Ann Otol 85: 1976
SCHWANNOMA OF THE JUGULAR FORAMEN RICHARD
R.
GACEK,
M.D.
WORCESTER, MASSACHUSETfS
SUMMARY - Two cases of schwannoma limited to the jugular foramen are described. This entity may clinically and radiologically resemble glomus jugulare tumors so closely, that only careful histological examination of an adequate surgical tumor specimen can provide a deflnitive diagnosis. Since documentation of this form of neurogenic tumor is lacking in the literature, it suggests that the entity is very rare or that such tumors may be erroneously classified as glomus jugulare tumors. The two cases in this study were managed surgically by subtotal removal via a wide transmastoid approach.
Although glomus jugulare tumors have been recognized as the most common neoplasm to arise from the jugular foramen, they must sometimes be differentiated from other entities which may simulate their clinical presentation. The glomus jugulare tumor usually appears as a red mass medial or lateral to the inferior portion of the tympanic membrane in a patient with hearing loss and a pulsating tinnitus. Radiological demonstration of enlargement of the iugular foramen or destruction of the lower portion of the petrous apex and an angiographic picture of a vascular tumor flush supplied by the ascending pharyngeal artery provide the objective laboratory evidence needed to confirm this diagnosis.F' The differential diagnosis includes many entities. 3 -5 It may be useful to divide these into three groups based on clinical and radiological similarities to the glomus jugulare tumor.
1. Those conditions which are relatively common but do not closely resemble the glornus jugulare tumor clinically or radiologically can usually be readily eliminated. These are carcinoma of the nasopharynx, ear or sphenoid sinus, acoustic neurinoma, and cholesteatoma. 2. Those diseases which closely resemble the glomus jugulare tumor clin-
ically but can be differentiated by radiologic and angiographic techniques are: inflammatory polyp, cholesterol granuloma (serous otitis media), dehiscent jugular bulb, aberrant internal carotid artery, and otosclerosis with a prominent Schwartze sign. 3. There are other benign tumors that arise from structures in the jugular foramen (nerves, dura, blood vessels) which so closely resemble the glomus jugulare tumor clinically and radiologically, that the diagnosis can only be made after careful histological evaluation of the tumor tissue. This report describes two patients having tumors closely mimicking glomus jugulare tumor with regard to both clinical and radiological presentation. Only after histological examination of the excised tumor mass could the diagnosis of schwannoma of the jugular foramen be made. Since the ultimate management of these tumors is different, the necessity for accurate tissue diagnosis is emphasized. CASE REPORTS
Case 1. P.L. is a 15-year-old female patient who was seen by a local otolaryngologist because she had failed a school hearing test. The patient also noted a pulsating tinnitus in the right ear. A reddish mass behind the inferior half of the right tympanic membrane was noted and a sensorineural hearing loss was documented in the right ear. Radiographic
From the Department of Otolaryngology, University of Massachusetts Medical School, Worcester, Massachusetts. 215
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Fig. 1. Base view tomogram of the skull showing the enlargement of the right jugular foramen (arrows) in Case 1. evaluation of the base of the skull showed a destructive lesion of the right jugular foramen typical of a glomus jugulare tumor. The patient was then referred to the author on July 26, 1974 for management. Examination revealed a reddish purple mass partially displacing the lower half of the tympanic membrane laterally. There was a questionable pulsatile appearance to this tumor mass. The remainder of the ear, nose and throat examination, and the cranial nerve evaluation was negative except for a partial right facial weakness and a moderately severe sensorineural hearing loss in the right ear. Conventional x-rays and poly tomes of the temporal bones revealed a large area of destruction in the jugular foramen which extended laterally to the tympanic cavity and medially to the hypoglossal canal (Fig. 1). Erosion of the undersurface of the petrous segment of the temporal bone and the inferior surface of the external auditory canal was also noted. The caroticojugular spine had been destroyed by the osteolytic lesion, and the tumor was noted to be in close apposition to the vertical portion of the facial nerve canal.
On September 24, 1974 surgical management of the tumor mass was carried out with six units of blood available. Following a postauricular incision which was extended down into the upper neck along the anterior border of the sternocleidomastoid muscle, the external carotid artery was identified and ligated just inferior to the ascending pharyngeal artery. At this time it was noted that the internal jugular vein was extremely small, measuring approximately 3 mm in diameter and flattened in appearance. Very little blood flow was present in this vein.
