Adenoid Cystic Carcinoma Overview
- Rare type of cancer that can occur in various body sites
- Most commonly found in salivary glands
- Can also be found in the breast, lacrimal gland, lung, brain, Bartholin gland, trachea, and paranasal sinuses
- Represents 28% of malignant submandibular gland tumors
- Patients may survive for years with metastases

Cause of Adenoid Cystic Carcinoma
- Activation of the oncogenic transcription factor gene MYB is the key genomic event
- MYB is often activated through gene fusion with the NFIB gene
- MYB can also be activated by copy number gain or enhancer elements
- In some cases, the MYBL1 gene is fused to NFIB or other partners
- MYB drives proliferation of ACC cells and regulates genes involved in cell cycle control

Treatment of Adenoid Cystic Carcinoma
- Primary treatment is surgical removal with clean margins
- Surgery can be challenging due to perineural discontinuous growth
- Adjuvant or palliative radiotherapy is commonly given after surgery
- Fast neutron therapy is effective for inoperable or recurrent tumors
- Chemotherapy is used for metastatic disease on a case-by-case basis

Images of Adenoid Cystic Carcinoma
- Coronal MRI images showing tumor spread along nerve tracts
- Histopathological images showing infiltration of nerves and immunostaining
- Fine needle aspiration specimen showing adenoid cystic carcinoma
- Images depicting comedonecrosis and relative incidence of submandibular/parotid tumors

Related Topics
- Primary cutaneous adenoid cystic carcinoma
- Collagenous spherulosis

Note: The content has been organized into 5 comprehensive groups based on the given information.

Adenoid cystic carcinoma (Wikipedia)

Adenoid cystic carcinoma is a rare type of cancer that can exist in many different body sites. This tumor most often occurs in the salivary glands, but it can also be found in many anatomic sites, including the breast, lacrimal gland, lung, brain, Bartholin gland, trachea, and the paranasal sinuses.

Adenoid cystic carcinoma
Other names	Adenocyst, Malignant cylindroma, Adenocystic, Adenoidcystic
Micrograph of an adenoid cystic carcinoma of a salivary gland (right of image): Normal serous glands, typical of the parotid gland, are also seen (left of image), H&E stain.
Specialty	Oncology

It is the third-most common malignant salivary gland tumor overall (after mucoepidermoid carcinoma and polymorphous adenocarcinoma). It represents 28% of malignant submandibular gland tumors, making it the single most common malignant salivary gland tumor in this region. Patients may survive for years with metastases because this tumor is generally well-differentiated and slow growing. In a 1999 study of a cohort of 160 ACC patients, disease-specific survival was 89% at 5 years, but only 40% at 15 years,^{[citation needed]} reflecting deaths from late-occurring metastatic disease.