Ann Otol 87 :1978
GLOMUS JUGULARE AND VAGALE JOSEPH GERSHON
H. OGURA,
MD
J. SPECTOR, MD
MOKHTAR GADO, MD
ST. LOUIS,
MISSOURI
The therapeutic results in 72 patients with glomus jugulare and nine with glomus vagale have indicated a surgical cure rate of 80% in the former and 100% in the latter. Radiotherapy demonstrated a 65% tumor response rate and a 25% cure rate histologically and clinically. Embolization with Gelfoam® beads in seven patients resulted in diminished tumor size and arterial circulation from the external carotid system. However, in all cases the tumor persisted. Embolization diminished intraoperative bleeding. Nevertheless, the use of embolization as a palliative modality demonstrated significant symptomatic relief in aged, debilitated patients and in all patients with large inoperable glomus jugulare tumors.
Glomus tumors or chemodectomas arise from the chemoreceptor system found along the jugular bulb, middle ear, vagus nerve and chest.':" They are the most common neoplasms of the middle ear. Many aspects of this article have been previously published.v-" This paper updates our results. In addition, we will emphasize embolization'< which can serve a useful purpose, in some cases, prior to resection and perhaps may be an adjunctive therapy for symptomatic relief in those tumors which are not resectable or in debilitated patients.
paralyzed. Less often VIII (labyrinthine invasion), IX, X, XI, XII, or sympathetic nerve paralysis occurred (Table 3). Bleeding from the ear or a protruding polyp in the external auditory canal was present in about one third of the cases of glomus jugulare tumors (Table 1). A mass was palpated in the neck in seven of nine cases of glomus vagale tumors (Table 2). Neurologic involvement of cranial nerves is of critical importance because these nerves may delineate routes and
ANALYSIS OF CLINICAL POPULATION
Glomus tympanum tumor, common tumors originating in the middle ear, and carotid body tumors have been ex-eluded from this series. In this study there are 72 glomus jugulare tumors and nine glomus vagale tumors. The male to female ratio is 6: 1. The average age is 53 years with a range of 16-83 years. Average duration of symptoms prior to diagnosis is 13.1 months. The incidence of multiple tumors is 10%. CLINICAL SIGNS AND SYMPTOMS
The majority of these cases had conductive hearing loss. pulsating tinnitus, or a mass behind the tympanic membrane (Tables 1 and 2). Neurologic deficits of the cranial nerves were common. Most often the VII nerve was
TABLE 1. PRESENTING SIGNS AND SYMPTOMS IN 72 GLOMUS JUGULARE CASES Signs and Symptoms Conductive loss Tinnitus Mass of external canal° Bleeding Mass behind TM Aural discharge Aural pain Vertigo Brown's sign Mass in neck Canal paresis Decreasing vision Cranial nerve palsy
No. of Patients 42 40 21 21 33 21 22 15 19 5 8 2 27
% 58 56 29 29 46 29 31 21 26
7 11 3 38
'Fourteen cases treated initially elsewhere.
From the Departments of Otolaryngology and Radiology, \Vashington University School of Medicine, St. Louis. Missouri. Presented at the meeting of the American Laryngologlcal Association, Palm Beach, Florida, April 22-23, 1978.
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GLOMUS JUGULARE AND V AGALE
TABLE 2. PRESENTING SIGNS AND SYMPTOMS IN 9 GLOMUS VAGALE CASES Signs and Symptoms
No. of Patients
Mass in neck Pharyngeal mass Pharyngeal pain Sudden deafness Black-out spells Vertigo Cranial nerve palsy
7 4 3
lfl
78 44 33
1 1
11 11 11
5
55
1
areas of the cancer's extension. There is cranial nerve involvement in 38% of glomus jugulare tumors and in 55% of glomus vagale tumors. Glomus jugulare tumors located in the jugular bulb have ready access to various portions in the temporal bone and the foramina at the base of the skull, since these tumors spread along the lines of least resistance. Intracranial extension, therefore, can follow along the carotid artery, cranial nerve foramina, into the nasopharynx, intravascularly into the lateral sinus and inferior petrosal sinus, or follow the temporal bone air cell system to the petrous apex, retrofacially or via mastoid air cells. The signs and symptoms of glomus vagale tumors are more difficult to diagnose and are less specific. The most predominant sign is a neck mass, high in the neck. The mass may be pulsatile and have a bruit. Ipsilateral paresis involving the IX and X nerves may be present in half of the cases. The XI and XII nerves are infrequently involved.
Occasionally pharyngeal swelling may be evident in the tonsillar area and lateral oropharyngeal wall. The mass is not associated with pain or severe discomfort. Generally, the symptoms are referable to the parapharyngeal space. NEURORADIOLOGIC EVALUATION
Plain x-rays of the skull and mastoid x-rays are the least useful tests and give minimal information on localization and growth patterns. Polytomes indicate 1) a general picture of the osteology, i.e., specific patterns of bone destruction, 2) areas of bone erosion, i.e., indicating direction of tumor growth, 3) a soft tissue mass in the middle ear with or without ossicular destruction, 4) invasion of cochlear or VII nerve (fallopian) canal. CAT scanning with or without contrast may give additional information with respect to CNS invasion, Retrograde venous [ugulography? indicates the inferior border of the tumor, intraluminal invasion or extraluminal compression of the jugular vein lumen or CNS invasion of the sigmoid sinus, lateral sinus, or inferior petrosal sinus. The arteriogram distinguishes between various types of chemodectomas as well as other vascular tumors. It delineates the superior and inferior borders of the tumor. It indicates the blood supply of the tumor. CNS invasion is indicated by arteriography by means of two findings: visualization of direct tumor invasion of the CNS and observation of abnormal CNS vessels arising from the internal carotid artery to supply the tumor.
