VERY-RARE CANCERSalivary Gland CancerWHO 5th Edition Classification

Adenoid Cystic Carcinoma (ACC)

Very Rare Malignancy (1–5 cases per 1,000,000 / year) • Clinical Staging, Genomic Targets & Vetted Specialists

A slow-growing salivary gland cancer notorious for spreading along nerves and for recurring or metastasising many years after apparently successful treatment. It also arises in the lacrimal gland, breast, trachea and other secretory sites. Lung metastases can remain stable for years.

Request Rare Cancer Tumor Board Find NCI Treatment CentersAsk Vani AI Navigator

Emergent Referral AdvisoryImmediate Action

Certain rare malignancies progress rapidly or carry acute risk of airway obstruction, acute spinal compression, hydrocephalus, or biopsy-seeding. Do not perform needle biopsy or surgery outside of an NCI-designated specialty sarcoma or neuro-oncology unit without multidisciplinary tumor board review.

Report immediately to an emergency department or tertiary oncologist upon:

  • Facial nerve weakness with a salivary mass - assume malignancy until proven otherwise
Who It Affects

Adults, median 50-60; slight female predominance

Annual Incidence

Approximately 3-4.5 per million per year

Clinical Evidence Review

Last audited: 2026-08-13 against NCCN, ESMO, and WHO 5th ed. guidelines.

Clinical Presentation & Hallmark Symptoms

Presenting signs most frequently observed across clinical case series

A slowly enlarging, often painless lump in the parotid, submandibular or palate region
Persistent facial numbness, tingling or weakness - a hallmark of perineural spread
Pain out of proportion to the size of the lump
Nasal obstruction or hoarseness depending on the site
Difficulty swallowing with pharyngeal involvement

Genomic Profiling & Defining Molecular Lesions

Critical diagnostic fusions, somatic mutations, and therapeutic targets

Rare malignancies frequently depend on distinct oncogenic drivers rather than conventional environmental carcinogens. Comprehensive Next-Generation Sequencing (NGS comprehensive panel) and FISH/IHC are mandatory to establish the true diagnosis and screen for basket trial agents.

MYB-NFIB fusion, t(6;9)MYBL1-NFIB - variantNOTCH1 activating mutations

Standard-of-Care Treatment Protocol

Frontline and multimodal strategies established under international consensus guidelines

1

Wide surgical resection with nerve assessment

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

2

Postoperative radiotherapy, including neutron, proton or carbon ion where available

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

3

Radiotherapy covering named nerve pathways to the skull base

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

4

Prolonged active surveillance of indolent lung metastases

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

5

Lenvatinib, axitinib and other antiangiogenic TKIs in progressive disease

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

Clinical Trial Advisory: For rare and ultra-rare malignancies, enrollment in an active clinical trial or expanded-access program is widely considered the preferred standard of care by ASCO and NCCN panels.

Prognosis & Disease Trajectory

Objective clinical outlook without false reassurance

A long disease. Five-year survival is relatively good at around 70-80%, but fifteen- and twenty-year survival fall substantially because of late recurrence. Solid histological subtype and perineural invasion predict worse outcome. Surveillance must extend for decades.

Note: Statistics reflect cohort averages. Individual outcomes depend heavily on performance status, resectability, biomarker expression, and timely access to specialized tertiary care.

Active Research, Biomarkers & Clinical Trials

Novel investigational agents, phase I/II trials, and international rare disease consortia

MYB/MYBL1-NFIB fusion targeting, NOTCH1 inhibitors for the NOTCH-activated aggressive subset, and antiangiogenic combinations are under study.

Need Help Matching to an Active Rare Cancer Trial?

ByOnco scans ClinicalTrials.gov, NCI trial networks, and institutional registries for open patient cohorts.

Request Trial Match
Expert Clinicians

Verified Specialists for Adenoid Cystic Carcinoma

Browse All Oncologists

Dr. Alan L. Ho

Medical Oncologist

15+ yrs exp

MSKCC

New York, USA

Head & Neck OncologyAdenoid Cystic CarcinomaTargeted Agents
Vetted DirectoryConsult Specialist

Dr. Gopal Iyer

Head & Neck Surgeon

20+ yrs exp

SingHealth

Singapore, Singapore

Head & Neck SurgerySalivary Gland TumorsMolecular Targets
Vetted DirectoryConsult Specialist

Prof. Laura V. Locati

Medical Oncologist

20+ yrs exp

Istituto Nazionale Tumori

Milan, Italy

Salivary Gland CancersAdenoid Cystic CarcinomaEuropean Guidelines
Vetted DirectoryConsult Specialist

Dr. Pankaj Chaturvedi

Head & Neck Surgical Oncologist

25+ yrs exp

Tata Memorial

Mumbai, India

Head & Neck SurgeryAdenoid Cystic CarcinomaPostoperative Radiotherapy
Vetted DirectoryConsult Specialist
Hospital Network

Designated Cancer Centers with Dedicated Programs

Search All 72 NCI Centers

Memorial Sloan Kettering Cancer Center

NY

NCI-Designated Comprehensive Cancer Center

World-leading rare tumor board & pediatric solid tumor protocols

Proton TherapyCAR-T Cell TherapyRobotic Surgery
1,000+ Active Clinical TrialsView Center Profile

University of Texas MD Anderson Cancer Center

TX

NCI-Designated Comprehensive Cancer Center

Largest specialized rare cancer and sarcoma multidisciplinary program

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
1,200+ Active Clinical TrialsView Center Profile

Dana-Farber / Harvard Cancer Center

MA

NCI-Designated Comprehensive Cancer Center

Pioneering genomic molecular tumor boards & rare histologies

Proton TherapyCAR-T Cell TherapyPhase I Unit
1,100+ Active Clinical TrialsView Center Profile

Mayo Clinic Comprehensive Cancer Center

MN

NCI-Designated Comprehensive Cancer Center

High-volume surgical oncology & rare endocrine/neuroendocrine expertise

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
600+ Active Clinical TrialsView Center Profile

Frequently Asked Clinical Questions

Authoritative guidance on diagnosis, tumor boards, genomic markers, and care options

Related Rare Malignancies in Catalog

very-rare

Angioimmunoblastic T-cell Lymphoma (AITL)

very-rare

Appendiceal Carcinoma

very-rare

Esthesioneuroblastoma

very-rare

Fibrolamellar Hepatocellular Carcinoma (FL-HCC)

Independent Tumor Board Review

Facing a Diagnosis of Adenoid Cystic Carcinoma?

Rare cancers demand rare expertise. Connect your medical records to US NCI-fellowship trained oncologists, subspecialist pathologists, and active clinical trial investigators within 12 to 72 hours.

Upload Scans for Second Opinion Schedule Virtual Teleconsult