Management is difficult due to the location of these tumours. Specific management of different tumour types is covered below.

General Considerations

There are limited evidence based guidelines for these tumours due to their scarcity. Case series provide the most information.

  • Surgery generally involves extensive craniofacial resection and may include orbital extenteration or base of skull repairs in some cases. T1 and T2 tumours may be resectable with minimal morbidity.
    • Recurrence is common after surgery and adjuvant radiotherapy is frequently recommended (70 Gy to residual disease, 60 Gy to high risk CTV (tumour bed, involved nodal regions), 50 Gy to elective nodes.
    • For tumours with perineural invasion, coverage of the maxillary nerve to the trigeminal ganglion is recommended (54 Gy).
  • Radiotherapy alone is often indicated for patients where surgery would be impossible or if the patient is not medically fit. It typically provides significantly poorer outcomes compared to other head and neck sites, likely due to numerous critical structures. IMRT may help to compensate for this in the modern setting.

Radiotherapy toxicity is not uncommon, particularly blindness due to cataract or optic pathway damage, brain necrosis if there is CNS involvement, or osteoradionecrosis.

Squamous Cell Carcinoma

Salivary Gland Tumours

Sinonasal Undifferentiated Carcinoma (SNUC)

Olfactory Neuroblastoma

This tumour is typically managed with combined modality therapy.
* Surgery involves removal of the tumour, often requiring specialist craniofacial surgery and neurosurgery to obtain a complete excision
* Adjuvant radiotherapy significantly improves disease control if administered post-operatively. The typical dose is 60 Gy to the tumour bed and 70 Gy to macroscopic disease or positive margins.
Primary radiotherapy is used when surgery is not possible. 70 Gy in the goal dose but may be difficult to administer. Kadish A tumours have a good control rate but radiotherapy only provides control in 50% of Kadish B and 20% of Kadish C tumours.
Lymphadenopathy is present in only 5% of patients and the neck is not usually included in treatment volumes unless there is disease present.
Chemotherapy has no established role.

Neuroendocrine Tumours