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

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