Onkológia 2/2013

Rare cancers of the head and neck

Rare tumors are described according to the definition for rare tumours by RARECARE (incidence < 6 per 100,000), as well as according to the definition for rare diseases by the European Commission (prevalence < 50 per 100,000). The rare cancers of head and neck are tumours of nasopharynx, salivary glands, nasal cavity and sinuses. There is a remarkable variation in geografic distribution of nasopharyngeal carcinoma, the main risk factor is EBV infection. It is a chemo-radiosensitive tumor, radiation therapy remains the mainstay of treatment, alone in early stages and with addition of chemotherapy in advanced carcinoma. Complete surgical resection, with or without radiotherapy according risk factors, is the cornerstone of diagnosis and treatment of salivary glands tumors. Watchful waiting may be the most appropriate strategy for metastatic patients with indolent disease and no symptoms. Chemotherapy is only palliative. The majority of paranasal sinus cancer arise in the maxillary and ethmoid sinuses. Because of their anatomic location they tend to be long asymptomatic. The patients have been treated with surgery and radiotherapy, which reduce the risk of local recurrence. Diagnosis and treatment of these rare cancers should be centralised.

Keywords: rare cancer, head and neck cancer, treatment.