TABLE 3. CRANIAL NERVE PALSIES IN GLOMUS JUGULARE AND GLOMUS VAGALE TUMORS
Cranial Nerve Palsies
Total incidence of palsies VII VIII (labyrinthine invasion) IX X XI XII Homer's (sympathetics)
No. of Glomus Vagale Patients
lfl
5
55
0 1 4 2 1 1 1
0 11 44 22 11 11 11
No. of Glomus Jugulare Patients
27 17 3 2 5 2 7 4
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lfl
38 24 4 3 6 3 10 6
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OGURAETAL TABLE 4. THERAPEUTIC RESULTS o
Glomus Jugulare Glomus Vagale
Surgery
Radiation
Combined
39/49 ( 80%)00 8/8 (100%)+
3/12 (25%)tt 0/1 (0%)
7/11 (64%) 0/0 (0%)
·Cure rate, 6.5 yea.rs NED. "Three cases beaded. two not resected. CLINICAL DETERMINANTS REGARDING SURGICAL INTERVENTION
Generally, extracranial tumors or those which arise posterior to the internal carotid artery are amenable to surgery. Consequently, such findings as paralysis of the V (always sensory) or VI (Dorel10's canal) nerves, papilledema (initially ipsilateral), or hydrocephalus represent stages of glomus tumors which are not resectable. Ipsilateral involvement of the IX, X, or XII nerves does not imply that the lesions are not resectable. However, tumor extension to the nasopharynx, the petrous apex or anteriorly along the carotid artery intracranially imply nonresectability. Posterior extension along the jugular vein or sigmoid sinus are resectable. Intracranial extension generally follows more than one pathway through the foramina of the base of the skull. This finding explains the frequent surgical failure rate. TUMOR EMBOLIZATION
If the tumor is advanced or extensive, embolization of selected branches of the external carotid artery may temporarily decrease tumor vascularitv and alleviate troublesome symptoms. Vascular embolization of glomus tumors with Gelfoam® beads is used as an adjunct to surgery or for palliation to control symptoms. This is accomplished by decreasing tumor swelling as its blood supply is diminished. Seven cases have been done in extensive lesions or in high-risk patients. In all cases, repeated carotid arteriograms three months to two years later indicated decreased tumor vasculature. SUR~ICAL
THERAPY
Surgery is the preferred treatment of
tOne case beaded, completely resected. ttThree cases beaded, all persisted.
choice (Table 4). About 80% of the glomus jugulare tumors and 90% of glomus vagale tumors are surgically resectable. Only 25% of glomus tumors are radiocurable. Because of the slow growth of the tumors and small metastatic rate, patients who are very old and debilitated are not operated upon. In most cases, these patients outlive their disease. A combined radical mastoid, hypotympanic and neck resection is the standard procedure for glomus jugulare tumors that do not invade the central nervous system. The jugular vein is always taken with the resection whether the tumors invade the jugular vein lumen or extraluminally compress the vein. It is advisable to approach the tumor from the neck (i.e., from below) by isolating and ligating the major feeding vessels from the external carotid artery. The ascending pharyngeal, posterior auricular, occipital, internal maxillary and feeding branches of the vertebral artery are usually involved. The cranial nerves are identified and preserved. The jugular vein is isolated but not tied initially. This is done to allow venous return of blood from the tumor to avoid swelling and congestion of the tumor with blood prior to resection. A standard radical mastoidectomy is then performed with facial nerve decompression and mobilization, if required. The sigmoid sinus is exposed, compressed and ligated. The lower end of the jugular vein is ligated below the level of tumor removal.
Resection of the glomus jugulare tumor is based on the inferior petrosal sinus, since it is not visible until the tumor is removed. This sinus lies medially to the tumor and may produce brisk bleeding that can only be controlled by compression. One must not press so hard
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GLOMUS /UGULARE AND V AGALE
625
Figs. 1, 2, and 3. Resection of glomus vagale tumor. T - Tumor; D - Hypoglossal nerve; C Internal jugular vein; B - Internal carotid artery; A - External carotid artery; E - XI nerve; F Carotid artery; V - Vagus nerve.
as to deviate the brain stem and precipitate a respiratory or cardiac arrest. Temporal bone resection should not be employed. It is the wrong operation for this tumor since it would cut across the planes of tumor spread, leaving the tumor medial to the resected margin. A careful and meticulous otologic procedure is the treatment of choice.
Resection of glomus vagale tumors only requires a neck approach (Figs. 1, 2, and 3). However, a standard cervical approach may not be adequate for exposure of the base of the skull, of the jugular foramen, or to control the bleeding. Occasionally, removal of the transverse processes of cervical vetebrae, styloid process, or mandibular osteotomy may be needed for exposure.
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OGURAETAL
Fig. 4. A) Arteriogram (subtraction technique) demonstrating large glomus vagale tumor and displacing internal carotid artery anteriorly. B) Capillary phase of arteriogram. Note rich vasculature of tumor. C) X-ray photograph of resected tumor specimen which delineates beads in tumor; patient was beaded three days prior to surgery.
Branches from the external carotid artery supplying the intravagale portion of the tumor are ligated. These are generally identified from the preoperative arteriogram. It is necessary to identify and preserve the X, XI, and XII cranial nerves. Dissection usually follows the carotid sheath. Occasionally, the internal
jugular vein may need to be sacrificed. Transection of the X nerve is usually not necessary. The blood supply from the vertebral artery is medial to the tumor and may need to be ligated after tumor removal. Radiotherapy in doses from 4600-6000
Fig. 5. A) Large glomus jugulare tumor (subtraction technique). Catheter in place (two arrows). Note tumor circulation from ascending pharyngeal artery (single arrow). B) Catheter in place in external carotid (two arrows). Blanched tumor vasculature and occlusion of ascending pharyngeal artery (single arrows).
