Intradural jugular foramen tumors.

Eleven patients with jugular foramen lesions with or without extradural extension were operated at University Hospital of Campinas (UNICAMP), in Campinas, Brazil, between 1998 and 2001. Neck dissection, mastoidectomy without transposition of the facial nerve and myofascial flap reconstruction of the cranial base with an especially developed technique were carried out in 7 patients. Four patients were operated using retrosigmoid craniectomy. Total excision was accomplished in 9 cases. All patients did not show evidence of disease progression at least after 2 years follow-up. There was no mortality. New lower cranial nerve deficits occurred in 5 patients. Nine maintain or improved their preoperative status based on Karnofsky and Glasgow Outcome Scale. A complex anatomy of this region demand wide exposures for treat those tumors. For this reason, an adequate approach for curative resection of most lesions and an efficient skull base reconstruction decreasing postoperative morbidity are essential.

Related papers:

title: Radiologic evaluation of the jugular foramen. Anatomy, vascular variants, anomalies, and tumors.
abstract: The jugular foramen varies considerably in size and shape, along with the jugular vein. The foramen is traversed by several vessels and nerves. CT, in various section planes, demonstrates the bone anatomy optimally, whereas MR (including MR angiography) reveals the vascular and soft tissue structures to best advantage. A diverse group of vascular anomalies originate in the foramen and adjacent carotid canal that must be differentiated from tumors. The most common tumor within the jugular foramen is the hypervascular glomus jugulare tumor followed by neurogenic tumors, predominantly the schwannoma. Less common lesions comprise meningioma, hemangiopericytoma, chondrosarcoma, and plasmacytoma. Metastases and malignant tumors arising in adjacent anatomic structures (nasopharynx, parotid, and temporal bone), in advanced stages, may spread to the jugular foramen. Endolymphatic sac tumors arise at the posterior medial aspect of the petrous bone and frequently extend to the jugular foramen. Irregular lytic bone destruction, with enlargement and hypervascularity, demonstrated by CT and MR imaging, are characteristic for glomus jugulare tumors. Benign tumors, most commonly the jugular foramen schwannoma, display an enlarged jugular foramen with well-defined bone margins.

title: The transcondylar approach to the jugular foramen: a comparative anatomic study.
abstract: The standard neurosurgical approach to the jugular foramen involves suboccipital craniectomy with access along the petrous bone. However, even after wide removal of the foramen magnum, only limited access into the infratemporal fossa can be obtained. The neurootologic exposures provide excellent infratemporal access but limited exposure to the posterior fossa, resulting in hearing loss and facial paresis or paralysis. Using cadaver specimens, we exposed the jugular foramen region by the transcondylar approach. A retromastoid incision is extended into the neck. The transverse foramen of the atlas is opened and the vertebral artery transposed medially, thereby providing exposure into the infratemporal fossa. A suboccipital craniectomy extending anterior to the sigmoid sinus is performed, and the posterolateral occipital condyle is resected. After resection of the sigmoid sinus, cranial nerves 9 through 12 are easily identified extracranially in the infratemporal fossa and can be followed proximally through their foramina to the brain stem. We compared the transcondylar approach to three standard approaches, morphometrically and anatomically, and found that the transcondylar approach not only compares favorably but also offers advantages in that it preserves auditory and facial nerve function and is useful for one-stage tumor resection.

title: Metastatic Carcinoma to the Jugular Foramen
abstract: Glomus jugulare tumors are the most common lesions of the jugular foramen. Other neoplasms, including meningioma, schwannoma, carcinoma, sarcoma, and metastatic lesions, are encountered occasionally in this region. As generally accepted, the clinical presentations and radiologic findings of glomus tumors differ from those of metastatic malignant lesions. Glomus jugulare tumor was originally diagnosed by CT and MRI in a patient admitted to our hospital. However, the carotid arteriography failed to disclose the classic picture of a glomus jugulare tumor. Subsequently, an incisional biopsy through the right upper neck was performed. The pathologic findings revealed a metastatic adenocarcinoma. Primary lung cancer with metastasis to the temporal bone was the final diagnosis.

title: Giant cell tumors of the jugular foramen.
abstract: To review the diagnosis and treatment of giant cell tumors of the jugular foramen.



