Adenoid Cystic Cancer: Symptoms, Root Causes, and Treatment Options
Adenoid cystic carcinoma (ACC) is a rare type of cancer that can start in any secretory glands throughout the body. Accounting for about 10% of all head and neck cancers, ACC is diagnosed in approximately 1,200 new cases annually in the United States.
Symptoms of ACC can vary depending on the location of the tumor. In the salivary gland, symptoms may include facial numbness, a painless lump, drooping in the face, trouble swallowing or opening the mouth. When a tumor develops in the lacrimal glands around the eyes, an individual may experience a bulging eye or changes in their vision. Tumors that appear in the skin may be painful and create bloody or pus discharge. If the tumor develops in the larynx and trachea, a person may experience issues with breathing or speech or notice hoarseness.
Treatment for ACC varies based on the tumor’s location and stage but generally includes surgery, radiation therapy, chemotherapy, and emerging targeted or advanced modalities.
By Location and Stage
1. Salivary Gland ACC (common location)
- In early stages, surgery is the primary treatment, aiming for complete tumor removal with clear margins. Lymph nodes near the tumor, especially in the neck, may also be removed and examined to check for spread.
- In advanced stages or post-surgery, radiation therapy is often recommended to improve local control because ACC has a propensity for local recurrence and perineural invasion.
- For metastatic or recurrent disease, chemotherapy and clinical trials exploring new therapies (e.g., targeted agents like REM-422) may be considered.
2. Lacrimal Gland ACC (LGACC)
- Due to the complex anatomy and high recurrence risk, treatment includes neoadjuvant intra-arterial cytoreductive chemotherapy (IACC), followed by surgery (often orbital exenteration), and radiation therapy. IACC delivers high doses of chemotherapy (cisplatin, adriamycin) directly to the tumor via the lacrimal artery to shrink the tumor before surgery, enhancing outcomes.
- In high-risk or recurrent cases, combined chemotherapy and radiation therapy play key roles in improving survival and disease control.
3. Laryngeal and Subglottic ACC
- The standard treatment involves extensive surgical resection, which may include total laryngectomy due to submucosal spread and nerve invasion.
- Postoperative radiotherapy is often combined with surgery to improve local control and potentially preserve laryngeal function. Techniques such as proton therapy, neutron therapy, and carbon ion radiation have shown promise, especially for inoperable cases or to reduce side effects.
- In cases of recurrence, repeat surgery may be considered if feasible.
4. Tracheal ACC
- Preferred when possible, surgery is used for tumor removal.
- Radiation therapy is used when surgery is not possible, tumor occupies a large portion of the trachea, or lymph nodes are involved. External beam radiation is standard; brachytherapy (direct radiation via bronchoscope) and bronchoscopic treatments like laser therapy and spray cryotherapy are options in some non-surgical cases to control airway obstruction or bleeding.
- Chemotherapy may be used adjunctively or in advanced cases, though ACC typically is less responsive to chemotherapy.
General Treatment Considerations
- Treatment is guided by TNM staging, which assesses tumor size, lymph node involvement, and distant metastasis.
- ACC is notorious for late recurrences and distant metastasis; thus, long-term monitoring is critical.
- Clinical trials with novel agents like REM-422 are underway for recurrent or metastatic ACC to improve outcomes beyond conventional treatments.
In conclusion, surgery combined with radiation therapy remains the cornerstone of ACC treatment across different anatomical sites and stages, with chemotherapy and newer modalities playing adjunctive roles, especially in advanced, recurrent, or metastatic disease.
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