Glomus jugulare tumors: historical overview of the management of this disease

2004 ◽  
Vol 17 (2) ◽  
pp. 1-5 ◽  
Author(s):  
L. Madison Michael ◽  
Jon H. Robertson
Keyword(s):  
Radiation Treatment ◽  
Cranial Nerves ◽  
Treatment Strategies ◽  
Historical Review ◽  
Jugular Bulb ◽  
Management Problem ◽  
Management Options ◽  
Glomus Jugulare ◽  
Considerable Controversy ◽  
Lower Cranial Nerves

The treatment of glomus jugulare tumors presents the surgeon with a significant management problem. Because the neoplasm originates in the region of the jugular bulb, it frequently involves the lower cranial nerves, with occasional extension into the posterior fossa. Despite extensive work on the development of surgical and radiation treatment strategies, considerable controversy still exists regarding the optimal management of these lesions. A historical review of the development of management options for glomus jugulare tumors is presented in an effort to offer a foundation for understanding their contemporary treatment.

scholarly journals Complex tumors of the glomus jugulare: criteria, treatment, and outcome

2004 ◽  
Vol 17 (2) ◽  
pp. 1356-1366
Author(s):  
Ossama Al-Mefty ◽  
Aramis Teixeira
Keyword(s):  
Malignant Tumor ◽  
Cranial Nerve ◽  
Treatment Plan ◽  
Cranial Nerves ◽  
Neurological Deficits ◽  
Adrenal Tumors ◽  
Glomus Jugulare ◽  
Associated Lesions ◽  
Vertebrobasilar Artery ◽  
Lower Cranial Nerves

Object Tumors of the glomus jugulare are benign, slow-growing paragangliomas. Their natural history, surgical treatment, and outcome have been well addressed in the recent literature; however, there remains a subgroup of complex tumors—multiple, giant, malignant, neuropeptide-secreting lesions, and those treated previously by an intervention with an adverse outcome—that is high risk, presents surgical challenges, and is associated with treatment controversy. In this article the authors report on a series of patients with complex glomus jugulare tumors and focus on treatment decisions, avoidance of complications, surgical refinements, and patient outcomes. Methods In this retrospective study, the patient population was composed of 11 male and 32 female patients (mean age 47 years) with complex tumors of the glomus jugulare who were treated by the senior author within the past 20 years. These include 38 patients with giant tumors, 11 with multiple paragangliomas (seven bilateral and four ipsilateral), two with tumors that hypersecreted catecholamine, and one with a malignant tumor. Six patients had associated lesions: one dural arteriovenous malformation, one carotid artery (CA) aneurysm, two adrenal tumors, and two other cranial tumors. All but one patient presented with neurological deficits. Cranial nerve deficits, particularly those associated with the lower cranial nerves, were the prominent feature. Twenty-eight patients underwent resection in an attempt at total removal, and gross-total resection was achieved in 24 patients. Particularly challenging were cases in which the patient had undergone prior embolization or CA occlusion, after which new feeding vessels from the internal CA and vertebrobasilar artery circulation developed. The surgical technique was tailored to each patient and each tumor. It was modified to preserve facial nerve function, particularly in patients with bilateral tumors. Intrabulbar dissection was performed to increase the likelihood that the lower cranial nerves would be preserved. Each tumor was isolated to improve its resectability and prevent blood loss. No operative mortality occurred. In one patient hemiplegia developed postoperatively due to CA thrombosis, but the patient recovered after an endovascular injection of urokinase. In four patients a cerebrospinal fluid leak was treated through spinal drainage, and in five patients infection developed in the external ear canal. Two of these infections progressed to osteomyelitis of the temporal bone. There were two recurrences, one in a patient with a malignant tumor who eventually died of the disease. Conclusions Despite the challenges encountered in treating complex glomus jugulare tumors, resection is indicated and successful. Multiple tumors mandate a treatment plan that addresses the risk of bilateral cranial nerve deficits. The intra-bulbar dissection technique can be used with any tumor, as long as the tumor itself has not penetrated the wall of the jugular bulb or infiltrated the cranial nerves. Tumors that hypersecrete catecholamine require perioperative management and malignant tumors carry a poor prognosis.

