Management of Metastatic Glomus Jugulare Tumors

Abstract

Metastatic glomus jugulare tumors (jugular paragangliomas) are very uncommon: consequently, no single report has been able to accurately describe their biologic or clinical behavior, and there are no established guidelines on caring for patients with these tumors. Identification of metastatic paraganglioma can be difficult because these tumors are known to occur synchronously, many other tumor types appear histologically similar to paragangliomas, and histologically the metastasis may not resemble the primary tumor. Immunohistochemistry, using neuroendocrine markers, and electron microscopy are the two most useful techniques available to identify definitely paragangliomas. A few studies have shown differences in the immunohistochemical staining patterns between benign and metastatic paragangliomas. We reviewed the literature and include two additional cases of metastatic glomus jugulare tumors in this article. We discuss the clinical management of these patients, the importance and usefulness of immunohistochemistry in characterizing these tumors, and their clinical outcomes.