Consensus statement and guidelines on the management of paragangliomas of the head and neck

Paragangliomas (PGLs) of the head and neck (H&N) are uncommon tumors that either arise spontaneously or as part of an inherited syndrome. Hereditary PGL is an autosomal-dominant tumor predisposition syndrome in which an affected individual has greatly increased risk of developing PGL at any or several sites in the autonomic nervous system. The mode of inheritance for some is affected by maternal imprinting. These tumors are generally very slow growing, often taking a decade to double in size. A few are or can become malignant and can metastasize widely. Because of their site of origin, patients with these tumors may develop cranial nerve deficits that have a significant impact on their quality of life. Patients may present to specialists from widely differing disciplines, and some of these may not appreciate the full implications of their patient's disease. As a result, management can become fragmented or inappropriate, and some aspects of care may even be overlooked. This article is the distillation of consensus opinion derived from current published and unpublished data in this field, with particular reference to the management of temporal bone PGLs. We propose guidelines for the management of both sporadic and hereditary PGLs. A multidisciplinary team approach to the management of this complex disorder is advocated. Progress could be made by adopting these guidelines and by widespread dissemination of standardized information. Collaborative research should be promoted with the aim of harnessing advances in molecular genetics to develop targeted therapies for patients, particularly those with hereditary PGL.