“Cylindroid adenocarcinoma” is an older name for adenoid cystic carcinoma (ACC), a rare cancer that starts in gland-forming (secretory) tissues—most often the salivary glands of the head and neck, but it can also arise in the trachea/bronchi, lacrimal gland, breast, sinonasal tract, skin adnexal glands, and other sites. ACC grows slowly yet has a strong tendency to track along nerves (perineural invasion) and to metastasize late, so people can feel well for years even while the tumor spreads. Under the microscope, ACC shows cribriform (“Swiss-cheese”), tubular, or solid patterns; the solid pattern behaves more aggressively. A hallmark molecular driver is the MYB–NFIB gene fusion (or related MYBL1 alterations). NCBICancer AustraliaRadiopaediaPMCmodernpathology.org
Cylindroid adenocarcinoma is an older or alternate term for adenoid cystic carcinoma (ACC). Pathologists once used the word “cylindroma/cylindroid” for its cylinder-like, cribriform (sieve-like) patterns seen under the microscope. Modern classifications call it adenoid cystic carcinoma, and coding standards list “adenocarcinoma, cylindroid” as a synonym for ACC. In simple words: this is the same disease people now call adenoid cystic carcinoma. It grows slowly but can spread along nerves (perineural spread) and may return years later. It most commonly starts in the salivary glands (parotid, submandibular, minor salivary glands inside the mouth), but it can also arise in the lacrimal gland, trachea, larynx, breast, skin, and other secretory glands. The main treatment is surgery when possible, usually followed by radiotherapy to lower the chance of local return. Some cases need systemic therapy (targeted drugs or chemotherapy) if they spread or cannot be removed. Wikipedia+1My Cancer Genome
“Another names
Cylindroid adenocarcinoma is best known today as adenoid cystic carcinoma (ACC). Other historical or clinical synonyms include adenocarcinoma, cylindroid; adenocystic carcinoma; malignant cylindroma/cylindroma (not to be confused with benign cutaneous cylindroma); and adenocyst. The word “cylindroma” came from early descriptions of rounded (“cylindrical”) basement-membrane–like spaces seen in the classic cribriform pattern. Modern pathology and cancer coding systems list “adenocarcinoma, cylindroid” and “cylindroma” as synonyms for ACC. Ohio State College of MedicineNCBIWikipedia
Types
1) Histologic patterns.
Tubular and cribriform patterns generally behave more indolently.
Solid pattern (≥30% of the tumor) is “high grade” and predicts worse outcomes. ACS PublicationsScienceDirectFrontiers
2) Site-based types.
Major/minor salivary gland ACC (most common); tracheobronchial, sinonasal, lacrimal, breast, skin adnexal locations are well-described variants that share the same core biology. Cancer AustraliaPMC
3) Molecular subsets.
MYB–NFIB fusion–positive; MYBL1-rearranged; NOTCH-activated subsets—NOTCH1-mutant tumors often show more aggressive behavior. ScienceDirectPMC+1
Causes
In ACC, true lifestyle risk factors are not well established. The strongest “causes” are molecular drivers inside the tumor cells. Below are 20 contributors—some causal, some promoting growth/spread. I note the strength of evidence in simple terms.
