Clinical and Experimental Otorhinolaryngology Vol. 2, No. 1: 28-32, March 2009 DOI 10.3342/ceo.2009.2.1.28 Original Article Clinical Presentation and Management of Jugular Foramen Paraganglioma Sa Myung Chung, MD Hyun Su Kim, MD Jinsei Jung, MD Ho-Ki Lee, MD, PhD Won Sang Lee, MD, PhD Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul, Korea Objectives. Jugular foramen paraganglioma is a locally invasive, benign tumor, which grow slowly and causes various symp- toms such as pulsatile tinnitus and low cranial nerve palsy. Complete surgical resection is regarded as the ideal man- agement of these tumors. The goal of this study is to identify the clinical characteristics and most effective surgical approach for jugular foramen paraganglioma. Methods. Retrospective analysis of 9 jugular foramen paraganglioma patients who underwent surgical resection between 1986 and 2005 was performed. Clinical records were reviewed for analysis of initial clinical symptoms and signs, audiological examinations, neurological deficits, radiological features, surgical approaches, extent of resection, treat- ment outcomes and complications. Results. Most common initial symptom was hoarseness, followed by pulsatile tinnitus. Seven out of 9 patients had at least one low cranial nerve palsy. Seven patients were classified as Fisch Type C tumor and remaining 2 as Fisch Type D tumor on radiologic examination. Total of 11 operations took place in 9 patients. Total resection was achieved in 6 cases, when partial resection was done in 3 cases. Two patients with partial resection received gamma knife radio- surgery (GKS), when remaining 1 case received both GKS and two times of revision operation. No mortality was encountered and there were few postoperative complications. Conclusion. Neurologic examination of low cranial nerve palsy is crucial since most patients had at least one low cranial nerve palsy. All tumors were detected in advanced stage due to slow growing nature and lack of symptom. Angiogra- phy with embolization is crucial for successful tumor removal without massive bleeding. Infratemporal fossa approach can be considered as a safe, satisfactory approach for removal of jugular foramen paragangliomas. In tumors with intracranial extension, combined approach is recommended in that it provides better surgical view and can main- tain the compliance of the patients. Key Words. Jugular foramen paraganglioma, Infratemporal approach, Intracranial extension, Combined approach INTRODUCTION Jugular foramen paragangliomas are rare, slow-growing, encap- sulated, hypervascular tumors that arise from jugular foramen of temporal bone (1). Jugular foramen paraganglioma are known to occur predominantly in the age of 50 to 60, and female to male ratio is reported to be 5:1 (1, 2). Incidence of multiple lesion is reported to be between 25 to 50% in familial cases, compared with less than 10% in sporadic cases (1). Received August 21, 2008 Accepted after revision January 21, 2009 Corresponding author : Won Sang Lee, MD, PhD Department of Otorhinolaryngology, Yonsei University College of Medicine, 134 Sinchon-dong, Seodaemun-gu, Seoul 120-752, Korea Tel : +82-2-2228-3606, Fax : +82-2-393-0580 E-mail : wsleemd@yumc.yonsei.ac.kr Jugular foramen paragangliomas are locally invasive, expand- ing within the temporal bone via pathways of least resistance, such as air cells, vascular lumens, skull base foramina, and eustachian tube (3, 4). Significant intracranial and extracranial extension may occur, as well as extension within the sigmoid sinus and inferior petrosal sinuses (3-5). Clinical course of jugular foramen paragangliomas reflects their slow growth and paucity of symptoms, which often results in a significant delay in diagnosis (1, 3, 6). The most common present- ing symptom is known to be pulsatile tinnitus, followed by hear- ing loss (2, 7). Complete surgical resection is the ideal management of most jugular foramen paragangliomas (2, 5, 7-9). Radiation therapy can be utilized as an alternative treatment modality for certain candidates including elderly and medically inoperable patients, and is also indicated in recurred tumor after surgical resection and in 28