Head and neck

Rare cancer of the head and neck

Reference centers for primary brain tumors are gathered in the domain 7 of EURACAN

 

They manage tumors originating from 3 different subsites: nasopharyngeal cancer, nasal and paranasal sinus cancers, salivary gland cancers and middle ear epithelial cancers. Their pathogenesis remains mostly unknown. Smoking may play a role in nasopharyngeal and nasal, paranasal sinus cancers. For these latter tumors, at least for some of them occupational exposure has been recognized.

For all of them early diagnosis would significantly contribute to improve overall outcome and reduce treatment related side effect and late toxicities. A Eu effort is ongoing to promote awareness and early detection of head and neck cancers www.makesensecampaign.eu.

Nasopharyngeal cancer (NPC) is extremely rare in EU, but endemic in some Asian countries. It is an undifferentiated squamous cell carcinoma mostly associated with Epstein Barr virus (EBV). EBV is not sufficient to induce this cancer: genetic predisposition and associated carcinogens play an important role. In Eu it often presents as locally advanced. In this setting an integrated multidisciplinary treatment is recommended. In this context treatment appropriateness and quality of care are  essential. Dedicated and concerted activities among multidisciplinary experts may make the difference in achieving optimized cure rates.

Nasal and paranasal tumors include 9 different histotypes that include olfactory neuroblastoma (66).. For some, a viral origin (HPV) has been suggested. An accurate histopathological diagnosis by an expert pathologist is needed and often requires molecular confirmation.  Due to their localizations, these tumors are frequently diagnosed at a late stage where an integrated multidisciplinary treatment is recommended. In this context treatment appropriateness and quality of care is essential. Dedicated and concerted activities among multidisciplinary experts may make the difference in achieving optimized cure rates. Similarly to other rare tumors research may be only possible through multinational collaborations such as EURACAN.

Salivary gland cancers includes more than 20 histotypes with very different behaviours ranging from slow growing tumors to locally invading, along nerves or to distant sites (67). Some of these cancers occur in a previous irradiation field. Expertise in pathological diagnosis is essential to guide the whole treatment process. Often a molecular diagnosis is needed to better classify tumor histotype. The heterogeneity of the disease justifies histotype tailored treatment approaches, often multidisciplinar. Trials are almost impossible to be conducted in single countries. Therefore EURACAN framework is essential to allow preclinical and clinical research to be conducted.

Middle ear tumor is essentially a squamous cell carcinoma, less frequently an adenocarcinoma. It is characterized by a local aggressiveness and regional spreading. Clinical data on these diseases are very scanty and their treatment is generally derived from that of squamous head and neck cancers that incudes surgery and radiation.

Overall, rare head and neck cancer gather a variety of very different malignant diseases, with distinct etiologies and natural history, requiring expert diagnosis expert treatments, and prospective collection of clinical data in order to better standardize the treatments.

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