scholarly journals Lower Cranial Nerves Compromise in Glomus Jugulare Tumors

2012 ◽  
Vol 147 (2_suppl) ◽  
pp. P212-P213
Author(s):  
Evandro M. Silva ◽  
Adriano T. Kitice ◽  
Gabriela R. Pascoto ◽  
Raimar Weber ◽  
Iulo Barauna ◽  
...  
Keyword(s):  
Cranial Nerves ◽  
Glomus Jugulare ◽  
Lower Cranial Nerves

Microsurgical Resection of Glomus Jugulare Tumors With Facial Nerve Reconstruction: 3-Dimensional Operative Video

2018 ◽  
Vol 16 (1) ◽  
pp. E1-E1 ◽  
Author(s):  
Duarte N C Cândido ◽  
Jean Gonçalves de Oliveira ◽  
Luis A B Borba
Keyword(s):  
Facial Nerve ◽  
Infratemporal Fossa ◽  
Tumor Resection ◽  
Cranial Nerves ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Senior Author ◽  
Nerve Reconstruction ◽  
Glomus Jugulare ◽  
Carotid Canal

Abstract Paragangliomas are tumors originating from the paraganglionic system (autonomic nervous system), mostly found at the region around the jugular bulb, for which reason they are also termed glomus jugulare tumors (GJT). Although these lesions appear to be histologically benign, clinically they present with great morbidity, especially due to invasion of nearby structures such as the lower cranial nerves. These are challenging tumors, as they need complex approaches and great knowledge of the skull base. We present the case of a 31-year-old woman, operated by the senior author, with a 1-year history of tinnitus, vertigo, and progressive hearing loss, that evolved with facial nerve palsy (House-Brackmann IV) 2 months before surgery. Magnetic resonance imaging and computed tomography scans demonstrated a typical lesion with intense flow voids at the jugular foramen region with invasion of the petrous and tympanic bone, carotid canal, and middle ear, and extending to the infratemporal fossa (type C2 of Fisch's classification for GJT). During the procedure the mastoid part of the facial nerve was identified involved by tumor and needed to be resected. We also describe the technique for nerve reconstruction, using an interposition graft from the great auricular nerve, harvested at the beginning of the surgery. We achieved total tumor resection with a remarkable postoperative course. The patient also presented with facial function after 6 months. The patient consented with publication of her images.

scholarly journals Glomus Jugulare Presenting with Isolated Facial Nerve Palsy

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Angelica A. Nunez ◽  
Luis R. Ramos-Duran ◽  
Albert C. Cuetter
Keyword(s):  
Nerve Palsy ◽  
Facial Nerve Palsy ◽  
Cranial Nerves ◽  
Petrous Bone ◽  
Jugular Foramen ◽  
Facial Weakness ◽  
Glomus Jugulare ◽  
Vascular Structures ◽  
Lower Cranial Nerves ◽  
Slow Growing

Glomus jugulare is a rare slow growing tumor occurring within the jugular foramen that rarely presents with isolated symptoms. Although histologically benign, these tumors are locally destructive because of their proximity to the petrous bone, the lower cranial nerves, and the major vascular structures (Miller et al. (2009) and Silverstone (1973)). We wish to report a glomus jugulare tumor eroding the petrous bone and producing an ipsilateral peripheral facial weakness. The mechanism of this erosion is discussed.

Lower Skull Base: Anatomic Study with Surgical Implications

1995 ◽  
Vol 104 (1) ◽  
pp. 57-61 ◽  
Author(s):  
Essam Saleh ◽  
Maged Naguib ◽  
Yasar Cokkeser ◽  
Miguel Aristegui ◽  
Mario Sanna
Keyword(s):  
Connective Tissue ◽  
Skull Base ◽  
Fibrous Tissue ◽  
Cranial Nerves ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Microsurgical Anatomy ◽  
Anatomic Study ◽  
Hypoglossal Canal ◽  
Lower Cranial Nerves