A typical case is reported. Symptoms, signs, and diagnostic studies are reviewed. Photomicrographs and angiographic studies showing the differences between these and glomus jugulare tumors are provided. A coherent approach to their management is presented.



These hypervascular, traditionally radioresistant tumors may cause pulsatile tinnitus, conductive hearing loss, and lower cranial nerve paresis. Angiographic studies showed a hypervascular lesion supplied by numerous small branches of the external carotid artery, making embolization difficult. Complete resection was achieved by an infratemporal fossa approach with preoperative embolization.



Giant cell tumors of the temporal bone may mimic glomus jugulare tumors with respect to anatomic location, cranial nerve deficits, and vascularity.

title: Endolymphatic sac tumors: report of three cases.
abstract: We present three cases of endolymphatic sac tumors and review the previously published cases. Despite frequent extension to the cerebellopontine angle, these rare tumors have only recently been recognized by neurosurgeons.



A 26-year-old man developed a progressive hearing loss, revealing an intrapetrous retrolabyrinthine tumor on the right side. A 28-year-old woman experienced a left cerebellopontine angle syndrome, with a lytic intrapetrous mass extending into the cerebellopontine angle. A 38-year-old woman presented with an intracranial hypertension syndrome caused by a tumor of the jugular foramen.



For the first and second patients, the tumors originated from the operculum of the endolymphatic sac. Total removal was achieved, via a transpetrosal approach, in these two cases. No recurrence was detected after a 20-month follow-up period. For the third patient, the tumor originated from the distal part of the sac. Recurrence was observed 8 years after subtotal removal via a retrosigmoid route. Histological analysis revealed a papillary-cystic adenocarcinomatous pattern in all cases, without features of aggressiveness.



Endolymphatic sac tumors are locally invasive neoplasms characterized by bipolar intrapetrous and posterior fossa involvement. The anatomic complexity of the endolymphatic sac may explain the distinct patterns of extension of these tumors. Early radical surgery is related to good outcomes.

title: Chondrosarcoma of the jugular foramen.
abstract: Chondrosarcoma, although uncommon, must be included in the differential diagnosis of skull base tumors. The potential for morbidity and mortality, similar to other lesions located at this anatomic site, is related to compromise of adjacent vital neurovascular structures. Optimal surgical management is controversial and reliant on adequate tumor exposure. A case of myxoid chondrosarcoma of the jugular foramen is presented with a review of the common presenting signs and symptoms along with the radiographic and histologic features of these lesions. The surgical approach to chondrosarcoma and other lesions at this location has undergone an evolution in technique in recent years. This report details the approach used by the authors, which allows for reconstruction of the skull base with subsequent normal anatomic contour and functional hearing. The technique employs hydroxylapatite for reconstruction, which has not been previously described in skull base applications.

title: Amyloidomas in the cerebellopontine angle and jugular foramen. Case report.
abstract: The 47-year-old man reported here showed large encapsulated masses in the left cerebellopontine angle and 6 years later in the enlarged left jugular foramen. Histologically, the tumors demonstrated a large deposit of amyloid composed of immunoglobulin light chain-derived proteins (AL). There was no evidence of chronic inflammatory or infectious processes or immunoglobulin abnormalities.

