What is the recommended treatment for adenoid cystic carcinoma of the nose?

Treatment of Adenoid Cystic Carcinoma of the Nose

The optimal treatment for adenoid cystic carcinoma (ACC) of the nose is radical surgical resection followed by postoperative radiation therapy, as this combined approach offers the best outcomes for reducing mortality and improving quality of life. 1

Diagnosis and Staging

• Definitive diagnosis requires endoscopically guided biopsy of the primary tumor
• Histologic classification should follow World Health Organization guidelines
• Complete staging workup should include:
  • CT and/or MRI of the primary site and neck
  • Chest CT (not chest X-ray, which lacks sensitivity for pulmonary metastases) 1

Primary Treatment Algorithm

Early Stage Disease (T1-T2, N0)

1. Surgical Resection

   • Goal: Complete resection with negative margins 1
   • En bloc tumor resection is recommended 2
   • Primary reconstruction should be performed when appropriate

2. Postoperative Radiation Therapy

   • Recommended for all patients with resected ACC, regardless of stage 1
   • Dose: 60-70 Gy in standard fractions 1
   • Target volume: Surgical bed and appropriate nodal levels
   • Special consideration: If perineural invasion present, radiation should cover associated nerves to skull base 1

Advanced Disease (T3-T4, N0-N2)

1. Surgical Approach

   • Radical surgical resection with wider margins
   • Ipsilateral neck dissection for T3-T4 tumors 1
   • Consider en bloc resection of cranial base for selected T4 patients 2

2. Radiation Therapy

   • Some evidence suggests preoperative radiation may decrease incidence of distant metastasis 2
   • Postoperative radiation is standard practice (60-70 Gy) 1
   • Consider intensity-modulated RT for better local tumor control and reduced xerostomia 3

3. Combined Approach

   • For very advanced disease: Consider combined radiochemotherapy 3

Management of Recurrent or Metastatic Disease

Local Recurrence

• Small local recurrences are potentially curable 3
• Treatment options include:
  • Revision surgery when feasible
  • Brachytherapy
  • Stereotactic RT
  • Intensity-modulated RT
  • Combination of surgery and RT with or without chemotherapy 3

Regional Recurrence

• Radical neck dissection if resectable 3
• Consider intraoperative placement of catheters for postoperative brachytherapy 3

Distant Metastases

• For limited metastases (≤5), consider local ablative treatments 1
• For widespread metastases, systemic therapy options are limited
• ACC shows minimal response to conventional chemotherapy 4, 5
• Consider clinical trials of targeted therapies when available 1, 4

Follow-up and Surveillance

• Regular clinical examinations of the nasopharynx and neck
• Evaluation of cranial nerve function
• Imaging surveillance:
  • Initial post-treatment imaging at 3 months
  • Yearly imaging for at least 2-5 years
  • Extended surveillance beyond 5 years due to late recurrences 1
• Annual chest CT for at least 2 years (not chest X-ray) 1

Prognostic Factors

• Tumor extension grade significantly affects survival rates 2
• Negative surgical margins are critical for better outcomes 6
• Perineural invasion is associated with poorer prognosis 6
• Despite metastatic disease, approximately 10% of patients with ACC can survive more than 10 years 1

Important Caveats

• ACC has a high propensity for perineural invasion and distant metastases, particularly to the lungs
• Late recurrences (even >10 years after treatment) are common, necessitating long-term follow-up
• The 5-year overall survival rate for sinonasal ACC is approximately 63%, with disease-specific survival around 71% 7
• Ki-67 expression may provide additional prognostic information, with higher values associated with treatment failure 6