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GLOMUS /UGULARE AND V AGALE
rads yields a 65% tumor response rate (histologically) and a 25% "cure" rate clinically and by arteriography. RESULTS
The therapeutic results are listed in Table 4. The best results were obtained by surgery. Preoperative radiation followed by surgery in glomus jugulare tumors may aid in resection of large tumors, Le., 64% cure rate. However, if combined neurosurgical and mastoidneck procedures are anticipated, radiation should not be used since there is a high risk of CSF leak and meningitis. Also, if fascia lata grafting of the dura is required, radiation cases will not heal adequately (we have had three deaths in the past). In the past six years, there have been no deaths from surgery with our present procedure. The major problem in most instances can be divided into two factors, i.e., exposure and bleeding. The problems of exposure have been outlined above except for those unresectable lesions which lie anteriorly to the internal carotid artery. Bleeding is, therefore, a major issue. The transfusion range for these cases is 0-14 units of blood with an average of 4 units. One patient had a pulmonary embolism and another patient had respiratory arrest while the tumor was being removed from the inferior petrosal sinus. All patients survived the surgery and left the hospital in reasonably good condition. The major complications included persistance of tumor, and facial nerve palsy in seven patients. In our series most of the lesions were large. The surgical cure rate for glomus jugulare tumors was 80% and glomus vagale 100%. For surgical failures, reoperation, radiation or embolization is employed. The latter procedure is usually of little longlasting benefit Reoperation may occasionally be easier technically than the initial surgery if the lesion is devascularized. Recently, we have used embolization of the external carotid artery branches in order to control surgical bleeding or symptoms for inoperable lesions. Obviously, tumor vas-
627
culature from the CNS was not affected by this technique. However, arteriograms, even two years later, demonstrate a diminished arterial supply to the tumor from the external carotid system. Of the seven cases that were treated, one was resected successfully with no transfusion and six cases had persistent tumor in the CNS, i.e., in those areas where tumor beading was not attempted. On arteriography the latter six cases had diminished blood supply from the external carotid system. This technique may prove to be a valuable adjunct in the future. DISCUSSION
Lussenhop and Spence" and Lussenhop et al 1 6 described the use of Silastic® artificial emboli in the treatment of cerebral arteriovenous malformations. They demonstrated that emboli are preferentially directed to the malformation, guided by two factors: 1) the larger caliber of the vessels feeding the malformation compared to those feeding the brain, and 2) the configuration of arterial branching, success being more likely to occur when the feeding arteries represent the continuation of the parent trunk. Newton and Adams" described embolization of a spinal angioma by injected lead pellets via a percutaneously introduced catheter as opposed to surgical arteriotomy utilized by other reports. 1 5 , 1 6 , l S Since then, percutaneous catheter embolization has become more popular19 •2o for treatment of cerebral and spinal arteriovenous malformations. It requires no surgical incision or general anesthesia, does not entail damage to the carotid or vertebral arteries and it permits direct clinical observation of the awake patient, an important guide during the procedure. Besides, the procedure can be repeated at a later date, if necessary, without sacrifice of an artery or reoperation through a previously scarred site. 2 0 Concomitantly, reports appeared describing the use of this technique in treatment of gastrointestinal hemorrhage.v intractable epistaxis," as well as extracranial vascular neoplasms." Proximal ligation of the external ca-
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628
OGURAETAL
rotid artery fails to produce an effective decrease in vascularity of the tumor fed by branches of this artery. This failure is the result of collateral flow through a multitude of anastomotic channels which connect these branches with those of other arteries in the neck such as the vertebral, the opposite external carotid, the ascending cervical and the deep cervical arteries. The "opening up" of such anastomotic channels has been demonstrated angiographically and has caused technical difficulties at reexploration. Embolization aims at blocking the vascular bed of the tumor, causing thrombosis and preventing the establishment of collateral channels as long as obliteration of the vascular bed is maintained. This technique is used in four instances: 1) preoperative reduction of the mass of tumor and anticipating reduction of intraoperative bleeding, done in three cases of glomus jugulare and one glomus vagale tumor (Fig. 4 A, B, and C); 2) in patients who refuse surgery, one patient; 3) in massive tumors that extend intracranially and are not resectable, one patient (Fig. 5 A, B); or 4) in debilitated, aged patients who cannot tolerate the surgical procedure, one patient. In the third and fourth groups, we had two patients where selective embolization was performed with dramatic symptomatic improvement and significant reduction of tumor vasculature on repeated arteriography. In a series of 27 cases of lesions extrinsic to the central nervous system treated with percutaneous embolization, Hilal and Michelsen" included eight case of glomus jugulare tumors. In five of these, embolization was performed as a preoperative procedure and resulted in complete excision without need for transfusion in three cases and permitted a wide partial resection in two cases. In two of the remaining three cases, embolization was the only therapeutic measure, and it resulted in disappearance of the symptoms in two cases (one of them was followed for five years). The last patient in the series was the only death in the entire experience of more than 100 patients.