MYB–NFIB fusion (key driver). A chromosome t(6;9) brings MYB under abnormal control, switching on many growth genes—the central event in most ACCs. Strong evidence. ScienceDirectPMC
MYBL1 rearrangements. A minority of tumors use MYBL1 (a MYB relative) instead of MYB to drive the same gene program. Strong evidence. PMC
MYB copy-number/enhancer activation. Some ACCs activate MYB without a fusion, by extra copies or hijacked enhancers. Strong evidence. Wikipedia
NOTCH1 pathway mutations. Define an aggressive subgroup; linked to solid histology and worse survival. Strong evidence. PMCScienceDirect+1
Low overall mutation burden with select hits. ACC genomes are relatively “quiet,” emphasizing the primacy of MYB/MYBL1 and a few pathways. Strong evidence. PMC
c-KIT (CD117) signaling. Frequent expression; useful diagnostically and may support tumor cell survival. Moderate evidence. PMCNature
Perineural niche signaling. Tumor–nerve crosstalk helps ACC grow along nerves (pain, numbness, spread). Strong evidence for behavior; mechanism still under study. PMCIIAR Journals
Angiogenesis and stromal support. Tumor micro-vessels and supportive stroma sustain slow but persistent growth. Moderate evidence. PMC
Epigenetic dysregulation. Abnormal gene-switching (methylation/chromatin) cooperates with MYB programs. Moderate evidence. PMC
PI3K/AKT and other survival pathways. Sporadically altered; may contribute to progression. Emerging evidence. PMC
Apoptosis resistance. MYB-driven networks dampen cell death signals, letting cells survive therapy. Moderate evidence. PMC
EMT-like changes and invasion genes. Programs that loosen cell adhesion favor local infiltration. Moderate evidence. PMC
Neurotrophic factors/chemokines. Cytokines along nerves (e.g., in head & neck) can attract tumor cells to nerve sheaths. Emerging evidence. PMC
High-grade transformation (HGT). Some ACCs “dedifferentiate” into a more aggressive form, often with new genetic hits. Strong clinicopathologic evidence. PMCScienceDirect
Age-related DNA damage. Like many cancers, mutations accumulate slowly with age, facilitating tumor start. General cancer principle; consistent with ACC’s age profile. PMC
Prior therapeutic radiation (rare pathway). Radiation can cause DNA breaks that, rarely, give rise to salivary gland cancers, including ACC. Limited but plausible evidence. PMC
Chronic glandular injury/inflammation (theory). Long-standing irritation may create a permissive microenvironment. Hypothesis; not a proven ACC-specific risk. PMC
Immune evasion. Low mutation burden and immune-cold milieu may help ACC escape immune detection. Emerging evidence. PMC
Stem-like cell programs. MYB and c-KIT have been linked to stemness features that sustain tumor self-renewal. Moderate evidence. Nature
Site-specific factors. Local ducts/nerve richness (e.g., in minor salivary glands, lacrimal gland) provide paths for spread and persistence. Clinicopathologic correlation. WebPathologyCancer Australia
Symptoms
Painless, firm lump in a salivary gland (palate, floor of mouth, cheek) that grows slowly over months or years. This is typical early ACC. NCBI
Deep, burning or electric pain as the tumor tracks along nerves (perineural invasion). Pain may precede a visible mass. PMC
Numbness or tingling in the face, palate, tongue, or jaw because nerves are involved. PMC
Facial muscle weakness or asymmetry if the facial nerve is affected. NCBI
Difficulty chewing or opening the mouth (trismus) when the mass involves masticator spaces. NCBI
Swallowing trouble (dysphagia) or throat fullness with oropharyngeal/minor salivary involvement. NCBI
Hoarseness, cough, or breathlessness when ACC arises in the trachea/bronchus. Cancer Australia
Nasal blockage, nosebleeds, or face pain/pressure with sinonasal ACC. Cancer Australia
Watery eye, bulging eye (proptosis), double vision, or eyelid droop when the lacrimal gland or orbital nerves are involved. Cancer Australia
Breast lump that is usually not very painful, sometimes found incidentally on imaging. PMC
Skin or subcutaneous nodules when ACC arises from sweat glands; lesions are often firm and slow growing. Cancer Australia
Loose teeth or jaw discomfort if the palate or alveolar ridge is involved. NCBI
Ear pain (referred otalgia) from deep head-and-neck nerve involvement. NCBI