Audiometric evaluation confirmed normal hearing in the left ear and a severe sensorineural hearing loss in the right ear. Vestibular testing showed minimal depression of the caloric response in the right ear. A presumptive diagnosis of glomus jugulare tumor on the right was made and the patient was admitted to the hospital for further evaluation. A left vertebral and a right internal carotid arteriogram were normal. The right external carotid arteriogram demonstrated a 1.5 to 2 em vascular blush with small irregular vessels feeding from the ascending pharyngeal artery and located in the area of destruction of the jugular fossa (Fig. 2). This was felt to be most compatible with a glomus jugulare tumor.
Fig. 2. Right external carotid anteriogram in Case 1 showed a circular vascular mass (arrows) supplied by the ascending pharyngeal artery.
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Fig. 3. Drawing of the surgical exposure of the tumor mass in Case 1. A radical mastoidectomy was carried out and extended by removal of the tym.panic bone anteriorly and inferiorly around the tumor mass in the hypotympanum. After removal of the tympanic membrane, malleus and incus, a reddish purple lobulated tumor mass was seen filling the hypotympanum up to the level of the round window niche and extending from the carotid canal anteriorly to the vertical facial nerve segment posteriorly (Fig. 3 ). The tumor displaced the vertical portion of the facial nerve laterally and extended posteriorly to the dura of the posterior cranial fossa . The facial nerve at this point was noted to be flattened against the tumor. Delineation of the bony semicircular canals and the dura of the posterior cranial fossa and sigmoid sinus was carried out to establish the safe limits of maximum exposure before removal of the tumor mass.
A generous portion of the tumor was removed from the hypotympanum and sent for frozen section. Minimal bleeding was produced following this biopsy procedure and the tumor mass appeared to be more friable than glomus jugulare tumor tissue. The frozen section report indicated no evidence of glomus jugulare tumor; however, a definite diagnosis could not be made. Tumor removal was then carried out in piecemeal fashion with cup forceps and blunt dissectors until the dural surface of the undersurface of the temporal bone and the internal carotid artery wall was seen anteriorly. A small amount of tumor tissue was left posteroinferiorly in the region
of the jugular formen, because severe venous bleeding followed attempted tumor removal in this area. Approximately 75 to 80 percent of the tumor was successfully removed. The radical mastoidectomy cavity was maintained and the defect in the hypotympanum packed open. Two units of whole blood were replaced at surgery. The patient followed a benign postoperative course and left the hospital after three days. Pathological examination of the 'permanent sections of the tumor specimen indicated a neurolemmoma (schwannoma) (Figs. 4 and 5). The patient's ear healed rapidly within six weeks. She has a profound right-sided sensorineural hearing loss but has completely recovered facial nerve function within two months. Case 2. E.N. is a 59-year-old female patient who was first seen by her local otolaryngologist in December 1972 for progressive hoarseness. Examination at that time revealed paralysis of the left vocal cord, the left side of the tongue and the left sternocleidomastoid and trapezius muscles. A purplish discoloration in the anterior hypotympanum of the left ear was also noted on otoscopic examination. A presumptive diagnosis of glomus [ugulare tumor of the left side was made, and an exploratory tympanotomy was carried out. Very little material was available for biopsy in the middle ear; that tissue which was obtained showed only hypervascularity but no diagnostic abnormality. Since facilities were not
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Fig. 4. A) Photomicrograph of the tumor mass removed from Case 1 demonstrates the tumor cells somewhat loosely dispersed among blood vessels as in the Antoni B type schwannoma. (500X). B) Higher power photomicrograph of the center of the field in 4A shows the streaming tendency of cell nuclei. (1250X)
Fig. 5. Photomicrograph of a different area in the tumor removed from Case 1 showing a more typical histologic appearance of schwannoma. (500X)
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Fig. 6. Frontal tomograms through the temporal bones in Case 2. The cuts from top to bottom represent anterior to posterior levels through the bone. The large radiolucent defect (arrows) on the left appears typical for a glomus jugulare tumor. available for retrograde jugular venography, the patient was treated for a glomus jugulare tumor on the basis of clinical and radiological findings. Radiation therapy totalling 4,880 rads on the Cobalt unit were delivered in January 1973. Audiometric evaluation in February 1972 and January 1974 reportedly showed a mild high-tone loss in the left ear with normal hearing in the right ear.
