid in severe neutropenia unless approved.
8) Whey protein or medical nutrition shakes
Dose: 20–30 g protein per serving to meet daily needs.
Function: Preserve lean mass and healing.
Mechanism: Amino acids stimulate muscle protein synthesis.
Caution: Adjust if renal issues.
9) L-Glutamine (for mucositis support; mixed data)
Dose: 10 g powder dissolved, up to TID short term as advised.
Function: Fuel for enterocytes; may ease mouth sores.
Mechanism: Supports mucosal repair.
Caution: Clear with team; avoid if contraindicated.
10) Coenzyme Q10
Dose: 100–200 mg/day with fat-containing meal.
Function: Mitochondrial support; may reduce fatigue.
Mechanism: Electron transport and antioxidant effects.
Caution: Possible warfarin interaction.
Immunity-booster / regenerative / stem-cell–related” drugs
1) Filgrastim (G-CSF)
Dose: ~5 mcg/kg/day subcutaneously during neutropenia per protocol.
Function/mechanism: Stimulates bone marrow to make neutrophils; reduces infection risk after chemo.
2) Pegfilgrastim (long-acting G-CSF)
Dose: Single 6 mg shot per chemo cycle (timing varies).
Function: Same as above with longer action; convenient once-per-cycle dosing.
3) Epoetin alfa or Darbepoetin
Dose: Per anemia protocols when appropriate.
Function: Stimulates red blood cell production to reduce transfusions.
Note: Use is restricted; risks and benefits reviewed carefully.
4) Romiplostim or Eltrombopag
Dose: Titrated to platelet counts.
Function: Increases platelets via thrombopoietin receptor stimulation; lowers bleeding risk when counts are low.
5) Palifermin (keratinocyte growth factor)
Dose: Peri-treatment courses when high mucositis risk.
Function: Protects mucosa during intensive therapy; speeds healing.
6) Mesenchymal stem-cell or cell-based therapies (experimental)
Dose: Research only.
Function: Investigational support for tissue healing; not standard for ACC treatment. Discuss only within clinical trials.
Surgeries
1) Wide local excision with margin control
Procedure: Surgeon removes the tumor plus a rim of normal tissue; may use intra-operative frozen sections.
Why: Best chance for local cure; reduces microscopic residual cancer.
2) Parotidectomy or submandibular gland excision
Procedure: Remove the involved salivary gland; preserve facial nerve if safe, or reconstruct if tumor involves the nerve.
Why: Primary treatment for gland ACC.
3) Maxillectomy or palate resection (minor salivary ACC)
Procedure: Remove part of the palate or maxilla; reconstruct with flaps or obturator.
Why: Achieve negative margins in sinus/palate sites.
4) Skull-base/perineural resection (selected cases)
Procedure: Multidisciplinary surgery to clear tumor tracking along nerves into skull base.
Why: Control pain and reduce local recurrence when feasible.
5) Tracheal/bronchial sleeve resection (airway ACC)
Procedure: Remove the involved airway segment and re-anastomose ends.
Why: Restore airflow and remove tumor while preserving lung.
Neck dissection is less common in ACC because lymph node spread is not frequent, but it may be done if nodes are involved.
Prevention tips
There is no proven way to fully prevent ACC. These tips reduce general cancer risks and protect health during and after treatment.
Do not use tobacco; avoid secondhand smoke.
Limit alcohol.
Use workplace protection if exposed to dusts, fumes, or solvents.
Protect head and neck from unnecessary radiation; keep a record of imaging.
Keep regular dental care and oral checks; report any painless mouth lumps.
Maintain a healthy weight and active lifestyle.
Eat a plant-forward diet rich in fruits, vegetables, whole grains, and lean protein.
Manage reflux and chronic sinus problems with your clinician.
Keep vaccinations up to date (flu, COVID-19, as advised).
Attend all follow-up visits; early detection of recurrence helps.
When to see doctors (red flags)
New or enlarging painless lump in the mouth, palate, tongue base, jaw, or neck.
Numbness, tingling, or weakness in the face or tongue.
Persistent hoarseness, cough, wheeze, or shortness of breath.
Pain that wakes you at night or persistent ear pain without ear disease.
Trouble swallowing, choking, or unexplained weight loss.
After treatment: any new pain, swelling, cough, bone ache, or neurological symptoms.
Fever, mouth sores, or bleeding if you are on chemotherapy or targeted therapy.
Any side effect that limits eating, drinking, or breathing.
What to eat and what to avoid
Eat more of:
Soft, moist, high-protein foods (eggs, yogurt, tofu, fish, tender chicken, legumes).
Smoothies with protein powder, nut butters, and berries.
Soups and stews with added olive oil for calories.
Scrambled eggs, oatmeal with milk, cottage cheese, avocado.
Plenty of water; consider oral rehydration if diarrhea.
Limit/avoid when symptomatic or on specific drugs:
- Very spicy, acidic, or rough foods if mouth is sore.
- Alcohol and alcohol-based mouthwashes (drying, irritating).
- Grapefruit and Seville orange (can affect several TKIs).
- St. John’s wort and high-dose antioxidants during chemo/RT (possible interactions).
- Hard, sharp foods if you have trismus or mucositis.
Frequently asked questions
1) Is ACC fast or slow?
ACC usually grows slowly but is persistent. It can return many years later. Long follow-up is essential.
2) Does ACC spread to lymph nodes?
Less often than other head-and-neck cancers. Lungs are the most common distant site.
3) What is the main treatment?
Surgery with clear margins plus radiation therapy to reduce local return.
4) Does chemotherapy work?
It can help control advanced disease and symptoms, especially when combined (e.g., platinum + taxane). It is not always dramatic.
5) Are there targeted drugs?
Yes. Multi-kinase inhibitors like lenvatinib or axitinib can help some patients. Trials for MYB/NOTCH pathways exist.
6) Does immunotherapy help?
Sometimes. Responses are less common in ACC than in some other cancers, but select patients can benefit.
7) Will I lose my voice or ability to eat?
Most people keep good function with modern surgery, radiation, and rehabilitation. Early therapy with SLP and dietitians helps a lot.
8) What about nerve pain?
ACC may follow nerves. Neuropathic pain medicines, nerve-glide therapy, and radiation can help.
9) How long will I be followed?
Often for life. Imaging and exams are spaced out over time but continue long term.
10) Can I work during treatment?
Many can, with adjustments. A stepwise return plan and fatigue management help.
11) Are dental visits safe?
Yes—and important. You may need fluoride trays and preventive care, especially after radiation.
12) Can I travel with lung metastases?
Often yes, if stable and cleared by your team. Bring records and medicines; avoid risky infections.
13) What about fertility and family planning?
Discuss before chemo or radiation. Sperm or egg preservation may be possible in some cases.
14) Should I join a clinical trial?
If available and you are eligible, trials are encouraged in ACC because it is rare and research is active.
15) What support is available?
Oncology social work, financial counseling, patient groups, rehabilitation services, and palliative care (symptom-focused support) are all useful.
Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.
The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members
Last Updated: September 08, 2025.