Unintended weight loss or fatigue in advanced/metastatic disease. National Organization for Rare Disorders
Symptoms from distant spread (often lungs first): persistent cough, shortness of breath, or bone pain. NCBI
Diagnostic tests
Physical examination
Comprehensive head-and-neck exam. The clinician inspects and feels the mouth, palate, tongue, floor of mouth, and cheeks to find firm, fixed lumps and to map tenderness. This bedside exam guides the whole work-up. NCBI
Bimanual palpation of salivary glands and floor of mouth. One hand inside and one outside the mouth helps outline deep masses and their mobility. It also detects duct blockage. NCBI
Cranial nerve examination. Testing facial movement, facial sensation, eye movements, palate elevation, tongue motion, and swallowing looks for perineural involvement. NCBI
Neck examination for lymph nodes. Though nodal spread is less frequent in ACC than in some cancers, enlarged or firm nodes may change staging. NCBI
Site-specific exams. Nasal endoscopy for sinonasal masses, gentle breast exam for breast lesions, and focused eye/orbit exam for lacrimal gland disease document local extension. Cancer Australia
“Manual”/bedside procedures
Flexible naso-/laryngoscopy. A thin camera looks at hidden parts of the nose, nasopharynx, and larynx, defining the visible tumor and airway safety in clinic. Radiopaedia
House–Brackmann facial grading. A simple scale scores facial nerve weakness to track change over time and to plan imaging and surgery. NCBI
In-office palpation-guided fine needle aspiration (FNA) setup. While the diagnosis requires cytology/histology (below), careful palpation ensures safe sampling of deep minor-gland lesions. NCBI
Laboratory & pathological tests
Fine-needle aspiration (FNA) cytology. Uses a thin needle to collect cells. Cytology can suggest ACC (basaloid cells, hyaline globules) and guides next tests. NCBI
Core needle or incisional biopsy. Provides solid tissue so the pathologist can see cribriform, tubular, or solid architecture and assess grade. ACS Publications
Definitive histopathology. The microscope shows the hallmark “Swiss-cheese”/cribriform pattern with basement-membrane–like material; solid areas indicate higher grade. Ohio State College of MedicineScienceDirect
Immunohistochemistry (IHC). ACC often expresses c-KIT (CD117) and shows myoepithelial-type markers (e.g., S100, SMA, p63); IHC helps distinguish ACC from look-alikes. PMC
Molecular testing (FISH/RT-PCR/NGS) for MYB/MYBL1 fusions. Confirms the ACC signature and can help in tough cases or small biopsies. ScienceDirectPMC
Assessment for perineural invasion and margins on surgical specimens. These features guide radiotherapy decisions and prognosis. PMC
NOTCH1 testing (where available). Identifies a higher-risk molecular subgroup that may enroll in trials of NOTCH-pathway drugs. PMC
Electrodiagnostic tests
Facial nerve EMG/ENoG (selected cases). If facial weakness is present, these studies measure nerve function and degeneration, complementing MRI for perineural spread. NCBI
Swallowing electrophysiology or laryngeal EMG (selected cases). When voice or swallow is affected, these tests localize neuromuscular involvement to plan therapy. NCBI
Imaging tests
MRI with contrast of the primary region and skull base. The most informative study for ACC because it shows soft tissue and perineural spread along named nerves toward the skull base. Radiopaedia
CT scan with contrast of head/neck and chest. Defines bone/airway involvement and screens lungs—the most common site of distant spread. Radiopaedia
FDG-PET/CT (selected situations). Surveys the whole body for metastatic disease or locates an occult primary when pathology reads “ACC.” Radiopaedia
Non-pharmacological treatments
1) Jaw-opening therapy for trismus (physiotherapy)
Description: Gentle, repeated mouth-opening exercises with devices (e.g., stacked tongue depressors or commercial jaw stretchers) prevent or treat tightness after surgery or radiotherapy. A therapist teaches posture, frequency, and how to avoid pain flares.
Purpose: Keep jaw motion, allow eating, brushing teeth, and dental care.
Mechanism: Regular low-load prolonged stretch remodels tight muscles and connective tissue and reduces fibrosis.