We have had no major complications from embolization in our small series. However, there have been some reported by other neuroradiologists. One patient had a temporary Homer's syndrome as our only complication in this series. Theoretically, Gelfoam® beads can escape into the intracranial circulation via the backflow to give rise to central nervous system complications, i.e., CNS infarctions. For example, with occlusion of the external carotid artery by embolization there may be an increased backflow to the internal carotid artery. Emboli may escape from the external system to lodge in the internal carotid circulation and infarct the brain. In another series of patients with nasopharyngeal angiofibromas, we have had, in one case out of 12, a transient CVA which cleared up in two hours. In another patient, emboli were detected in the middle cerebral artery, but the patient was asymptomatic. Selective angiography of the external carotid artery is an indispensable prerequisite to embolization. The vessels feeding the tumor are identified. The catheter should be advanced as close as possible to the lesion before emboli are introduced. This will reduce the chances for reflux of emboli back into the carotid bifurcation where stray emboli may enter the internal carotid artery. This may be reduced further by the use of a double lumen balloon catheter." Radiopaque SilastiC® spheres and Gelfoam® were most frequently used for embolization. SiIastic® spheres (.5-4 rom in diameter) are impregnated with barium and are, therefore, radiopaque for visualization on radiographs. They require, however, a special, large diameter catheter which may be difficult to maneuver into various arterial branches. A 3 rom sphere requires a .110 inch inner diameter catheter." Gelfoam®, on the other hand, may be cut in relatively large pieces which can be squeezed through the usual angiographic catheter. It may be mixed with tantalum powder, a radiopaque marker, to permit radiographic detection. Its most serious disadvantage is recanalization of the occluded vessels. This renders it suitable
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GLOMUS JUGULARE AND VAGALE
only for temporary preoperative embolization. Manelfe et al,24 using the renal artery of the dog as the experimental model, studied the results of embolization by serial angiograms up to 30 days after the procedure. They demonstrated secondary recanalization in two out of three animals. More recently, liquid Silasticw, an intravascular adhesive, has been developed in a low viscosity liquid form'"
629
which can be injected through a small double lumen catheter and will polymerize into a hard rubbery substance in 5-6 minutes producing permanent obliteration of the vascular bed of the tumor. In conclusion, glomus jugulare and vagale tumors present a major problem for therapy. Recent refinements in diagnostic evaluation and treatment techniques have improved the therapuetic outcome.
REFERENCES 1. Guild SR: A hitherto unrecognized structure: The glomus [ugulare in man, abstracted. Anat Rec (Suppl 19:28) 1941 2. Buril JAW: The jugular body and its tumors. Aust NZ J Surg 24: 199-206, 1955 3. White EG: Die Struktur des Glomus caroticum. Seine Pathologies und Physiologie und seine Beziehung zum nerven system. Beitr Pathol 96:177-227, 1935 4. Zak FG: An expanded concept of tumor of the glomic tissue. NY State J Med 4:11531165, 1954 5. Rosenwasser H: Glomus jugulare tumor nemograph, Arch Otolaryngol 88:29-66, 1968 6. Rosenwasser H: Glomus jugulare tumors. Proc R Soc Med 67:259-263,1974 7. Lattest R: Non-chromaffin paraganglioma of ganglion nodosum, carotid body and aortic arches. Cancer 3:667-694, 1950 8. Spector GJ, Maisel RH, Ogura JH: Glomus tumors of the middle ear: An analysis of 46 patients. Laryngoscope 83:1652-1672, 1973 9. Spector GJ, Maisel RH, Ogura JH: Glomus [ugulare tumors: Clinical pathological analysis. Ann Otol Rhinol Laryngol 83:26-30, 1974 10. Spector GJ, Compango J, Perez C, et al: Glomus [ugulare: Effects of radiotherapy. Cancer 35:1316-1319, 1975 11. Spector GJ, Druck NS, Gado M: Neurologic manifestation of glomus jugulare in the head and neck. Arch NeuroI33:271-274, 1976 12. Spector GJ, Fierstein J, Ogura JH: A comparison of therapeutic modalities of glomus tumors in the temporal bone. Laryngoscope 86:690-696, 1976 13. Druck NS, Spector GJ, Ciralskv R, et al: Malignant glomus vagale, Arch Otolaryngol 102:634-636, 1976 14. Black 0, Myers E, Parmes S: Surgical treatment of vagal chemodectoma, Laryngo-
scope 87:1259-1269,1977 15. Luessenhop AJ, Spence WT: Artificial embolization of cerebral arteries. Report of use in a case of arteriovenous malformation. JAMA 172: 1153-1155, 1960 16. Luessenhop AJ, Kachmann R, Shevlin W, et al: Clinical evaluation of artificial embolization in the management of large cerebral arteriovenous malformations. J Neurosurg 23: 400-417, 1965 17. Newton TH, Adams JE: Angiographic demonstration and non-surgical embolization of spinal cord angioma. Radiology 91:873-876, 1968 18. Boulos R, Kricheff I, Chase N: Value of cerebral angiography in the embolization treatment of cerebral arteriovenous malformations. Radiology 97 :65-70, 1970 19. Doppman JL, DiChiro G, Omaya AK: Percutaneous embolization of spinal cord arteriovenous malformations. J Neurosurg 34: 48-55, 1971 20. Kricheff I, Madayag M, Braunstein P: Transfemoral catheter embolization of cerebral and posterior fossa arteriovenous malformations, Radiology 103:107-111,1972 21. Rosch J, Dotter CT, Brown MJ: Selective arterial embolization: A new method of control' of acute gastrointestinal bleeding. Radiology 102:303-306, 1972 22. Sokologg J, Wickbon I, McDonald D, et al: Therapeutic percutaneous embolization in intractable epistaxis. Radiology 222:285287, 1974 23. Hilal SK, Michelsen JW: Therapeutic percutaneous embolization for extracranial vascular lesions of the head, neck and spine. J Neurosurg 43:275-287, 1975 24. Manelfe C, Barder JF, Fabre J: A comparative study of the short and long term effects of different materials for embolization. Presented at the meeting of the American Society of Neuroradiology, New Orleans, 1978
REPRINTS - Joseph H. Ogura, MD, Dept. of Otolaryngology, Washington University Medical School, 517 S. Euclid, St. Louis, MO 63110.