title: Jugular foramen schwannomas.
abstract: Thirteen patients with schwannomas of the jugular foramen were operated on at the Cleveland Clinic between 1974 and 1983. The authors' experience in managing these rare tumors is presented. Three major growth patterns of jugular foramen schwannoma were seen, and it is postulated that the position of the tumor depends on its point of origin from the nerves as they pass through the pars nervosa of the jugular foramen. The more distal lesions will expand inferiorly out of the base of the skull, and the more proximal lesions will enlarge into the posterior fossa. Tumors in the mid region will tend to expand primarily into bone. The schwannoma was primarily intracranial in six patients. In five patients the tumor expanded the bone at the base of the skull, with only a small intracranial component, and in two patients the tumor was primarily extracranial, with a small extension into the bone or posterior fossa. The presentation of the patients varied according to the tumor growth pattern. Deafness, vertigo, and ataxia were present in all patients with a major intracranial component, and in most of these there were only minimal deficits of the jugular foramen nerves. By contrast, lower cranial nerve involvement, including hoarseness and weakness of the trapezius and sternocleidomastoid muscles, occurred in patients in whom the tumor was primarily, within the bone or extracranial. Three of the five patients with the major component of the schwannoma within the bone also had deafness. Symptomatic history was longest in those with tumor mainly involving the bone at the base of the skull, and shortest in patients with entirely extracranial tumor. Surgical resection was accomplished with a joint neurosurgical-otological approach, usually combining a posterior fossa exploration with either a translabyrinthine transcochlear or infralabyrinthine procedure. The exact nature of the operation depended upon the presence of intracranial tumor and on the extent of bone or extracranial involvement. Total excision was performed in all cases. There was no operative mortality, and surgery resulted in loss of function of the ninth, 10th, and 11th cranial nerves in most patients. The major postoperative morbidity consisted of swallowing difficulties and sputum aspiration.

title: Surgical treatment of jugular foramen schwannomas.
abstract: Sixteen patients with schwannomas of the jugular foramen were operated on in the Department of Neurosurgery of the Nordstadt Hospital in Hannover, Germany, between 1986 and 1992. Patients with neurofibromatosis were excluded. The records of the 16 patients were retrospectively reviewed. There were five women and 11 men (mean age 43 years) with a symptom duration ranging from 6 months to 20 years (mean 5 years). The predominant symptoms were hearing loss, hoarseness, and cerebellar symptoms. Computerized tomography (CT), magnetic resonance (MR) imaging, and angiography were performed in all 16 patients. Depending on the radiological and surgical features, the tumors were classified into four types: Type A, a tumor primarily at the cerebellopontine angle with minimal enlargement of the jugular foramen (eight cases); Type B, a tumor primarily at the jugular foramen with intracranial extension (two cases); Type C, a primarily extracranial tumor with extension into the jugular foramen (one case); and Type D, a dumbbell-shaped tumor with both intra- and extracranial components (five cases). A retromastoid suboccipital craniectomy was performed for Type A tumors, and a combined cervical-mastoidectomy for Types B, C, and D. Total tumor removal was achieved in all cases. There was no operative mortality. Postoperative complications were cerebrospinal fluid leakage in one patient and mastoiditis in two patients. The follow-up period ranged from 12 to 42 months (mean 22 months). All patients were alive at the last follow-up review, and CT and/or MR imaging showed no tumor recurrence.

title: The management of dysphagia in jugular foramen surgery.
abstract: From 1985-1994, the Skull Base Unit at St. Vincent's Hospital, Sydney, operated on 61 patients with tumours involving the jugular foramen. Pre-operative assessment by a Speech Pathologist and the institution of swallowing techniques prior to surgery have improved post-operative morbidity. Ancillary procedures at the time of surgery were not required in the majority of cases. An individual assessment of each patient early in the postoperative period was found to be more important with regard to the benefits of supplementary surgery. The majority of patients with dysphagia settled with conservative management and only a few underwent ancillary surgery. It is perceived that the cortical and subcortical control of swallowing is a major factor in the rehabilitation of these patients.