Benefits: Better mouth opening, less pain, easier nutrition and speech, lower risk of long-term lockjaw.
2) Neck and shoulder mobility program (physiotherapy)
Description: After parotid or neck surgery, stiffness and shoulder droop can develop. A tailored plan includes range-of-motion drills, scapular setting, and progressive strengthening.
Purpose: Restore normal posture, neck turn, and shoulder use.
Mechanism: Gradual loading improves muscle strength and tendon glide; lymph flow and scar mobility also improve.
Benefits: Less pain, better overhead reach, improved quality of life.
3) Scar and soft-tissue mobilization (physiotherapy)
Description: Trained therapists use massage, myofascial release, and desensitization over surgical scars and irradiated skin.
Purpose: Reduce tight bands, numbness/tingling, and restricted movement.
Mechanism: Mechanical input realigns collagen, improves microcirculation, and calms nerve over-sensitization.
Benefits: Softer scars, better mobility, less itch or pull.
4) Swallow therapy (dysphagia rehabilitation) (physiotherapy/speech therapy)
Description: An SLP (speech-language pathologist) teaches safe-swallow maneuvers, texture modifications, and exercises (e.g., effortful swallow, Mendelsohn).
Purpose: Maintain safe, efficient swallowing during and after treatment.
Mechanism: Neuromuscular re-education improves timing and strength of swallowing muscles.
Benefits: Fewer choking events, better nutrition, lower aspiration risk.
5) Speech/voice therapy (physiotherapy/speech therapy)
Description: Voice hygiene, resonant voice therapy, and compensatory articulation for those with laryngeal or palatal involvement.
Purpose: Clearer speech and safer voice use.
Mechanism: Targets efficient vibration and breath support; strengthens compensatory pathways.
Benefits: Improved intelligibility and social participation.
6) Lymphedema management (physiotherapy)
Description: Manual lymphatic drainage, compression garments, and posture/breathing drills to reduce head-and-neck swelling.
Purpose: Control edema that can follow lymph node surgery or radiation.
Mechanism: External pressure and manual routing encourage alternate lymph pathways.
Benefits: Slimmer neck/face, less heaviness, better swallowing.
7) Posture and cervical stabilization (physiotherapy)
Description: Ergonomic coaching and deep neck flexor work to counter forward-head posture and muscle imbalance.
Purpose: Reduce neck pain and headaches; support airway and swallow.
Mechanism: Motor control retraining and endurance building.
Benefits: Less pain and fatigue; better function.
8) Aerobic conditioning (physiotherapy/exercise oncology)
Description: Walking or cycling 3–5 days/week at moderate intensity, individualized to symptoms and treatment stage.
Purpose: Fight cancer-related fatigue and maintain heart-lung fitness.
Mechanism: Mitochondrial and cardiovascular adaptations improve oxygen use.
Benefits: More energy, better mood and sleep, improved treatment tolerance.
9) Progressive resistance training (physiotherapy)
Description: 2–3 sessions/week focusing on major muscle groups using bands or weights.
Purpose: Prevent muscle loss, improve function.
Mechanism: Muscle protein synthesis and neuromuscular recruitment.
Benefits: Strength, balance, independence.
10) Breathing and airway clearance (physiotherapy)
Description: Diaphragmatic breathing, huff coughing, and incentive spirometry if lungs are affected or post-op.
Purpose: Preserve lung function, reduce infections.
Mechanism: Better ventilation and mucus clearance.
Benefits: Less breathlessness, fewer chest infections.
11) Neuropathic pain self-management (physiotherapy/OT)
Description: Desensitization, graded motor imagery, gentle neural glides.
Purpose: Ease nerve-related pain from perineural spread or treatment.
Mechanism: Normalizes nerve mobility and central pain processing.
Benefits: Less burning/tingling, better sleep.
12) Balance and fall-prevention (physiotherapy)
Description: Vestibular drills, footwork, and home safety review.
