What are the treatment options for esthesioneuroblastoma?

Treatment Options for Esthesioneuroblastoma

The optimal treatment approach for esthesioneuroblastoma involves multimodal therapy with surgery as the primary intervention, followed by adjuvant radiation therapy, with chemotherapy reserved for advanced disease. 1

Initial Surgical Management

• Complete surgical resection should be the initial treatment to establish diagnosis, relieve symptoms, and maximize local control 1
• Surgical approaches include:
  • Traditional craniofacial resections for extensive disease 2
  • Minimally invasive endoscopic resections for selected cases 2
  • Endoscopic-assisted cranionasal resection as a hybrid approach 2
• Complete resection (>90% of tumor) significantly improves event-free survival and reduces local recurrence rates 1
• For unresectable tumors, consider neoadjuvant chemotherapy to achieve cytoreduction before attempting surgery 1

Radiation Therapy

• Adjuvant radiation therapy after surgery is standard of care, even when negative margins are achieved 1
• Conventional dose ranges from 45-60 Gy to the primary site 1
• For patients with unresectable disease, definitive radiation therapy alone should be applied 1
• Preoperative radiation therapy may be considered in selected cases to improve resectability 3

Chemotherapy

• Adjuvant chemotherapy should be added for advanced disease (Kadish C) 4
• Effective agents include cyclophosphamide, vincristine, doxorubicin, and ifosfamide 1
• Ten-year highest survival rates were obtained in patients treated by the combination of surgery, radiotherapy and chemotherapy for advanced disease 4

Treatment by Disease Stage

• Kadish A (limited to nasal cavity):

  • Surgery followed by adjuvant radiation therapy 1, 2
  • Consider surgery alone for very small, completely resected tumors 2

• Kadish B (extension to paranasal sinuses):

  • Surgery followed by adjuvant radiation therapy 1, 3
  • Preoperative radiotherapy may be considered 3

• Kadish C (extension beyond paranasal sinuses):

  • Multimodal approach with surgery, radiation, and chemotherapy 1, 4
  • Preoperative chemoradiotherapy followed by surgery 3

Imaging and Follow-up

• MRI with IV contrast is the preferred imaging modality for diagnosis and follow-up 5
• Somatostatin analog PET/CT or PET/MRI can be helpful in disease extent evaluation and planning of radionuclide therapy 5
• Patients should be followed with neurological assessment and neuroimaging at 3-month intervals 5

Outcomes and Prognosis

• Five-year disease-specific survival can reach up to 90% with appropriate multimodal therapy 1
• Ten-year disease-specific survival of approximately 80% has been reported 2
• Positive surgical margins and advanced TNM staging negatively impact survival 2

Common Pitfalls to Avoid

• Inappropriate grouping of esthesioneuroblastoma with other sinonasal malignancies can lead to incorrect treatment decisions 1
• Delayed diagnosis is common due to the clinically hidden primary site at the anterior skull base 6
• Esthesioneuroblastoma has a significantly better prognosis than other sinonasal malignancies (5-year survival of ~90% vs 37% for mucosal melanoma) 1
• Thorough histological evaluation with immunohistochemistry is essential for correct diagnosis and differentiation from other sinonasal tumors 3