--- title: "Adenoid Cystic Carcinoma" description: "ENT notes on Adenoid Cystic Carcinoma - Histogenesis, Histology, Symptoms, Investigations, Treatment and Recurrence." source: "The ENT Resident" author: "Dr. Mausumi Das" canonical_url: "https://www.theentresident.com/ent-notes-lectures/adenoid-cystic-carcinoma-histology-treatment" categories: ["Oral Cavity and Salivary Glands"] video: "https://www.youtube.com/watch?v=ZSB0_DCJ4GE" image: "https://media.theentresident.com/og-images/adenoid-cystic-carcinoma-histology-treatment.png" --- # Adenoid Cystic Carcinoma ENT notes on Adenoid Cystic Carcinoma - Histogenesis, Histology, Symptoms, Investigations, Treatment and Recurrence. Watch the full video lecture on YouTube: https://www.youtube.com/watch?v=ZSB0_DCJ4GE ## 🧬 Adenoid Cystic Carcinoma ### 🧠 Definition of Adenoid Cystic Carcinoma Adenoid cystic carcinoma is a **malignant epithelial salivary gland tumor** that is **slow growing yet highly aggressive**, with a marked tendency for **recurrence and perineural invasion**. - **Most common malignant tumor of the submandibular gland** - Accounts for **~ 10% of all salivary gland tumors** - Common age group: **5th–7th decade** - **Female preponderance** > What is Neurotropism? > What is the other name of Adenoid Cystic Carcinoma? > Which is the most common malignant salivary gland tumor? > Which is the second most common malignant salivary gland tumor? --- ### 🧬 Histogenesis of Adenoid Cystic Carcinoma Explained by the **Reserve Cell (Stem Cell) Theory**: - Tumor arises from **pluripotent reserve cells of intercalated ducts** - These differentiate into: - **Ductal cells** - **Myoepithelial cells** > What are the Genetic Alterations seen in Adenoid Cystic Carcinoma? --- ### πŸ” Histology of Adenoid Cystic Carcinoma #### Gross Features - **Poorly encapsulated tumor** - Firm, **gray-white** - Cut section shows **solid tumor with small cystic spaces** > What is characteristic appearance seen in Adenoid Cystic Carcinoma? #### Microscopic Features Tumor consists of **ductal cells + myoepithelial cells** arranged in **three patterns**: πŸ§€ **Histological Patterns of Adenoid Cystic Carcinoma** - Cribriform - Tubular - Solid 🚩 Most tumors show **mixed patterns** – classification depends on the **predominant pattern** > Which is the most common Histological Patterns of Adenoid Cystic Carcinoma? > Which is the most aggressive Histological Patterns of Adenoid Cystic Carcinoma? Why? > What is the Histological Grading of Adenoid Cystic Carcinoma? --- ### πŸ“ Sites of Involvement of Adenoid Cystic Carcinoma - **Minor salivary glands** - **Major salivary glands**: - Parotid: 20–25% - Submandibular: 10–15% - Sublingual: ~5% > What is the most common site of involvement of Adenoid Cystic Carcinoma? > What is the most common site of involvement of Adenoid Cystic Carcinoma in minor salivary glands? --- ### 🩺 Clinical Features of Adenoid Cystic Carcinoma - **Slow-growing, firm mass** - **Pain occurs early** (important malignant feature) - Progressive numbness - Facial nerve palsy (parotid involvement) - Fixation to deeper structures - Surface ulceration > What is Neurotropism? What is it's implications in spread of Adenoid Cystic Carcinoma? **Metastasis** - Regional lymph nodes – rare - **Distant metastasis**:- Lung, Bone, Liver > What is the most common site of distant metastasis in Adenoid Cystic Carcinoma? --- ### πŸ”Ž Investigations of Adenoid Cystic Carcinoma - **Ultrasound** – first line, FNAC guidance - **FNAC** – diagnostic - **Biopsy** – confirms pattern, grade, perineural invasion - **MRI (Gold standard)** - **CT scan** – bone involvement - **PET-CT** – staging and distant metastasis --- ### πŸ₯ Treatment of Adenoid Cystic Carcinoma #### πŸ”ͺ Surgery (Mainstay) - **Wide local excision / total gland excision** - Bone resection if involved (palate, mandible, maxilla) - Facial nerve preserved unless infiltrated #### 🦴 Neck Dissection - **Clinically node-positive** β†’ Therapeutic neck dissection - **Clinically N0** β†’ Usually **not done** > What are the indications of Neck Dissection in Clinically N0 neck in Adenoid Cystic Carcinoma? > What are the indications of Adjuvant Radiotherapy in Adenoid Cystic Carcinoma? #### πŸ’Š Chemotherapy in Adenoid Cystic Carcinoma - Very limited role - Mainly **palliative** - Drugs with temporary response: - Cisplatin - Doxorubicin - Cyclophosphamide --- ### πŸ“‰ Prognosis of Adenoid Cystic Carcinoma #### Poor Prognostic Factors - **Solid histological pattern (Grade 3)** - Perineural invasion - Vascular invasion - Positive margins - Advanced stage - Submandibular / sublingual involvement #### Survival Rates - **5-year survival:** 60–75% - **10-year survival:** 30–50% --- ### πŸ“Œ Exam Pearls - Slow growing but aggressive = **Adenoid cystic carcinoma** - **Swiss cheese appearance** - **Perineural invasion β†’ skip lesions** - **Solid pattern = worst prognosis** - Most common malignancy of **submandibular gland** --- ## About This Note This free ENT note on Adenoid Cystic Carcinoma was written by Dr. Mausumi Das and is paired with a corresponding video lecture. 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