Purpose: Reduce falls if weakness or neuropathy is present.
Mechanism: Improves sensory integration and reflexes.
Benefits: Greater confidence and safety.
13) Oral hygiene and dental care coaching (physiotherapy/OT collaboration)
Description: Daily fluoride, saliva substitutes, soft-brush technique, and dental visits.
Purpose: Prevent cavities and osteoradionecrosis risk after radiation.
Mechanism: Protects enamel and supports salivary function.
Benefits: Fewer dental complications and infections.
14) Skin care after radiotherapy (physiotherapy/oncology nursing)
Description: Gentle cleansing, moisturizers, sun protection, and early wound care for radiated skin.
Purpose: Maintain skin barrier and comfort.
Mechanism: Supports epidermal repair and reduces friction injury.
Benefits: Less irritation and breakdown.
15) Cancer prehabilitation (physiotherapy multidisciplinary)
Description: Exercise, nutrition, and mental health support started before surgery or radiotherapy.
Purpose: Enter treatment stronger to recover faster.
Mechanism: Builds reserves and reduces inflammation.
Benefits: Shorter hospital stays and better outcomes.
Mind-Body & Educational Therapies
16) Mindfulness-based stress reduction — reduces anxiety, improves sleep by training attention and calm breathing.
17) Cognitive-behavioral therapy (CBT) — tools to reframe worry and manage pain/fatigue behaviors.
18) Guided imagery/relaxation audio — lowers autonomic arousal; helpful before scans or radiotherapy.
19) Yoga or tai chi (gentle forms) — combines movement, balance, and breath; improves fatigue and mood.
20) Meaning-centered therapy — supports purpose and coping with a rare cancer.
21) Patient education sessions — clear teaching about treatment plan, side effects, and red-flag symptoms.
22) Nutrition counseling — protein/energy planning, texture advice, hydration strategies.
23) Smoking and betel-quid cessation support — lowers general head-neck cancer risks and treatment complications.
24) Sleep hygiene program — regular schedule, light control, and stimulus control.
25) Peer support group or one-to-one navigation — practical tips and emotional support from others with ACC.
(Evidence notes: These supportive measures are standard in head-and-neck oncology rehab and survivorship. They complement—not replace—medical treatments.)
Drug treatments
Important safety note: exact drug choices and dosing are individualized by your oncology team based on your health, site, prior therapy, and clinical trials. The following are commonly discussed options with typical dosing references; many aim to stabilize disease more than shrink it.
1) Lenvatinib (VEGFR/FGFR inhibitor; targeted therapy)
Class/Purpose: Multi-kinase inhibitor that blocks tumor blood-vessel signaling; used for progressive recurrent/metastatic ACC.
Typical dose/time: 24 mg orally once daily (dose reductions common).
Mechanism: Inhibits VEGFR1-3, FGFR, and others, starving tumors of blood supply and signaling.
Side effects: Hypertension, fatigue, diarrhea, hand-foot skin reaction, proteinuria. ASCO PublicationsPMC
2) Axitinib (VEGFR inhibitor; targeted)
Dose/time: 5 mg orally twice daily (adjust as tolerated).
Mechanism/benefit: VEGFR blockade; phase II studies show objective responses in a subset and prolonged stability in others.
Side effects: Hypertension, diarrhea, fatigue, dysphonia. PMC
3) Apatinib / other VEGFR TKIs (investigational/variable access)
Dose: Commonly 500 mg daily in studies (country-specific).
Mechanism/benefit: VEGFR2 blockade; reports suggest disease control in ACC cohorts.
Side effects: Similar VEGF-pathway toxicities. ScienceDirect
4) Pazopanib (VEGFR/PDGFR inhibitor)
Dose: 800 mg orally daily.
Mechanism: Anti-angiogenic; may stabilize disease.
Side effects: Hepatotoxicity, hypertension, diarrhea, hair color change. JAMA Network
5) Sorafenib (multi-kinase inhibitor)
Dose: 400 mg orally twice daily.