title: Neurovascular considerations in surgery of glomus tumors with intracranial extensions
abstract: Paragangliomas of the skull base, by virtue of their location, locally infiltrative behavior, and vascular nature, are difficult tumors to resect. Surgical removal is especially complicated when intracranial extensions are encountered. Our experience with a one-stage resection of intracranial extensions of glomus tumors in 20 patients is presented. These 20 patients had a total of 29 paragangliomas: 23 glomus jugulare or tympanicum tumors, 5 carotid body tumors, and 1 pterygopalatine lesion. Ten patients had intradural extension; the other 10 had intracranial extradural tumors. The primary complicating treatment factor was the loss of surgical planes in 6 patients with prior surgery and or radiotherapy. The presence of multiple paragangliomas (20%) and catecholamine secretion by the tumors (15%) complicated surgical treatment as well. Surgical morbidity was primarily related to deficits of lower cranial nerves (50%).

title: Jugular Foramen: Microscopic Anatomic Features and Implications for Neural Preservation with Reference to Glomus Tumors Involving the Temporal Bone
abstract: OBJECTIVEOur goals were to study the normal histological features of the jugular foramen, compare them with the histopathological features of glomus tumors involving the temporal bone, and thus provide insight into the surgical management of these tumors with respect to cranial nerve function. METHODSTen jugular foramen blocks were obtained from five human cadavers after removal of the brain. Microscopic studies of these blocks were performed, with particular attention to fibrous or bony compartmentalization of the jugular foramen, the relationships of the caudal cranial nerves to the jugular bulb/jugular vein and internal carotid artery, and the fascicular structures of the nerves. In addition, we studied the histopathological features of 11 glomus tumors involving the temporal bone (10 patients), with respect to nerve invasion, associated fibrosis, and carotid artery adventitial invasion. RESULTSA dural septum separating the IXth cranial nerve from the fascicles of Cranial Nerves X and XI, at the intracranial opening, was noted. Only two specimens, however, had a septum (one bony and one fibrous) producing internal compartmentalization of the jugular foramen. The cranial nerves remained fasciculated within the foramen, with the vagus nerve containing multiple fascicles and the glossopharyngeal and accessory nerves containing one and two fascicles, respectively. All of these nerve fascicles lay medial to the superior jugular bulb, with the IXth cranial nerve located anteriorly and the XIth cranial nerve posteriorly. All nerve fascicles had separate connective tissue sheaths. A dense connective tissue sheath was always present between the IXth cranial nerve and the internal carotid artery, at the level of the carotid canal. The inferior petrosal sinus was present between the IXth and Xth cranial nerves, as single or multiple venous channels. The glomus tumors infiltrated between the cranial nerve fascicles and inside the perineurium. They also produced reactive fibrosis. In one patient, in whom the internal carotid artery was also excised, the tumor invaded the adventitia. CONCLUSIONWithin the jugular foramen, the cranial nerves lie anteromedial to the jugular bulb and maintain a multifascicular histoarchitecture (particularly the Xth cranial nerve). Glomus tumors of the temporal bone can invade the cranial nerve fascicles, and infiltration of these nerves can occur despite normal function. In these situations, total resection may not be possible without sacrifice of these nerves.

title: Surgical management of previously untreated glomus jugulare tumors
abstract: The treatment of glomus jugulare tumors is controversial. Changes in the surgical treatment of glomus jugulare tumors at The House Ear Clinic have allowed complete resection in 85% of patients with minimal morbidity and no surgical mortalities. Our experience with 52 previously untreated patients with glomus jugulare tumors is reviewed.

title: Panel discussion: Glomus jugulare tumors of the temporal bone. The surgical management of glomus tumors
abstract: The authors describe the diagnosis and surgical management of 70 glomus tumors seen at the Otology Group in an 8 yr. period. The need for bilateral arteriograms to detect associated tumors (carotid body and intervagale) is stressed.

title: Panel discussion: Glomus jugulare tumors of the temporal bone. Patterns of invasion in the temporal bone
abstract: The purpose of this paper is to enumerate and define the exact patterns of glomus jugulare tumor invasion in the temporal bone. Special attention will be focused on those pathways which lead to central nervous system (CNS) invasion.