With advances in the lateral approaches to the skull base and the increasing success of the management of jugular foramen lesions, a thorough knowledge of the anatomy of this region is needed. The purpose of the present work is to study the detailed microsurgical anatomy of the lower skull base and the jugular foramen area as seen through the lateral approaches. Forty preserved skull base specimens and 5 fresh cadavers were dissected. The shape of the jugular bulb and its relationship to nearby structures were recorded. The different venous connections of the bulb were noted. The hypoglossal canal was identified and its contents were observed. The lower cranial nerves were studied at the level of the upper neck, at their exit from the inferior skull base, and in the jugular foramen. The results of the present study showed the complex and variable anatomy of this area. The classic compartments of the jugular foramen were not always present. Cranial nerves IX through XI followed different patterns while passing through the jugular foramen, being separated from the jugular bulb by bone, thick fibrous tissue, or thin connective tissue.

scholarly journals Surgical treatment of dumbbell-shaped jugular foramen schwannomas

2004 ◽  
Vol 17 (2) ◽  
pp. 56-62 ◽  
Author(s):  
Paulo A. S. Kadri ◽  
Ossama Al-Mefty
Keyword(s):  
Cranial Nerve ◽  
Recovery Of Function ◽  
Preoperative Evaluation ◽  
Cranial Nerves ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Senior Author ◽  
Neurological Deficits ◽  
Cystic Degeneration ◽  
Lower Cranial Nerves

Object Schwannomas of the jugular foramen are rare, comprising between 2 and 4% of intracranial schwannomas. The authors retrospectively analyzed their surgical experience with schwannomas of the lower cranial nerves that presented with intra- and extracranial extensions through an enlarged jugular foramen. The transcondylar suprajugular approach was used without sacrificing the labyrinth or the integrity of the jugular bulb. In this report the clinical and radiological features are discussed and complications are analyzed. Methods This retrospective study includes six patients (three women and three men, mean age 31.6 years) with dumbbell-shaped jugular foramen schwannomas that were surgically treated by the senior author during a 5.5-year period. One patient had undergone previous surgery elsewhere. Glossopharyngeal and vagal nerve deficits were the most common signs (appearing in all patients), followed by hypoglossal and accessory nerve deficits (66.6%). Two or more signs or symptoms were present in every patient. Three tumors presented with cystic degeneration. In four patients the jugular bulb was not patent on neuroimaging studies. The suprajugular approach was used in five patients; the origin of the tumor from the 10th cranial nerve could be defined in three of them. All lesions were completely resected. No death or additional postoperative cranial nerve deficits occurred in this series. Aspiration pneumonia developed in one patient. Preoperative deficits of the ninth and 10th cranial nerves improved in one third of the patients and half recovered mobility of the tongue. No recurrence was discovered during the mean follow-up period of 32.8 months. Conclusions With careful, extensive preoperative evaluation and appropriate planning of the surgical approach, dumbbell-shaped jugular foramen schwannomas can be radically and safely resected without creating additional neurological deficits. Furthermore, recovery of function in the affected cranial nerves can be expected.

Complex tumors of the glomus jugulare: criteria, treatment, and outcome

2002 ◽  
Vol 97 (6) ◽  
pp. 1356-1366 ◽  
Author(s):  
Ossama Al-Mefty ◽  
Aramis Teixeira
Keyword(s):  
Malignant Tumor ◽  
Cranial Nerve ◽  
Treatment Plan ◽  
Cranial Nerves ◽  
Content Type ◽  
Glomus Jugulare ◽  
Associated Lesions ◽  
Vertebrobasilar Artery ◽  
Lower Cranial Nerves ◽  
Fine Print