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GLOMUS JUGULARE AND VAGALE JOSEPH GERSHON
H. OGURA,
MD
J. SPECTOR, MD
MOKHTAR GADO, MD
ST. LOUIS,
MISSOURI
The therapeutic results in 72 patients with glomus jugulare and nine with glomus vagale have indicated a surgical cure rate of 80% in the former and 100% in the latter. Radiotherapy demonstrated a 65% tumor response rate and a 25% cure rate histologically and clinically. Embolization with Gelfoam® beads in seven patients resulted in diminished tumor size and arterial circulation from the external carotid system. However, in all cases the tumor persisted. Embolization diminished intraoperative bleeding. Nevertheless, the use of embolization as a palliative modality demonstrated significant symptomatic relief in aged, debilitated patients and in all patients with large inoperable glomus jugulare tumors.
Glomus tumors or chemodectomas arise from the chemoreceptor system found along the jugular bulb, middle ear, vagus nerve and chest.':" They are the most common neoplasms of the middle ear. Many aspects of this article have been previously published.v-" This paper updates our results. In addition, we will emphasize embolization'< which can serve a useful purpose, in some cases, prior to resection and perhaps may be an adjunctive therapy for symptomatic relief in those tumors which are not resectable or in debilitated patients.
paralyzed. Less often VIII (labyrinthine invasion), IX, X, XI, XII, or sympathetic nerve paralysis occurred (Table 3). Bleeding from the ear or a protruding polyp in the external auditory canal was present in about one third of the cases of glomus jugulare tumors (Table 1). A mass was palpated in the neck in seven of nine cases of glomus vagale tumors (Table 2). Neurologic involvement of cranial nerves is of critical importance because these nerves may delineate routes and
ANALYSIS OF CLINICAL POPULATION
Glomus tympanum tumor, common tumors originating in the middle ear, and carotid body tumors have been ex-eluded from this series. In this study there are 72 glomus jugulare tumors and nine glomus vagale tumors. The male to female ratio is 6: 1. The average age is 53 years with a range of 16-83 years. Average duration of symptoms prior to diagnosis is 13.1 months. The incidence of multiple tumors is 10%. CLINICAL SIGNS AND SYMPTOMS
The majority of these cases had conductive hearing loss. pulsating tinnitus, or a mass behind the tympanic membrane (Tables 1 and 2). Neurologic deficits of the cranial nerves were common. Most often the VII nerve was
TABLE 1. PRESENTING SIGNS AND SYMPTOMS IN 72 GLOMUS JUGULARE CASES Signs and Symptoms Conductive loss Tinnitus Mass of external canal° Bleeding Mass behind TM Aural discharge Aural pain Vertigo Brown's sign Mass in neck Canal paresis Decreasing vision Cranial nerve palsy
No. of Patients 42 40 21 21 33 21 22 15 19 5 8 2 27
% 58 56 29 29 46 29 31 21 26
7 11 3 38
'Fourteen cases treated initially elsewhere.
From the Departments of Otolaryngology and Radiology, \Vashington University School of Medicine, St. Louis. Missouri. Presented at the meeting of the American Laryngologlcal Association, Palm Beach, Florida, April 22-23, 1978.
622
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GLOMUS JUGULARE AND V AGALE
TABLE 2. PRESENTING SIGNS AND SYMPTOMS IN 9 GLOMUS VAGALE CASES Signs and Symptoms
No. of Patients
Mass in neck Pharyngeal mass Pharyngeal pain Sudden deafness Black-out spells Vertigo Cranial nerve palsy
7 4 3
lfl
78 44 33
1 1
11 11 11
5
55
1
areas of the cancer's extension. There is cranial nerve involvement in 38% of glomus jugulare tumors and in 55% of glomus vagale tumors. Glomus jugulare tumors located in the jugular bulb have ready access to various portions in the temporal bone and the foramina at the base of the skull, since these tumors spread along the lines of least resistance. Intracranial extension, therefore, can follow along the carotid artery, cranial nerve foramina, into the nasopharynx, intravascularly into the lateral sinus and inferior petrosal sinus, or follow the temporal bone air cell system to the petrous apex, retrofacially or via mastoid air cells. The signs and symptoms of glomus vagale tumors are more difficult to diagnose and are less specific. The most predominant sign is a neck mass, high in the neck. The mass may be pulsatile and have a bruit. Ipsilateral paresis involving the IX and X nerves may be present in half of the cases. The XI and XII nerves are infrequently involved.
Occasionally pharyngeal swelling may be evident in the tonsillar area and lateral oropharyngeal wall. The mass is not associated with pain or severe discomfort. Generally, the symptoms are referable to the parapharyngeal space. NEURORADIOLOGIC EVALUATION
Plain x-rays of the skull and mastoid x-rays are the least useful tests and give minimal information on localization and growth patterns. Polytomes indicate 1) a general picture of the osteology, i.e., specific patterns of bone destruction, 2) areas of bone erosion, i.e., indicating direction of tumor growth, 3) a soft tissue mass in the middle ear with or without ossicular destruction, 4) invasion of cochlear or VII nerve (fallopian) canal. CAT scanning with or without contrast may give additional information with respect to CNS invasion, Retrograde venous [ugulography? indicates the inferior border of the tumor, intraluminal invasion or extraluminal compression of the jugular vein lumen or CNS invasion of the sigmoid sinus, lateral sinus, or inferior petrosal sinus. The arteriogram distinguishes between various types of chemodectomas as well as other vascular tumors. It delineates the superior and inferior borders of the tumor. It indicates the blood supply of the tumor. CNS invasion is indicated by arteriography by means of two findings: visualization of direct tumor invasion of the CNS and observation of abnormal CNS vessels arising from the internal carotid artery to supply the tumor.