Mechanism/benefit: Anti-angiogenic/RAF pathway blockade; disease stabilization seen in small series.
Side effects: Hand-foot reaction, rash, hypertension. JAMA Network
6) Sunitinib / Regorafenib (multi-kinase inhibitors)
Dose: Sunitinib 37.5–50 mg daily (schedule varies); Regorafenib 160 mg daily (3 weeks on/1 week off).
Mechanism: Anti-angiogenic; mixed results but sometimes useful for stabilization.
Side effects: Fatigue, hand-foot reaction, mucositis. JAMA Network
7) CAP regimen (Cyclophosphamide + Doxorubicin + Cisplatin; chemotherapy)
Dose (typical cycle q3 weeks): Cyclophosphamide 500–600 mg/m² IV day 1; Doxorubicin 50–60 mg/m² IV day 1; Cisplatin 60–100 mg/m² IV day 1 (varies by protocol).
Mechanism: Cytotoxic combination; among the more “active” regimens historically in salivary cancers; responses are modest and side effects significant.
Side effects: Fatigue, nausea, low blood counts, kidney/heart risks (monitoring required). PubMed+1Annals of Oncology
8) Cisplatin + 5-FU (chemotherapy)
Dose: Cisplatin 80–100 mg/m² day 1 + 5-FU 1,000 mg/m²/day as 96-hour infusion (cycle q3–4 weeks).
Mechanism: DNA damage + antimetabolite; sometimes used for palliation.
Side effects: Nausea, mucositis, myelosuppression (supportive care needed). Chinese Clinical Oncology
9) Carboplatin + Paclitaxel (chemotherapy)
Dose: Carboplatin AUC 5–6 day 1 + Paclitaxel 175 mg/m² day 1 q3 weeks (or weekly variants).
Mechanism: Microtubule + DNA cross-linking; palliative control in selected patients.
Side effects: Neuropathy, neutropenia, fatigue. (Salivary cancer practice patterns vary.) NCBI
10) Pembrolizumab (immunotherapy; PD-1 inhibitor)
Dose: 200 mg IV q3 weeks or 400 mg IV q6 weeks.
Mechanism: Re-activates T-cells; responses in ACC are uncommon, with disease stabilization more typical; combining with focal RT has not clearly improved systemic response.
Side effects: Immune-related (thyroiditis, colitis, hepatitis) that need prompt care. PMCPubMed
11) Nivolumab (PD-1 inhibitor)
Dose: 240 mg IV q2 weeks or 480 mg q4 weeks.
Mechanism/benefit: Similar to pembrolizumab; limited objective responses in ACC but can stabilize disease in some.
Side effects: Immune-related toxicities. NCBI
12) Cetuximab (EGFR antibody) ± platinum/5-FU
Dose: 400 mg/m² loading then 250 mg/m² weekly (or biweekly regimens).
Mechanism: EGFR blockade; activity in ACC is limited and not routine unless part of a broader plan.
Side effects: Acneiform rash, infusion reactions. NCBI
13) Bevacizumab combinations (anti-VEGF antibody; selected cases/trials)
Dose: 5–10 mg/kg IV q2–3 weeks with chemo or TKIs in studies.
Mechanism: Anti-angiogenic; used in experimental settings.
Side effects: Hypertension, bleeding risk, wound-healing delay. JAMA Network
14) Interferon-alpha (rare, selected)
Mechanism: Immune modulation and anti-proliferative effects; historical use in salivary cancers is limited today.
Side effects: Flu-like symptoms, mood changes. NCBI
15) NOTCH-pathway inhibitors (e.g., AL101/γ-secretase inhibitors; clinical trials)
Dose: Per trial protocol.
Mechanism: Targets tumors with NOTCH1 activation—an aggressive molecular subtype.
Side effects: Diarrhea, fatigue, electrolyte changes; use is investigational. Nature
(Ask your oncologist about clinical trial availability; many promising agents are only in studies.)
