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  • A Classification Scheme for Large Glomus Jugulare Tumors Extending Intracranially: Experience with 22 Patients

    Authors

    Michael T. Lawton, MD*
    Suresh Sankhla, MD†
    Talat Kiris, MD††

    C. Phillip Daspit, MD

    Michael J. Holliday, MD‡
    Donlin M. Long, MD, PhD‡
    Robert F. Spetzler, MD

    Division of Neurological Surgery, Barrow Neurological Institute, Mercy Healthcare Arizona, Phoenix, Arizona
    ‡Johns Hopkins University School of Medicine, Baltimore, MD
    Current Addresses: *Department of Neurological Surgery, University of California, San Francisco, California
    †Department of Neurosurgery, Bhatia General Hospital, Bombay, India
    ††Department of Neurosurgery, University of Istanbul, Istanbul, Turkey

    Abstract

    A classification for large glomus jugulare tumors describing their pattern of intracranial extension was devised and applied to a series of 22 patients (9 men, 13 women; mean age, 45 years). Type I tumors grew concentrically about the jugular bulb (n=3); Type II tumors extended posterolaterally into the cerebellopontine angle (n=10); Type III tumors extended inferiorly into the foramen magnum (n=2); Type IV tumors extended anteriorly into the infrazygomatic fossa (n=5); and Type V tumors extended superomedially into the posterior cavernous sinus (n=2). All tumors were resected surgically by a transpetrosal approach. A retrosigmoid approach was added to expose Type II tumors, a far-lateral approach for Type III tumors, an infrazygomatic approach for Type IV tumors, and a complete petrosectomy with a transcochlear approach for Type V tumors. The pattern of intracranial growth reflected by the proposed classification provides useful information regarding the clinical presentation, selection of surgical approach, and treatment outcome of glomus jugulare tumors.

    Key Words : glomus jugulare, tumors

    Glomus jugulare tumors arise from the paraganglionic tissue located in the wall of the jugular bulb and along Jacobson’s nerve.2,3,10,11,21,25,33 They grow slowly and in multiple directions, compressing adjacent cranial nerves and invading vascular structures. Typically, these tumors are associated with pulsatile tinnitus, hearing loss, or a middle ear mass, and patients tend to seek treatment from otolaryngologists. As a result, the literature on glomus jugulare tumors mostly has been written by otologists, and the nomenclature describing surgical approaches to these tumors can be confusing for neurosurgeons.4,7-9,14-17,28,32

    When patients with glomus jugulare tumors do present to neurosurgeons, their tumors typically extend intracranially. The nomenclature for neurosurgical approaches to this region differs from that describing otological approaches.1,19,23,24,31 A unified nomenclature describing surgical approaches to large glomus jugulare tumors with an intracranial extension that is accepted and understood by both neurosurgeons and otologists would be helpful, particularly since treatment usually involves a combined otological-neurosurgical operation.

    Because large glomus jugulare tumors with an intracranial extension are relatively rare, related neurosurgical series tend to be small. Therefore, we examined two experiences with large glomus jugulare tumors from two institutions (Barrow Neurological Institute and the Johns Hopkins University School of Medicine), yielding a combined series of 22 patients. We introduce a classification scheme that describes the pattern of intracranial growth and consequently helps select the optimal surgical approach to a tumor. We also examined the various surgical approaches in an attempt to standardize terminology.

    Clinical Materials and Methods

    Twenty-two patients with glomus jugulare tumors with intracranial extensions were treated surgically (Table 1). Twelve patients were treated at the Johns Hopkins Hospital between 1979 and 1990, and 10 patients were treated at the Barrow Neurological Institute between 1982 and 1995. There were 9 men and 13 women with a mean age of 44.5 years (range, 9 to 76 years).

    Patients presented with a variety of symptoms and signs (Table 2). The interval from symptom onset to presentation ranged from 2 months to 17 years (mean, 4.1 years). Ten patients underwent prior treatment at outside institutions with biopsy (n=4), biopsy and radiation therapy (n=1), or surgical resection (n=5).

    Fifteen patients had tumor on the left side, six had tumor on the right side, and one had bilateral tumors. Based upon preoperative computed tomography (CT) or magnetic resonance (MR) imaging, 5 patients had Jenkins-Fisch Class C tumors and 17 patients had Class D tumors.17 By the Jackson-Glasscock classification system,16 7 patients had Grade III tumors and 15 patients had Grade IV tumors.