TABLE 3. CRANIAL NERVE PALSIES IN GLOMUS JUGULARE AND GLOMUS VAGALE TUMORS
Cranial Nerve Palsies
Total incidence of palsies VII VIII (labyrinthine invasion) IX X XI XII Homer's (sympathetics)
No. of Glomus Vagale Patients
lfl
5
55
0 1 4 2 1 1 1
0 11 44 22 11 11 11
No. of Glomus Jugulare Patients
27 17 3 2 5 2 7 4
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lfl
38 24 4 3 6 3 10 6
624
OGURAETAL TABLE 4. THERAPEUTIC RESULTS o
Glomus Jugulare Glomus Vagale
Surgery
Radiation
Combined
39/49 ( 80%)00 8/8 (100%)+
3/12 (25%)tt 0/1 (0%)
7/11 (64%) 0/0 (0%)
·Cure rate, 6.5 yea.rs NED. "Three cases beaded. two not resected. CLINICAL DETERMINANTS REGARDING SURGICAL INTERVENTION
Generally, extracranial tumors or those which arise posterior to the internal carotid artery are amenable to surgery. Consequently, such findings as paralysis of the V (always sensory) or VI (Dorel10's canal) nerves, papilledema (initially ipsilateral), or hydrocephalus represent stages of glomus tumors which are not resectable. Ipsilateral involvement of the IX, X, or XII nerves does not imply that the lesions are not resectable. However, tumor extension to the nasopharynx, the petrous apex or anteriorly along the carotid artery intracranially imply nonresectability. Posterior extension along the jugular vein or sigmoid sinus are resectable. Intracranial extension generally follows more than one pathway through the foramina of the base of the skull. This finding explains the frequent surgical failure rate. TUMOR EMBOLIZATION
If the tumor is advanced or extensive, embolization of selected branches of the external carotid artery may temporarily decrease tumor vascularitv and alleviate troublesome symptoms. Vascular embolization of glomus tumors with Gelfoam® beads is used as an adjunct to surgery or for palliation to control symptoms. This is accomplished by decreasing tumor swelling as its blood supply is diminished. Seven cases have been done in extensive lesions or in high-risk patients. In all cases, repeated carotid arteriograms three months to two years later indicated decreased tumor vasculature. SUR~ICAL
THERAPY
Surgery is the preferred treatment of
tOne case beaded, completely resected. ttThree cases beaded, all persisted.
choice (Table 4). About 80% of the glomus jugulare tumors and 90% of glomus vagale tumors are surgically resectable. Only 25% of glomus tumors are radiocurable. Because of the slow growth of the tumors and small metastatic rate, patients who are very old and debilitated are not operated upon. In most cases, these patients outlive their disease. A combined radical mastoid, hypotympanic and neck resection is the standard procedure for glomus jugulare tumors that do not invade the central nervous system. The jugular vein is always taken with the resection whether the tumors invade the jugular vein lumen or extraluminally compress the vein. It is advisable to approach the tumor from the neck (i.e., from below) by isolating and ligating the major feeding vessels from the external carotid artery. The ascending pharyngeal, posterior auricular, occipital, internal maxillary and feeding branches of the vertebral artery are usually involved. The cranial nerves are identified and preserved. The jugular vein is isolated but not tied initially. This is done to allow venous return of blood from the tumor to avoid swelling and congestion of the tumor with blood prior to resection. A standard radical mastoidectomy is then performed with facial nerve decompression and mobilization, if required. The sigmoid sinus is exposed, compressed and ligated. The lower end of the jugular vein is ligated below the level of tumor removal.
Resection of the glomus jugulare tumor is based on the inferior petrosal sinus, since it is not visible until the tumor is removed. This sinus lies medially to the tumor and may produce brisk bleeding that can only be controlled by compression. One must not press so hard
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Figs. 1, 2, and 3. Resection of glomus vagale tumor. T - Tumor; D - Hypoglossal nerve; C Internal jugular vein; B - Internal carotid artery; A - External carotid artery; E - XI nerve; F Carotid artery; V - Vagus nerve.
as to deviate the brain stem and precipitate a respiratory or cardiac arrest. Temporal bone resection should not be employed. It is the wrong operation for this tumor since it would cut across the planes of tumor spread, leaving the tumor medial to the resected margin. A careful and meticulous otologic procedure is the treatment of choice.
Resection of glomus vagale tumors only requires a neck approach (Figs. 1, 2, and 3). However, a standard cervical approach may not be adequate for exposure of the base of the skull, of the jugular foramen, or to control the bleeding. Occasionally, removal of the transverse processes of cervical vetebrae, styloid process, or mandibular osteotomy may be needed for exposure.
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Fig. 4. A) Arteriogram (subtraction technique) demonstrating large glomus vagale tumor and displacing internal carotid artery anteriorly. B) Capillary phase of arteriogram. Note rich vasculature of tumor. C) X-ray photograph of resected tumor specimen which delineates beads in tumor; patient was beaded three days prior to surgery.
Branches from the external carotid artery supplying the intravagale portion of the tumor are ligated. These are generally identified from the preoperative arteriogram. It is necessary to identify and preserve the X, XI, and XII cranial nerves. Dissection usually follows the carotid sheath. Occasionally, the internal
jugular vein may need to be sacrificed. Transection of the X nerve is usually not necessary. The blood supply from the vertebral artery is medial to the tumor and may need to be ligated after tumor removal. Radiotherapy in doses from 4600-6000
Fig. 5. A) Large glomus jugulare tumor (subtraction technique). Catheter in place (two arrows). Note tumor circulation from ascending pharyngeal artery (single arrow). B) Catheter in place in external carotid (two arrows). Blanched tumor vasculature and occlusion of ascending pharyngeal artery (single arrows).