Fig. 7. Left external carotid arteriogram in Case 2. The diffuse vascular flush (arrows) is supplied br the ascending pharyngeal and occipita arteries.
The patient had no change in signs or symptoms following radiation therapy until the Fall of 1974, when she was admitted to the hospital for left hemifacial spasm. At this time a neurosurgical consultant felt that the facial nerve finding indicated progression of the tumor, and a decision to operate was entertained. However, since the unchanged otologic findings and cranial nerve deficits pointed toward little or no tumor enlargement, surgical management was delayed and the patient was referred to the Massachusetts Eye and Ear Infirmary for management. Examination of this patient on October 1, 1974 at the Massachusetts Eye and Ear Infirmary revealed a normal right ear drum and a purplish discoloration behind the anteroinferior portion of an intact tympanic membrane in the left ear. The cranial nerve deficits were confirmed. These included atrophy and paralysis of the left side of the palate, the left
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Fig. 8. Drawing of the surgical exposure of the tumor mass in Case 2. half of the tongue, the left sternocleidomastoid and trapezius muscles, and the left vocal cord. In addition, 75% paralysis of the left side of the face was also noted. Audiometric evaluation revealed a total sensorineural hearing loss in the left ear and normal hearing in the right. Electronystagmography demonstrated a markedly diminished response on the left side. X-rays obtained on October 2, 1974 included both conventional and polytomes of the mastoid bones. A large destructive lesion of the left jugular foramen was noted. The area of destruction extended into the middle ear, the inferior part of the petrous apex, the adjacent portion of the occipital bone, and the region of the hypoglossal foramen (Fig. 6). This osteolytic defect was compatible with a large glomus jugulare tumor." The patient was admitted to the hospital on October 15, 1974 for additional diagnostic studies. Neurological evaluation confirmed deficits of the seventh through twelfth cranial nerves. A left external carotid arteriogram indicated a vascular mass in the left jugular foramen with feeding vessels arising from the ascending pharyngeal artery and branches of the occipital artery (Fig. 7). It was noted that this vascular blush was somewhat less pronounced than is usually seen with glomus iugulare tumors. Following discharge after the diagnostic evaluation, the patient was readmitted for surgical management of the vascular tumor in the left ear. With six units of whole blood available, the operation was carried out on
November 20, 1974. The postauricular incision was extended into the neck along the anterior border of the sternocleidomastoid, and the left external carotid artery was identified and ligated. The internal jugular vein could not be found in the carotid sheath and was thought to be completely atrophic. A radical mastoidectomy was carried out and extended to remove the tympanic bone anteriorly and inferiorly in such a manner as to skirt the perimeter of the tumor mass which filled the hypotympanum (Fig. 8). The bony labyrinth was skeletonized for surgical landmarks, and the bone over the vertical portion of the facial nerve was thinned down to a bony plate. The tumor in the hypotympanum appeared as a smooth dome-like mass with a pale tan color. It reached superiorly to the level of the round window niche and anteriorly through the carotid canal to make contact with the internal carotid artery wall. The posterior limit of the tumor in the jugular foramen was reached by removing the bone from the sigmoid sinus and the posterior fossa dura. A large segment of tissue from the hypotympanic mass was excised and sent for frozen section. The report indicated fibrous tissue but no evidence of glomus jugulare tumor. Approximately 90 percent removal of the tumor mass was accomplished in piecemeal fashion until the dural surfaces at the depth of the cavity could be seen. The blood loss was minimal and did not require replacement. Several myelinated nerves could be identified following the curvature of the surgical defect.