    We categorized the pattern of intracranial growth of large glomus jugulare tumors into five types (Table 3). Type I tumors grew concentrically around the jugular bulb and were usually small. Type II tumors extended posterolaterally into the cerebellopontine angle.Type III tumors extended inferiorly into the foramen magnum. Type IV tumors extended anteriorly through the petrous bone and into the infrazygomatic fossa. Type V tumors extended medially and superiorly toward the clivus and cavernous sinus. Based on this classification system, there were 3 Type I tumors, 10 Type II tumors, 2 Type III tumors, 5 Type IV tumors, and 2 Type V tumors.

    The internal carotid artery (ICA) was encased by tumor in eight patients and the vertebral artery was encased in one patient. In none of these patients was the artery stenosed or occluded.

    Results

    Embolization

    All patients underwent preoperative transfemoral embolization of feeding arteries. The most common feeding arteries were the ascending pharyngeal, occipital, posterior auricular, internal maxillary, and middle meningeal arteries. Seven tumors had feeding arteries from the ICA, but these small caliber vessels were not embolized.

    Surgical Outcomes

    Surgical approaches were categorized into those performed by the neuro-otologists and those performed by neurosurgeons. The neuro-otologists had performed 10 retrolabyrinthine exposures, 5 translabyrinthine exposures, and 7 transcochlear exposures. The neurosurgeons had performed 18 retrosigmoid approaches and 1 far-lateral approach. Additional exposure from a craniotomy was not required in three patients.

    Facial nerve management consisted of skeletonization in nine patients, mobilization of the mastoid segment in four patients (partial transposition), and complete transposition in seven patients. In two patients tumor had invaded the facial nerve. A segment of the nerve was resected with tumor and the nerve was repaired with a graft. The glossopharyngeal and vagus nerves were anatomically preserved in 18 patients, resected with tumor in 4 patients, and repaired in 2 patients with direct reanastomosis (1 patient) or grafting (1 patient). The spinal accessory nerve was resected with tumor in two patients. The ICA and vertebral arteries were preserved in the eight patients in whom these vessels were encased.

    Gross total tumor resection was achieved in 19 patients (86%). In three patients, the tumor resection was subtotal because tumor encased the cranial nerves. These three patients underwent radiation therapy. There were no surgical deaths in the series.

    Complications

    Two patients had permanent treatment-associated neurological deterioration (surgical morbidity, 9%). One patient suffered a brain stem infarction during preoperative embolization of his tumor. The other patient developed a postoperative epidural hematoma and deteriorated despite evacuation. Seven patients (32%) required a tracheostomy, seven (32%) required a feeding jejunostomy, and one (5%) required a lumboperitoneal shunt (Table 4).

    Patient Outcomes

    According to the Glasgow Outcome Scale (GOS), 7 patients (32%) made a good recovery (GOS 1), 13 patients (59%) had moderate deficits (GOS 2), 1 patient (5%) had severe deficits (GOS 3), and 1 patient (5%) died (GOS 5). Overall, neurological status improved by one GOS score in 9 patients (41%, Table 5). The mean duration of follow-up was 3.7 years.

    At late follow-up, facial nerve function had improved relative to preoperative function in 6 patients (27%), was unchanged in 13 patients (59%), and worsened in 3 patients (14%) according to the House-Brackmann grading system13 (Table 6). Facial nerve function is best protected by leaving the nerve in its bony canal. In four patients, partial transposition of the facial nerve did not worsen its function. Complete transposition of the facial nerve, however, did worsen facial nerve function, and the deterioration was permanent in three of seven patients. In the other four patients, facial nerve function either improved (one patient) or returned to its preoperative baseline (three patients). Of the two patients who underwent grafting of the facial nerve, its function improved in one.

    Tumor recurred in three patients (14%) at a mean of 3.7 years after surgery. Two of these tumors had been judged to be totally resected at surgery. The tumor judged to be subtotally resected had received radiation therapy. All three patients were treated for their tumor recurrences. One patient underwent reoperation for gross total tumor resection and was free of tumors 4.3 years after his second operation. One patient underwent reoperation, but the tumor recurred again and he ultimately died from progressive tumor growth 5.2 years after his initial resection. One patient’s recurrent tumor was irradiated and has remained stable as judged by radiography.

    Two additional patients died of causes unrelated to their glomus jugulare tumors: one from a colon carcinoma 13 months after resection and the other from an undetermined cause 5.5 years after surgery.

    Patients with Type I, II, and V tumors tended to have the best outcome scores (Table 7). In contrast, outcomes in patients with Type III and IV tumors were not as good.

    Discussion

    Classification Systems for Glomus Jugulare Tumors

    A classification system is needed for large glomus jugulare tumors with intracranial extension that provides greater detail about the pattern of extension. Such a classification might elucidate the clinical constellation of presenting symptoms, assist in the selection of surgical approach, and standardize discussions about the outcomes associated with these tumors. The classification presented in this report attempts to accomplish these objectives.