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rads yields a 65% tumor response rate (histologically) and a 25% "cure" rate clinically and by arteriography. RESULTS
The therapeutic results are listed in Table 4. The best results were obtained by surgery. Preoperative radiation followed by surgery in glomus jugulare tumors may aid in resection of large tumors, Le., 64% cure rate. However, if combined neurosurgical and mastoidneck procedures are anticipated, radiation should not be used since there is a high risk of CSF leak and meningitis. Also, if fascia lata grafting of the dura is required, radiation cases will not heal adequately (we have had three deaths in the past). In the past six years, there have been no deaths from surgery with our present procedure. The major problem in most instances can be divided into two factors, i.e., exposure and bleeding. The problems of exposure have been outlined above except for those unresectable lesions which lie anteriorly to the internal carotid artery. Bleeding is, therefore, a major issue. The transfusion range for these cases is 0-14 units of blood with an average of 4 units. One patient had a pulmonary embolism and another patient had respiratory arrest while the tumor was being removed from the inferior petrosal sinus. All patients survived the surgery and left the hospital in reasonably good condition. The major complications included persistance of tumor, and facial nerve palsy in seven patients. In our series most of the lesions were large. The surgical cure rate for glomus jugulare tumors was 80% and glomus vagale 100%. For surgical failures, reoperation, radiation or embolization is employed. The latter procedure is usually of little longlasting benefit Reoperation may occasionally be easier technically than the initial surgery if the lesion is devascularized. Recently, we have used embolization of the external carotid artery branches in order to control surgical bleeding or symptoms for inoperable lesions. Obviously, tumor vas-
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culature from the CNS was not affected by this technique. However, arteriograms, even two years later, demonstrate a diminished arterial supply to the tumor from the external carotid system. Of the seven cases that were treated, one was resected successfully with no transfusion and six cases had persistent tumor in the CNS, i.e., in those areas where tumor beading was not attempted. On arteriography the latter six cases had diminished blood supply from the external carotid system. This technique may prove to be a valuable adjunct in the future. DISCUSSION
Lussenhop and Spence" and Lussenhop et al 1 6 described the use of Silastic® artificial emboli in the treatment of cerebral arteriovenous malformations. They demonstrated that emboli are preferentially directed to the malformation, guided by two factors: 1) the larger caliber of the vessels feeding the malformation compared to those feeding the brain, and 2) the configuration of arterial branching, success being more likely to occur when the feeding arteries represent the continuation of the parent trunk. Newton and Adams" described embolization of a spinal angioma by injected lead pellets via a percutaneously introduced catheter as opposed to surgical arteriotomy utilized by other reports. 1 5 , 1 6 , l S Since then, percutaneous catheter embolization has become more popular19 •2o for treatment of cerebral and spinal arteriovenous malformations. It requires no surgical incision or general anesthesia, does not entail damage to the carotid or vertebral arteries and it permits direct clinical observation of the awake patient, an important guide during the procedure. Besides, the procedure can be repeated at a later date, if necessary, without sacrifice of an artery or reoperation through a previously scarred site. 2 0 Concomitantly, reports appeared describing the use of this technique in treatment of gastrointestinal hemorrhage.v intractable epistaxis," as well as extracranial vascular neoplasms." Proximal ligation of the external ca-
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rotid artery fails to produce an effective decrease in vascularity of the tumor fed by branches of this artery. This failure is the result of collateral flow through a multitude of anastomotic channels which connect these branches with those of other arteries in the neck such as the vertebral, the opposite external carotid, the ascending cervical and the deep cervical arteries. The "opening up" of such anastomotic channels has been demonstrated angiographically and has caused technical difficulties at reexploration. Embolization aims at blocking the vascular bed of the tumor, causing thrombosis and preventing the establishment of collateral channels as long as obliteration of the vascular bed is maintained. This technique is used in four instances: 1) preoperative reduction of the mass of tumor and anticipating reduction of intraoperative bleeding, done in three cases of glomus jugulare and one glomus vagale tumor (Fig. 4 A, B, and C); 2) in patients who refuse surgery, one patient; 3) in massive tumors that extend intracranially and are not resectable, one patient (Fig. 5 A, B); or 4) in debilitated, aged patients who cannot tolerate the surgical procedure, one patient. In the third and fourth groups, we had two patients where selective embolization was performed with dramatic symptomatic improvement and significant reduction of tumor vasculature on repeated arteriography. In a series of 27 cases of lesions extrinsic to the central nervous system treated with percutaneous embolization, Hilal and Michelsen" included eight case of glomus jugulare tumors. In five of these, embolization was performed as a preoperative procedure and resulted in complete excision without need for transfusion in three cases and permitted a wide partial resection in two cases. In two of the remaining three cases, embolization was the only therapeutic measure, and it resulted in disappearance of the symptoms in two cases (one of them was followed for five years). The last patient in the series was the only death in the entire experience of more than 100 patients.