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Fig. 9. Photomicrograph of the tumor specimen removed from Case 2. A more distinct tendency for palisading of nuclei and the presence of abundant collagenous tissue represents the Antoni A pattern of schwannoma. (500X) The cavity was packed with Gelfoam®o as in a radical mastoidectomy. The postoperative course was uneventful and the patient was discharged on November 25, 1974. Permanent sections of the pathological specimen indicated schwannoma (Fig. 9). The out-patient follow up was characterized by rapid healing of the mastoid cavity in a three to four week period and a noticeable return of facial nerve function. The patient now has approximately 60% facial nerve function. No improvement in the other cranial nerve deficits has been noted. No further surgery is planned in this patient except for possible TeHon®oo injection of the left vocal cord after a 6 to 12 month period if there is no return of function. DISCUSSION
Diagnosis. Virtually no description of neurogenic tumors limited to the jugular foramen can be found in the English literature. This conclusion is supported by reports which reviewed glomus jugulare tumors,3.4.6 those which reviewed all neoplasms of the middle ear and mastoid.t" and those which summarized neurogenic tumors of the head and neck,lO-14 Simpson and Dallachy" and Alford and Cuilford! included neurofibroma of IX through XII
cranial nerves in the differential diagnosis of glomus jugulare tumor but could not cite documentation of this entity. Rosenwasser," Bradley and Maxwell," and Coodman" could only find schwannoma of the facial nerve presenting as a middle ear neoplasm. Conley.l" Putney et al,ll Katz et al,12 and Batsakis'" referred to the well known presentation of neurogenic tumors of IX through XII cranial nerves as tumors of the parapharyngeal space with or without extension into the base of the skull. Das Gupta et al 14 reviewed 303 cases of solitary schwannomas and found 136 occurred in the head and neck area. Although there were over 20 different locations for these tumors in the head and neck, the jugular foramen and middle ear were not included. This void may be due to the fact that these tumors are rare in this location or may suggest that they have not been recognized as neurogenic tumors but possibly called variants of glomus jugulare tumor. The clinical similarities of this tumor to glomus body tumors of the
° The Upjohn 00
Co., Kalamazoo, Mich. E.!. DuPont de Nemours & Co., Inc., Wilmington, DE
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jugular foramen are: 1) They occur onstrated after removal of a biopsy more commonly in female patients; 2) specimen. They may present clinically with pulsating tinnitus and hearing loss; 3) Oto- . The histological features of glomus scopic examination may reveal a red- jugulare tumors are unique and quite dish tumor mass in the hypotympanum; characteristic. The tumor cells are large, 4) The most commonly involved cranial polyhedral chief cells with small nuclei nerves are seven and eight, but the which are not hyperchromatic. Frenerves of the jugular foramen (nine, quently the cytoplasm of these cells has ten and eleven) and the hypoglossal a vacuolated appearance. These cells nerve may be affected in extensive tu- are characteristically arranged in groups mors; 5) Conventional x-rays and poly- (Zellbahlen) separated by fibrous tissue tomes of the jugular foramen reveal an septa containing blood vessels. enlargement of the jugular foramen; 6) Neurogenic tumors arise from proExternal carotid arteriography may liferation of the neurolemmal or demonstrate a vascular lesion supplied 13 15 by vessels from the ascending pharyn- schwann cells of myelinated nerves. • They are appropriately called neurogeal artery. lemmoma or schwannoma. These tumor Clinically no differentiation between cells are elongate spindle cells, usually these two tumors can be made. Even with large nuclei which can range from with conventional x-ray films of the a cigar shape to round. They are acbase of the skull and polytomes of the companied by thin fibrous strands jugular foramen, the osteolytic enlarge- which are usually arranged into mergment of the jugular foramen is indis- ing streams. Characteristically the nutinguishable in both types of tumors. clei tend to align themselves in a paliWhile arteriography shows a vascular sade pattern as in the Antoni A type flush with both types of tumor, it may (Fig. 9). However, there may be a wide be diagnostically significant that the degree of cell pleomorphism with irpattern in a neurofibroma is not as regular cell types dispersed in rather prominent as in the glomus jugulare loose connective tissue and no clear evidence of palisading (Antoni type B) tumor. (Fig. 4). These wide differences may Although a definitive diagnosis can- make the diagnosis of neurolemmoma not be made after clinical or radi- difficult. It is possible that such cases ological evaluation, the initial step in may be misclassified on the basis of management is the same. Surgical ex- clinical features. ploration of the middle ear and mastoid Although these tumors may arise from cavity must be carried out with sufficient blood available to replace the any of the cranial nerves passing blood loss usually associated with re- through the jugular foramen, it is not moval of glomus jugulare tumors. After always possible to identify the site of wide exposure of the tumor has been origin. When a functional nerve deficit achieved, tissue diagnosis from a frozen heralds the presentation of such a tusection is helpful. While the final histo- mor, it may point to the origin of the pathological diagnosis may not be ob- tumor as in Case 2. However, it is well tained at this time, it is usually possible known that these benign tumors may in the to determine the presence or absence of not produce a functional deficit 10 the characteristic chief cells of glomus nerve of origin as in Case 1. ,1 4 tumors. Other features of the tumor at Treatment. The most effective treatsurgery may provide clues to the dif- ment of both glomus tumors and neuroferentiation of these two types of tu- fibroma of the jugular foramen is surmors. The neurofibroma will have a gical removal. While radiation therapy pale tan appearance and its significantly may play a limited role in the control lower propensity to bleed will be dem- of glomus jugulare tumors that are un-
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resectable, this mode of therapy has no effect on neurogenic tumors. Accurate diagnosis is essential to avoid the administration of unnecessary radiation therapy to residual neurogenic tumor tissue on the assumption that it represents glomus body tumor.