    Several classification systems have been proposed. In 1971, Kempe and coworkers19 introduced a classification system based on angiographic information, specifically, the size and vascular supply of the tumor. With contemporary imaging modalities, this classification system has become obsolete. Two similar classification systems, one by Jenkins and Fisch17 and one by Jackson, Glasscock and coworkers,16 have become the current standards.

    The Jenkins-Fisch system17 classifies glomus jugulare tumors into four types depending on the tumor’s size and relationship to petrous anatomy. Type A tumors are limited to the middle ear cleft. Type B tumors are limited to the tympanomastoid area. Type C tumors involve the infralabyrinthine compartment and extend to the petrous apex. Type D tumors extend intracranially and are further subdivided by diameter into Types D1 (< 2 cm) and D2 (> 2 cm).

    The Jackson-Glasscock system16 also classifies glomus jugulare tumors into four types depending on their size and relationship to petrous anatomy. Type I tumors involve the jugular bulb, middle ear, and mastoid. Type II tumors extend under the internal auditory canal. Type III tumors extend into the petrous apex and might extend intracranially. Type IV tumors extend beyond the petrous apex into the clivus or infrazygomatic fossa and might extend intracranially. These two widely used classification systems recognize the presence of intracranial extension but fail to detail the patterns of intracranial extension.

    A recent classification from de la Cruz5 also is based on the anatomic extent of the lesion and categorizes tumors as tympanic, tympanomastoid, jugular bulb, carotid artery, or transdural. Again, intracranial extension is not described in detail. This classification, however, does correlate the anatomic classification with the appropriate surgical approach. The approaches recommended for these five tumor types are transcanal, mastoid-extended facial recess, mastoid-neck, infrazygomatic fossa, and infrazygomatic-intracranial, respectively.

    The classification system proposed here details the direction of intracranial extension, which often determines the clinical constellation of presenting symptoms. The classification system also guides surgical planning by correlating intracranial tumor location with selection of surgical approach.

    Nomenclature

    The word temporal is used to describe brain parenchyma (temporal lobe), bone (temporal bone), and an extracranial anatomic space (infratemporal fossa). These various meanings associated with the word temporal have been the source of considerable confusion. The overall lack of uniformity in anatomical terminology also hampers communication.6,7,12,14,19,20,23,26-28 For example, the temporal fossa is also referred to as the middle fossa; the infratemporal fossa is also referred to as the zygomatic or infrazygomatic fossa.

    To eliminate this confusion, we use temporal to refer only to the temporal lobe of the brain. Petrous is used instead of temporal to describe the petrous portion of the temporal bone. Middle is used instead of temporal to describe the bone that comprises the floor of the middle fossa. Infrazygomatic is used instead of infratemporal to describe the extracranial space beneath the middle fossa floor.

    Proposed Classification System and Surgical Approach

    Various surgical approaches have been proposed for the exposure and resection of large glomus jugulare tumors. All of these approaches involve (1) a neck dissection, with exposure of the ICA, external carotid artery, internal jugular vein, and cranial nerves IX through XII; (2) a mastoidectomy, with exposure of the sigmoid sinus, jugular bulb, and skeletonization of the facial nerve; and (3) a craniotomy to access the portion of the tumor that extends intracranially.

    In the classification system proposed here, Type I glomus jugulare tumors are smaller than other types of tumors and often can be approached with just a transpetrosal exposure.18,29 The retrolabyrinthine exposure is usually adequate, but a translabyrinthine exposure may be needed for larger tumors. Unlike the other types of tumors in the classification system, a craniotomy is not required for Type I tumors.

    Type II tumors are approached with a transpetrosal exposure and a retrosigmoid exposure. The extent of petrous bone removed again depends on the size of the tumor and the patient’s preoperative deficits. The retrosigmoid craniotomy adds exposure for the lateral extension of the tumor.

    Type III tumors are rare—there were just two such tumors in this series. The addition of a far-lateral exposure30 provides the necessary access inferior to the tumor extension into the foramen magnum.

    For tumor Types I, II, and III, the petrous bone removed with either a retro- or translabyrinthine exposure is usually adequate for tumor resection. However, tumor Types IV and V extend anteriorly, medially, or both and require the additional anterior and medial exposure of a complete petrosectomy. Therefore, the transcochlear exposure is used to reach these tumors. Type IV tumors may sometimes require the additional exposure of the infrazygomatic fossa that is created by a Fisch approach.7 Type V tumors can climb the clivus to the extent that a pterional or orbitozygomatic craniotomy22 may be needed to access tumor tissue that extends into the cavernous sinus. However, none of the Type V tumors treated in this patient series required this extra exposure.

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