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

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.

2002 ◽  
Vol 97 (6) ◽  
pp. 1356-1366 ◽  
Author(s):  
Ossama Al-Mefty ◽  
Aramis Teixeira

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.


2004 ◽  
Vol 17 (2) ◽  
pp. 56-62 ◽  
Author(s):  
Paulo A. S. Kadri ◽  
Ossama Al-Mefty

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.


2000 ◽  
Vol 93 (4) ◽  
pp. 586-593 ◽  
Author(s):  
Johann Romstöck ◽  
Christian Strauss ◽  
Rudolf Fahlbusch

Object. Electromyography (EMG) monitoring is expected to reduce the incidence of motor cranial nerve deficits in cerebellopontine angle surgery. The aim of this study was to provide a detailed analysis of intraoperative EMG phenomena with respect to their surgical significance.Methods. Using a system that continuously records facial and lower cranial nerve EMG signals during the entire operative procedure, the authors examined 30 patients undergoing surgery on acoustic neuroma (24 patients) or meningioma (six patients). Free-running EMG signals were recorded from muscles targeted by the facial, trigeminal, and lower cranial nerves, and were analyzed off-line with respect to waveform characteristics, frequencies, and amplitudes. Intraoperative measurements were correlated with typical surgical maneuvers and postoperative outcomes.Characteristic EMG discharges were obtained: spikes and bursts were recorded immediately following the direct manipulation of a dissecting instrument near the cranial nerve, but also during periods when the nerve had not yet been exposed. Bursts could be precisely attributed to contact activity. Three distinct types of trains were identified: A, B, and C trains. Whereas B and C trains are irrelevant with respect to postoperative outcome, the A train—a sinusoidal, symmetrical sequence of high-frequency and low-amplitude signals—was observed in 19 patients and could be well correlated with additional postoperative facial nerve paresis (in 18 patients).Conclusions. It could be demonstrated that the occurrence of A trains is a highly reliable predictor for postoperative facial palsy. Although some degree of functional worsening is to be expected postoperatively, there is a good chance of avoiding major deficits by warning the surgeon early. Continuous EMG monitoring is superior to electrical nerve stimulation or acoustic loudspeaker monitoring alone. The detailed analysis of EMG-waveform characteristics is able to provide more accurate warning criteria during surgery.


2014 ◽  
Vol 120 (2) ◽  
pp. 377-381 ◽  
Author(s):  
Brandon G. Gaynor ◽  
Mohamed Samy Elhammady ◽  
Daniel Jethanamest ◽  
Simon I. Angeli ◽  
Mohammad A. Aziz-Sultan

Object The resection of glomus jugulare tumors can be challenging because of their inherent vascularity. Preoperative embolization has been advocated as a means of reducing operative times, blood loss, and surgical complications. However, the incidence of cranial neuropathy associated with the embolization of these tumors has not been established. The authors of this study describe their experience with cranial neuropathy following transarterial embolization of glomus jugulare tumors using ethylene vinyl alcohol (Onyx, eV3 Inc.). Methods The authors retrospectively reviewed all cases of glomus jugulare tumors that had been treated with preoperative embolization using Onyx at their institution in the period from 2006 to 2012. Patient demographics, clinical presentation, grade and amount of Onyx used, degree of angiographic devascularization, and procedural complications were recorded. Results Over a 6-year period, 11 patients with glomus jugulare tumors underwent preoperative embolization with Onyx. All embolization procedures were completed in one session. The overall mean percent of tumor devascularization was 90.7%. No evidence of nontarget embolization was seen on postembolization angiograms. There were 2 cases (18%) of permanent cranial neuropathy attributed to the embolization procedures (facial nerve paralysis and lower cranial nerve dysfunction). Conclusions Embolizing glomus jugulare tumors with Onyx can produce a dramatic reduction in tumor vascularity. However, the intimate anatomical relationship and overlapping blood supply between these tumors and cranial nerves may contribute to a high incidence of cranial neuropathy following Onyx embolization.


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

2020 ◽  
Vol 13 (7) ◽  
pp. e234949
Author(s):  
Trishal Jeeva-Patel ◽  
Edward A Margolin ◽  
Daniel Mandell

Dolichoectasia refers to distinct elongation, dilatation and tortuosity of an artery. We present a rare well-illustrated case of dolichoectatic vertebrobasilar artery compressing the cisternal portion of the sixth cranial nerve resulting in chronic sixth nerve palsy. High spatial resolution, three-dimensional, heavily T2-weighted MRI sequences are uniquely positioned to assess the cranial nerves especially in their cisternal and canalicular portions and need to be performed for all patients with non-resolving cranial nerve palsies. Dolichoectatic vessels can be the cause of neurovascular conflict and cause non-resolving oculomotor palsies.


Author(s):  
Gregg MacLean ◽  
Alan Guberman ◽  
Antonio Giulivi

ABSTRACT:Dysarthria, dysphagia and repeated aspiration in a 54-year-old woman diagnosed and treated for myasthenia gravis 7 years earlier were initially thought to represent a late exacerbation of myasthenia. A cervical mass invading the jugular foramen and causing multiple lower cranial nerve palsies was biopsied and found to represent invasive ectopic thymoma.


2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Angelica A. Nunez ◽  
Luis R. Ramos-Duran ◽  
Albert C. Cuetter

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.


2004 ◽  
Vol 17 (2) ◽  
pp. 1-5 ◽  
Author(s):  
L. Madison Michael ◽  
Jon H. Robertson

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.


2020 ◽  
Vol 2 (2) ◽  
pp. V14
Author(s):  
Ezequiel Goldschmidt ◽  
Philippe Lavigne ◽  
Carl Snyderman ◽  
Paul A. Gardner

This video depicts the case of a 59-year-old woman that presented to the emergency department with the worst headache of her life. CT showed subarachnoid hemorrhage and digital subtraction angiogram demonstrated a right-side posterior inferior cerebellar artery (PICA) aneurysm. Given the medial and ventral position of the aneurysm, deep to the lower cranial nerves, which obviated distal control from an open approach, and the absence of an endovascular option able to reliably preserve the PICA, an endonasal approach was offered. A far medial approach was performed, and the aneurysm was successfully clipped. The patient developed a postoperative CSF leak with persistent posthemorrhagic hydrocephalus treated with reexploration and an eventual ventriculoperitoneal shunt. The patient was discharged without neurological deficits.The video can be found here: https://youtu.be/_9hsM2CaMow.


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