with regard to subtotal removal of the tumor, it would appear that the otological approach is the preferred choice because of the low morbidity associated with it, and the exteriorization of the tumor capsule into an area that can be observed (mastoid cavity).
There are two surgical approaches to the jugular foramen: 1) The neurosurgical approach via the posterior cranial fossa carries a high incidence of morbidity and probably would not result in complete removal of tumor since the tumor extends inferiorly through the jugular foramen and laterally into the mastoid and middle ear. 2) The otological approach carries a low morbidity to the patient and may not result in complete removal of the tumor, since the medial extent of a large tumor in this region may not be reached. However, the tumor area may be considered exteriorized and lend itself to follow up observation. Since slow growth is generally characteristic of this type of tummor, long survival may be associated with complete or subtotal tumor removal.
CONCLUSION
Since both surgical approaches achieve essentially the same end result
These two case reports illustrate the close similarity which schwannoma of the jugular foramen may have to glomus jugulare tumors. While some indication as to the nature of the tumor may be provided by close study of the angiographic pattern, only surgical exploration and a tissue diagnosis can produce an accurate diagnosis. Even in cases where tissue has been obtained for histological evaluation, the wide range of histological patterns which these neurogenic tumors may assume makes it important to study closely all areas of the tumor tissue. It is possible that neurogenic tumors of the jugular foramen have been interpreted both clinically and histologically as glomus jugulare tumors. Such may be the case with those glomus jugulare tumors which have been considered atypical in their relative lack of vascularity and histological pattern.!"
Reprints: Richard R. Gacek, M.D., Dept. of Otolaryngology, Univ, of Massachusetts Medical School, 55 Lake Ave. North, Worester, MA 63110. REFERENCES I. Riemenschneider PA, Hoople GD, Brewer D, et al: Roentgeongraphic diagnosis of the glomus [ugulare, Am J Roentgenol Radium Ther Nucl Med 69 :59-65, 1953 2. Holesh S: Diagnosis of tumors of the glomus jugulare. Lancet 268:169-170, 1955 3. Simpson IC, Dallachy R: A review of tumors of the glomus jugulare with reports of three further cases. J Laryngol Otol 72: 194 226, 1958 4. Alford BR, Guilford FR: A comprehensive study of tumors of the glomus [ugulare. Laryngoscope 72:765-787, 1962
mors, Arch Otolaryngol 88:29-66, 1968 7. Rosenwasser H: Neoplasms involving the middle ear. Arch Otolaryngol 32:38-53, 1940 8. Bradley WH, Maxwell JH: Neoplasms of the middle ear and mastoid. Report of fifty-four cases. Laryngoscope 64:533-556, 1954 9. Goodman ML: Middle ear and mastoid neoplasms. Ann Otol Rhinol Laryngol 80:419-424, 1971 10. Conley JJ: Neurogenous tumors in the neck. Arch Otolaryngol 61: 167-180, 1955
5. Valvasorri GE, Buckingham RA: Middle ear masses mimicking glomus tumors: Radiographic and otoscopic recognition. Ann Otol Rhinol Laryngol 83:606-612, 1974
II. Putney FJ, Moran JJ, Thomas GK: Neurogenic tumors of the head and neck. Laryngoscope 74:1037-1059, 1964
6. Rosenwasser H: Glomus jugulare tu-
12. Katz AD, Passy V, Kaplan L: Neuro-
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genous neoplasms of major nerves of face and neck. Arch Surg 103:51-56, 1971 13. Batsakis ]G: Tumors of the peripheral nervous system, in Tumors of the Head and Neck. Baltimore, The Williams and Wilkins Company, 1974 chap 16 14. Das Gupta TK, Brasfield RD, Strong
WE, et al: Benign solitary schwannomas (Neurilemomas). Cancer 24:355-366, 1969 15. Dykstra PC: The pathology of acoustic neuromas. Arch OtolaryngoI80:605-616, 1964 16. Mattick WL, Burke EM: Glomus [ugulare tumors. Laryngoscope 62:311-322, 1952
PSYCHOSOCIAL CARE OF THE DYING PATIENT The first national training conference for physicians on Psychosocial Care of the Dying Patient: Doctor-Patient Relationships in Terminal Illness, will be held April 29-May 1, 1976 at the St. Francis Hotel, San Francisco, California. This program will be presented by the Cancer Research Institute and Extended Programs in Medical Education, University of California School of Medicine, San Francisco, California.