Dietary molecular “supplements
Important: No supplement has proven to cure ACC. Always discuss supplements with your oncology team to avoid drug interactions, especially with VEGFR inhibitors and chemotherapy.
High-protein medical nutrition shakes — support muscle and wound healing; dose: 1–2 servings/day as needed.
Omega-3 fatty acids (fish oil) — may help cancer-related weight loss and inflammation; common dose 1–2 g EPA+DHA/day; monitor with anticoagulants.
Vitamin D — correct deficiency for bone and immune health; dose per blood level (often 800–2000 IU/day).
Probiotics — may help antibiotic-related diarrhea; choose clinically tested strains; avoid if severely immunosuppressed.
Ginger extract — can reduce nausea; 0.5–1 g/day in divided doses.
Curcumin (turmeric extract) — anti-inflammatory; potential CYP interactions; 500–1000 mg/day with medical oversight.
Green tea extract (EGCG) — antioxidant; possible interaction with some TKIs; use cautiously.
Magnesium — replaces losses from diarrhea; dose per labs; excessive amounts can cause diarrhea.
Selenium — antioxidant support at nutritional doses only (e.g., 50–100 mcg/day); avoid high doses.
B-complex (esp. B6) — may help certain neuropathic symptoms at modest doses; avoid excessive dosing.
(These are generic oncology-supportive options; evidence quality varies and is not ACC-specific.)
Immunity booster / regenerative / stem-cell” drugs
There are no approved “stem-cell drugs” to treat ACC itself. However, your team may use supportive biologic agents to protect you during intensive treatments:
Filgrastim (G-CSF) — boosts white cells after chemo; typical 5 mcg/kg/day SC until ANC recovers.
Pegfilgrastim — long-acting G-CSF; single 6 mg SC dose per chemo cycle, given ≥24 hours after chemo.
Epoetin alfa / darbepoetin — for selected chemo-induced anemia to reduce transfusions (used carefully per guidelines).
Romiplostim / eltrombopag — for certain low-platelet states to support chemotherapy (specialist-directed).
Palifermin — keratinocyte growth factor to reduce severe mouth sores in high-risk regimens (specific indications).
Seasonal vaccines (influenza, COVID-19) — not drugs for ACC, but important immune protection during therapy.
Why this list? These agents do not treat the tumor, but they help your body tolerate treatment and lower infection or mucosal-injury risks. Always used under oncology guidance. (General supportive-care standards.)
Surgeries
1) Wide local excision of primary tumor
Procedure: Remove the tumor with a rim of healthy tissue; may include bone shaving or resection if invaded.
Why: Best chance for local control when feasible.
2) Parotidectomy or submandibular gland excision
Procedure: Remove the affected major salivary gland; preserve facial nerve if oncologically safe.
Why: Standard for gland-origin ACC.
3) Neck dissection (selective)
Procedure: Remove lymph nodes at risk (ACC has lower nodal rates than many cancers, but selected dissections are done based on site/stage).
Why: Staging and control when nodes are clinically involved or high-risk.
4) Skull base/perineural corridor surgery
Procedure: Multidisciplinary approach to remove tumor tracking along nerves toward the skull base.
Why: ACC commonly spreads along nerves; complete clearance may improve control.
5) Tracheal or laryngeal segment resection and reconstruction (site-specific)
Procedure: Remove involved airway segment with reconstruction.
Why: Restore breathing/voice and remove disease in airway ACC.
Most patients with significant risk features receive postoperative radiotherapy to reduce local recurrence. PMCASCO Publications
Prevention tips
Do not smoke; stop all tobacco and betel-quid.
Limit alcohol.
Protect your skin and scars from sun after radiotherapy.
Keep excellent oral hygiene and see a dentist experienced with head-neck radiation.
Maintain good nutrition and protein intake.
Stay physically active within your limits.