We have had no major complications from embolization in our small series. However, there have been some reported by other neuroradiologists. One patient had a temporary Homer's syndrome as our only complication in this series. Theoretically, Gelfoam® beads can escape into the intracranial circulation via the backflow to give rise to central nervous system complications, i.e., CNS infarctions. For example, with occlusion of the external carotid artery by embolization there may be an increased backflow to the internal carotid artery. Emboli may escape from the external system to lodge in the internal carotid circulation and infarct the brain. In another series of patients with nasopharyngeal angiofibromas, we have had, in one case out of 12, a transient CVA which cleared up in two hours. In another patient, emboli were detected in the middle cerebral artery, but the patient was asymptomatic. Selective angiography of the external carotid artery is an indispensable prerequisite to embolization. The vessels feeding the tumor are identified. The catheter should be advanced as close as possible to the lesion before emboli are introduced. This will reduce the chances for reflux of emboli back into the carotid bifurcation where stray emboli may enter the internal carotid artery. This may be reduced further by the use of a double lumen balloon catheter." Radiopaque SilastiC® spheres and Gelfoam® were most frequently used for embolization. SiIastic® spheres (.5-4 rom in diameter) are impregnated with barium and are, therefore, radiopaque for visualization on radiographs. They require, however, a special, large diameter catheter which may be difficult to maneuver into various arterial branches. A 3 rom sphere requires a .110 inch inner diameter catheter." Gelfoam®, on the other hand, may be cut in relatively large pieces which can be squeezed through the usual angiographic catheter. It may be mixed with tantalum powder, a radiopaque marker, to permit radiographic detection. Its most serious disadvantage is recanalization of the occluded vessels. This renders it suitable
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only for temporary preoperative embolization. Manelfe et al,24 using the renal artery of the dog as the experimental model, studied the results of embolization by serial angiograms up to 30 days after the procedure. They demonstrated secondary recanalization in two out of three animals. More recently, liquid Silasticw, an intravascular adhesive, has been developed in a low viscosity liquid form'"
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which can be injected through a small double lumen catheter and will polymerize into a hard rubbery substance in 5-6 minutes producing permanent obliteration of the vascular bed of the tumor. In conclusion, glomus jugulare and vagale tumors present a major problem for therapy. Recent refinements in diagnostic evaluation and treatment techniques have improved the therapuetic outcome.
REFERENCES 1. Guild SR: A hitherto unrecognized structure: The glomus [ugulare in man, abstracted. Anat Rec (Suppl 19:28) 1941 2. Buril JAW: The jugular body and its tumors. Aust NZ J Surg 24: 199-206, 1955 3. White EG: Die Struktur des Glomus caroticum. Seine Pathologies und Physiologie und seine Beziehung zum nerven system. Beitr Pathol 96:177-227, 1935 4. Zak FG: An expanded concept of tumor of the glomic tissue. NY State J Med 4:11531165, 1954 5. Rosenwasser H: Glomus jugulare tumor nemograph, Arch Otolaryngol 88:29-66, 1968 6. Rosenwasser H: Glomus jugulare tumors. Proc R Soc Med 67:259-263,1974 7. Lattest R: Non-chromaffin paraganglioma of ganglion nodosum, carotid body and aortic arches. Cancer 3:667-694, 1950 8. Spector GJ, Maisel RH, Ogura JH: Glomus tumors of the middle ear: An analysis of 46 patients. Laryngoscope 83:1652-1672, 1973 9. Spector GJ, Maisel RH, Ogura JH: Glomus [ugulare tumors: Clinical pathological analysis. Ann Otol Rhinol Laryngol 83:26-30, 1974 10. Spector GJ, Compango J, Perez C, et al: Glomus [ugulare: Effects of radiotherapy. Cancer 35:1316-1319, 1975 11. Spector GJ, Druck NS, Gado M: Neurologic manifestation of glomus jugulare in the head and neck. Arch NeuroI33:271-274, 1976 12. Spector GJ, Fierstein J, Ogura JH: A comparison of therapeutic modalities of glomus tumors in the temporal bone. Laryngoscope 86:690-696, 1976 13. Druck NS, Spector GJ, Ciralskv R, et al: Malignant glomus vagale, Arch Otolaryngol 102:634-636, 1976 14. Black 0, Myers E, Parmes S: Surgical treatment of vagal chemodectoma, Laryngo-
scope 87:1259-1269,1977 15. Luessenhop AJ, Spence WT: Artificial embolization of cerebral arteries. Report of use in a case of arteriovenous malformation. JAMA 172: 1153-1155, 1960 16. Luessenhop AJ, Kachmann R, Shevlin W, et al: Clinical evaluation of artificial embolization in the management of large cerebral arteriovenous malformations. J Neurosurg 23: 400-417, 1965 17. Newton TH, Adams JE: Angiographic demonstration and non-surgical embolization of spinal cord angioma. Radiology 91:873-876, 1968 18. Boulos R, Kricheff I, Chase N: Value of cerebral angiography in the embolization treatment of cerebral arteriovenous malformations. Radiology 97 :65-70, 1970 19. Doppman JL, DiChiro G, Omaya AK: Percutaneous embolization of spinal cord arteriovenous malformations. J Neurosurg 34: 48-55, 1971 20. Kricheff I, Madayag M, Braunstein P: Transfemoral catheter embolization of cerebral and posterior fossa arteriovenous malformations, Radiology 103:107-111,1972 21. Rosch J, Dotter CT, Brown MJ: Selective arterial embolization: A new method of control' of acute gastrointestinal bleeding. Radiology 102:303-306, 1972 22. Sokologg J, Wickbon I, McDonald D, et al: Therapeutic percutaneous embolization in intractable epistaxis. Radiology 222:285287, 1974 23. Hilal SK, Michelsen JW: Therapeutic percutaneous embolization for extracranial vascular lesions of the head, neck and spine. J Neurosurg 43:275-287, 1975 24. Manelfe C, Barder JF, Fabre J: A comparative study of the short and long term effects of different materials for embolization. Presented at the meeting of the American Society of Neuroradiology, New Orleans, 1978
REPRINTS - Joseph H. Ogura, MD, Dept. of Otolaryngology, Washington University Medical School, 517 S. Euclid, St. Louis, MO 63110.
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