title: The microsurgical anatomy of the jugular foramen.
abstract: The microsurgical anatomy of the jugular foramen was studied in 10 fixed cadavers, each cadaver consisting of the whole head and neck. Five of the cadavers were injected with latex. The jugular foraminal region was exposed using the infratemporal fossa type A approach of Fisch and Pillsbury in five cadavers (10 sides) and the combined cervical dissection-mastoidectomy-suboccipital craniectomy approach in five cadavers (10 sides). The right foramen was larger than the left in seven cases (70%), equal in two cases (20%), and smaller in one case (10%). The dura covering the intracranial portal of the foramen had two perforations, a smaller anteromedial perforation through which passed the ninth cranial nerve (CN IX), and a larger posterolateral perforation, through which passed the 10th and 11th cranial nerves (CNs X and XI) and the distal sigmoid sinus. The perforations were separated by a fibrous septum in 16 specimens (80%). After exiting the posterior fossa, CNs IX, X, and XI all lay anteromedial to the superior jugular bulb (SJB) within the jugular foramen. The inferior petrosal sinus (IPS) entered the foramen between CNs IX and X in most cases; however, in 10% of our cases it entered the foramen between CNs X and XI, and in 10% it entered the foramen caudal to CN XI. The IPS terminated in the SJB in 90% of our cases; in 40%, the IPS termination consisted of multiple channels draining into both the SJB and internal jugular vein. This study shows that the arrangement of the neurovascular structures within the jugular foramen does not conform to the hitherto widely accepted notion of discrete compartmentalization into an anteromedial pars nervosa containing CN IX and the IPS and a posterolateral pars venosa containing the SJB, CNs X and XI, and the posterior meningeal artery.

title: Complications in the management of large glomus jugulae tumors
abstract: This study analyzes the complications encountered in the surgical treatment of 17 patients with large glomus jugulare tumors. All 17 patients sustained either new cranial nerve palsies or exacerbation of preexisting palsies. These involved, in descending order of frequency, the facial nerve, the vagus and glossopharyngeal nerves, and the hypoglossal nerve. Post‐operative palsies of the facial nerve involved 17 patients, as compared to 5 preoperatively. Fifteen patients had postoperative partial or complete paralysis of the vagus nerve as compared to eight preoperatively. Ten patients had postoperative palsies of the hypoglossal nerve as compared to six preoperatively. Other complications included CSF leak, meningitis, and wound infection. Aspiration and dysphagia were encountered postoperatively in 13 patients. Teflonr̀ injection of paretic vocal cords and cricopharyngeal myotomy effectively improved the ability to swallow and the quality of the voice. Prompt recognition and treatment of complications is essential for effective surgical management of large glomus jugulare tumors.

title: Glomus tumors: diagnosis and treatment
abstract: Glomus tumors can be classified as to their origin in the temporal bone. Those limited to the middle ear and mastoid are referred to as tympanicum tumors while those originating in the jugular bulb are known as jugulare tumors. Diagnostic studies such as polytomes of the temporal bone, retrograde venograms and arteriograms now make it possible for the surgeon to determine not only the origin of the tumor but also the extent of its involvement in the temporal bone and base of the skull.

title: Removal of jugular foramen tumors: the fallopian bridge technique.
abstract: Despite recent advances in neuroradiographic and electrophysiologic assessment, the surgical extirpation of lesions of the bony skull base remains challenging. Moreover, as surgeons have gained experience in removing tumors from the irregular osteologic confines of the skull base, attention has been directed toward preservation of vital neural and vascular structures traversing the operative field. This report describes the creation of a fallopian bridge with preservation of the facial nerve in removing tumors that arise within or juxtaposed to the jugular fossa. Thirty-five patients are reported herein with analysis of pathology, surgical approach, and outcome. An algorithm for use of the fallopian bridge, as opposed to facial nerve mobilization and rerouting, is presented with particular emphasis on limitation of this selective procedure.