The Basic Science of Glomus Jugulare Tumors

Jason Heth, M.D.


Neurosurg Focus. 2004;17(2) 

In This Article

Abstract and Introduction

Glomus tumors are a fascinating group of lesions. It is a challenge for neurosurgeons and otolaryngologists to resect them completely with minimal morbidity. Laboratory researchers have discovered extremely interesting genetic and molecular biology factors involved in the development and growth of glomus tumors. In this article the author reviews the genetics, protein mutations, angiogenesis and apoptosis associated with tumor formation, and the secretion of vasoactive substances is discussed as well. It is hoped that with further research less invasive measures may be developed to treat these tumors.

Skull base surgery has made significant strides in its ability to resect skull base tumors. Advanced instrumentation, surgical procedures, anesthetic agents, and reconstructive techniques have made this possible. Several tumors continue to present challenges despite their benign biological character; glomus tumors fit this description. Glomus jugulare tumors are fascinating lesions that arise from paraganglia cells situated near the jugular foramen. Their intimate association with the lower cranial nerves and inner ear complicates their management, because injuries to these structures can cause significant morbidity and functional disability. Knowledge of the basic biology of glomus tumors may result in improved therapies or a molecular-level noninvasive treatment. Basic research in glomus tumors has surged and subsequent advances in understanding of the basic biology of glomus jugulare tumors have been exciting. This review examines the basic epidemiological features of glomus tumors that led to further inquiry. Such investigations have resulted in important advances in the understanding of the genetic and molecular biology aspects of glomus tumors. Angiogenesis and apoptosis in tumor formation are examined, and production of vasoactive substances is reviewed.

A preliminary note about terminology is in order be cause these tumors have been called by many names. Glomus tumors and paragangliomas are two conventions in common use. In keeping with the chosen topic, the tumors of interest will be referred to as glomus jugulare tumors. In many studies all tumor subgroups are pooled to in crease the amount of data, and therefore all tumors in this review will be referred to as glomus tumors.