Purpose of review: To provide an overview on the recent advances in the diagnosis and treatment of glomus tumors (paragangliomas).
Recent findings: The review focuses on the genetics of paragangliomas and discusses the role of newer therapeutic modalities in the management of jugulotympanic paragangliomas.
Summary: Recent advances in the field of genetics allowed the identification of three main loci involved in hereditary paragangliomas. Genetic screening of patients with family history identifies affected individuals. The management of glomus tumors continues to be a challenging task. Surgery remains the mainstay of treatment for young healthy patients with functional cranial nerve deficits. Gamma knife radiosurgery is offered as an adjunctive to limited surgery and as the primary treatment modality in poor surgical candidates or patients with bilateral disease.