Object. Tumors of the glomus jugulare are benign, slow-growing paragangliomas. Their natural history, surgical treatment, and outcome have been well addressed in the recent literature; however, there remains a subgroup of complex tumors—multiple, giant, malignant, neuropeptide-secreting lesions, and those treated previously by an intervention with an adverse outcome—that is high risk, presents surgical challenges, and is associated with treatment controversy. In this article the authors report on a series of patients with complex glomus jugulare tumors and focus on treatment decisions, avoidance of complications, surgical refinements, and patient outcomes. Methods. In this retrospective study, the patient population was composed of 11 male and 32 female patients (mean age 47 years) with complex tumors of the glomus jugulare who were treated by the senior author within the past 20 years. These include 38 patients with giant tumors, 11 with multiple paragangliomas (seven bilateral and four ipsilateral), two with tumors that hypersecreted catecholamine, and one with a malignant tumor. Six patients had associated lesions: one dural arteriovenous malformation, one carotid artery (CA) aneurysm, two adrenal tumors, and two other cranial tumors. All but one patient presented with neurological deficits. Cranial nerve deficits, particularly those associated with the lower cranial nerves, were the prominent feature. Twenty-eight patients underwent resection in an attempt at total removal, and gross-total resection was achieved in 24 patients. Particularly challenging were cases in which the patient had undergone prior embolization or CA occlusion, after which new feeding vessels from the internal CA and vertebrobasilar artery circulation developed. The surgical technique was tailored to each patient and each tumor. It was modified to preserve facial nerve function, particularly in patients with bilateral tumors. Intrabulbar dissection was performed to increase the likelihood that the lower cranial nerves would be preserved. Each tumor was isolated to improve its resectability and prevent blood loss. No operative mortality occurred. In one patient hemiplegia developed postoperatively due to CA thrombosis, but the patient recovered after an endovascular injection of urokinase. In four patients a cerebrospinal fluid leak was treated through spinal drainage, and in five patients infection developed in the external ear canal. Two of these infections progressed to osteomyelitis of the temporal bone. There were two recurrences, one in a patient with a malignant tumor who eventually died of the disease. Conclusions. Despite the challenges encountered in treating complex glomus jugulare tumors, resection is indicated and successful. Multiple tumors mandate a treatment plan that addresses the risk of bilateral cranial nerve deficits. The intrabulbar dissection technique can be used with any tumor, as long as the tumor itself has not penetrated the wall of the jugular bulb or infiltrated the cranial nerves. Tumors that hypersecrete catecholamine require perioperative management and malignant tumors carry a poor prognosis.

scholarly journals Suprabulbar Approach to Jugular Fossa Tumors: 2-Dimensional Operative Video

2021 ◽  
Author(s):  
Kyle C Wu ◽  
Emad Aboud ◽  
Ossama Al-Mefty
Keyword(s):  
Doppler Ultrasonography ◽  
Cranial Nerves ◽  
Jugular Foramen ◽  
Jugular Bulb ◽  
Neurological Dysfunction ◽  
Impaired Hearing ◽  
Venous Flow ◽  
Jugular Fossa ◽  
Distal Aspect ◽  
Lower Cranial Nerves

Abstract Owing to their scarcity, location, and intricate neurovascular associations, jugular fossa tumors are among the most challenging pathologies encountered by the neurosurgeon.1 While paragangliomas originate within and often occlude the jugular bulb, schwannomas and meningiomas are extra-bulbar and typically do not impede venous flow.2 Schwannomas typically arise from an extradural origin, expanding the jugular foramen.3-5 Meningiomas are intradural and cause hyperostosis of the jugular tubercle.6 We described and have been exposing and resecting jugular fossa tumors through a presigmoid suprabulbar infralabyrinthine window6 that has been detailed in cadaveric studies.7,8 This approach maintains the patency of the jugular bulb without breaching the labyrinths or manipulating the facial nerve. It is applicable to cases with partially impaired hearing and intact lower cranial nerves. The carotid artery can be identified by neuronavigation and micro-Doppler ultrasonography. This approach provides a direct lateral trajectory with a short distance to the jugular fossa and cerebellopontine angle. Early exposure and central debulking of the tumor minimize manipulation of the exquisitely sensitive lower cranial nerves. The distal aspect of these tumors can be removed with endoscopic assisted techniques.9 The first patient is a 49-yr-old woman with a previously irradiated schwannoma who presented with worsening neurologic deficits—an extradural suprabulbar approach was used to resect this tumor. The second patient is a 27-yr-old woman with an enlarging meningioma and associated neurological dysfunction; this tumor was resected using the suprabulbar approach with opening of the presigmoid dura. Both patients have consented to surgery and publication of images. Image at 2:27 and 6:38 reprinted from Arnautović et al, with permission from JNSPG. Image at 2:50 and 6:45 ©Ossama Al-Mefty 1997, reused with permission.

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