Keep vaccinations up to date (flu/COVID-19 per local guidance).
Follow swallow and jaw-opening exercises to prevent long-term dysfunction.
Attend regular follow-ups and imaging—ACC can recur late.
Ask about clinical trials early if disease returns.
When to see a doctor urgently
New or growing lump in the mouth, jaw, face, neck, or near the eye.
New facial numbness, tingling, or weakness.
Worsening trouble swallowing, persistent hoarseness, or cough.
Unexplained weight loss, fatigue, or bone pain.
Any red-flag symptoms after treatment: fever, uncontrolled pain, bleeding, severe mouth sores, dehydration, shortness of breath, or signs of infection. (Seek emergency care when severe.)
What to eat and what to avoid
Eat more: 1) Soft, high-protein foods (eggs, yogurt, dal, fish) for healing. 2) Healthy fats (olive oil, nut butters) to maintain weight. 3) Moist foods with sauces if dry mouth. 4) High-calorie smoothies when appetite is low. 5) Plenty of fluids (water, oral rehydration).
Limit/avoid: 6) Very spicy, acidic, or rough foods during mucositis. 7) Alcohol (irritates mucosa, interacts with meds). 8) Tobacco/betel quid. 9) Herbals that interact with TKIs (e.g., St. John’s wort; always clear supplements with your oncologist). 10) Grapefruit with certain TKIs (may raise drug levels—ask your pharmacist).
Frequently asked questions (FAQs)
1) Is cylindroid adenocarcinoma the same as adenoid cystic carcinoma?
Yes. “Cylindroid adenocarcinoma” is an older/synonymous label for ACC (also called malignant cylindroma). Modern sources use adenoid cystic carcinoma. Wikipedia+1
2) Where does it usually start?
Most often in salivary glands, but it can also arise in other secretory glands such as the lacrimal gland, trachea, and larynx. My Cancer Genome
3) Why does it come back years later?
ACC grows slowly and can spread along nerves or seed the lungs microscopically. Late recurrences are a known feature, so long-term follow-up is essential. Wikipedia
4) What is the main treatment when it is localized?
Surgery with clear margins, usually followed by postoperative radiotherapy if risk factors are present. ASCO Publications
5) Does chemotherapy cure ACC?
No. Traditional chemotherapy may shrink or stabilize tumors in some patients, but it is not curative for metastatic ACC. Annals of Oncology
6) Which newer drugs look promising?
VEGFR-targeted TKIs like lenvatinib and axitinib have shown the most consistent disease control in trials; responses occur in a minority but many patients experience stable disease. ASCO PublicationsPMC
7) Does immunotherapy help?
So far, pembrolizumab and similar drugs have low response rates in ACC; some patients experience stability rather than shrinkage. PMC
8) What makes some ACCs more aggressive?
Tumors with NOTCH1 activation and a solid histologic pattern tend to behave more aggressively. ASCO PublicationsWikipedia
9) Are there blood markers to track ACC?
No validated serum tumor markers exist; doctors rely on exams and imaging.
10) What imaging is best to check nerve spread?
MRI with contrast is best for mapping perineural pathways and skull base involvement. PubMed
11) Which site does it spread to most often?
The lungs are the most common distant site; bone, brain, and liver can also be involved. Wikipedia
12) Should everyone get genetic testing of the tumor?
Molecular testing (e.g., MYB/MYBL1, NOTCH1) is helpful for diagnosis refinement, prognosis, and trial matching. PMC
13) Is lymph-node spread common?
Less common than in many head-and-neck cancers, but nodes are still evaluated and treated when involved. Wikipedia
14) What if surgery is not possible?
Definitive radiotherapy (including advanced techniques) can control the primary site; systemic therapy and trials are considered. PMC
15) Are there clinical trials for ACC?
Yes—especially for VEGFR inhibitors, NOTCH inhibitors, and other targeted approaches. Ask your oncologist about current trials in your region. JAMA Network
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.
