Dear Colleagues,

This Special Issue of Cancers will focus on papers from the "First International Congress Update on the Management of HEAD & NECK PARAGANGLIOMAS” (linked to https://paragangliomas.com/), due to take place in Piacenza, Italy on September 20–22, 2023.

Head & neck paragangliomas are hypervascular neural crest tumors that mainly originate from the parasympathetic paraganglia of the lower cranial nerves. These tumors are rare (<0.5% of all head-and-neck tumors), present at any age, peaking between the 4th and 7th decades, with female preference, and tend to grow slowly, but are invasive and radio/chemo-resistant. Depending on the site, they cause serious or even life-threatening complications, including hearing loss, dysphagia, cranial nerve palsy, bradycardia, hemorrhage, infiltration of the skull base and intracranial growth. Metastatic risk is low but never excludable, warranting lifelong follow-up. As with other forms of paraganglioma, head & neck paragangliomas occur in hereditary syndromes linked to predisposing mutations in diverse genes, many of which are implicated in the Krebs cycle and in the hypoxia response. Genetic predisposition cannot be ruled out for apparently sporadic tumors: in fact, at least 35-40% of all head & neck paraganglioma patients carry predisposing gene variants.

Much knowledge about the biological, genetic, pathological, neuroendocrinological and epidemiological characteristics of head & neck paragangliomas has been accumulated in the last two decades, together with significant improvements in diagnostic imaging. This knowledge opens new perspectives on disease etiology, molecular markers, and targeted therapies and directly impacts the management of head and neck paraganglioma patients and their families. However, surgery remains the only effective therapy. Surgical approaches and techniques have also evolved within the last 3 decades, and tumors once considered inoperable can now be safely removed, provided that special expertise is available. Presently, there is a need of improving the dissemination of specialized knowledge on state-of-the-art head & neck paraganglioma surgery, and it is also necessary that surgical and follow-up strategies consider biological, genetic, and pathological data predictive of aggressive disease, synchronous or metachronous tumors, and possible occurrence of familial clusters.

The aim of the Congress, reflected in this Special Issue, is to give an up-to-date overview of the specialized head & neck paraganglioma field. The Congress papers will integrate current scientific developments with surgical management and will provide guidelines for early diagnosis, treatment and follow-up of patients and at-risk relatives.

Prof. Dr. Mario Sanna
Prof. Dr. Renato Mariani-Costantini
Dr. Francesca Schiavi
Prof. Dr. Karel Pacak
Dr. Anasuya Guha
Guest Editors

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