CONFERENCE The Second International Conference on the Diagnosis and Treatment of Craniofacial Anomalies will be held at the New York University Medical Center, New York, New York on May 3-5, 1976. The Conference is organized under the auspices of the Educational Foundation of the American Society of Plastic and Reconstructive Surgeons. The Conference will focus on four major subject areas: orbitocephalic malformations, including orbital clefts and hypertelorism, craniofacial synostosis syndromes, mandibulofacial dyostosis (Treacher-Collins Syndrome) and craniofacial microsomia (maldevelopment of the first and second branchial arches). Emphasis will be placed on etiology, embryology, genetics, classification, pathology and the role of the different clinical specialties in the diagnosis and treatment of these anomalies. Special sessions will be devoted to associated speech and airway problems, helical deformities, audiology and middle ear surgery. For detailed information please write: John Marquis Converse, M.D., Institute of Reconstructive Plastic Surgery, 550 First Avenue, New York, New York 10016.
EUROPEAN SYMPOSIUM A European Symposium, Neurophysiology of Hearing: Interpretation of Human Electrophysiological Data, sponsored by the European Economic Community (Medical Research Committee: Ad hoc Working Group on Deafness) and organized by M. Portmann and J. M. Aran, will be held May 20-22, 1976, in Bordeaux, France. Registration fees: 350.00 F.F. For further information write: M. Portmann, 114 Avenue d'Ares, 33000 Bordeaux, France.
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SCHWANNOMA OF THE JUGULAR FORAMEN RICHARD
R.
GACEK,
M.D.
WORCESTER, MASSACHUSETfS
SUMMARY - Two cases of schwannoma limited to the jugular foramen are described. This entity may clinically and radiologically resemble glomus jugulare tumors so closely, that only careful histological examination of an adequate surgical tumor specimen can provide a deflnitive diagnosis. Since documentation of this form of neurogenic tumor is lacking in the literature, it suggests that the entity is very rare or that such tumors may be erroneously classified as glomus jugulare tumors. The two cases in this study were managed surgically by subtotal removal via a wide transmastoid approach.
Although glomus jugulare tumors have been recognized as the most common neoplasm to arise from the jugular foramen, they must sometimes be differentiated from other entities which may simulate their clinical presentation. The glomus jugulare tumor usually appears as a red mass medial or lateral to the inferior portion of the tympanic membrane in a patient with hearing loss and a pulsating tinnitus. Radiological demonstration of enlargement of the iugular foramen or destruction of the lower portion of the petrous apex and an angiographic picture of a vascular tumor flush supplied by the ascending pharyngeal artery provide the objective laboratory evidence needed to confirm this diagnosis.F' The differential diagnosis includes many entities. 3 -5 It may be useful to divide these into three groups based on clinical and radiological similarities to the glomus jugulare tumor.
1. Those conditions which are relatively common but do not closely resemble the glornus jugulare tumor clinically or radiologically can usually be readily eliminated. These are carcinoma of the nasopharynx, ear or sphenoid sinus, acoustic neurinoma, and cholesteatoma. 2. Those diseases which closely resemble the glomus jugulare tumor clin-
ically but can be differentiated by radiologic and angiographic techniques are: inflammatory polyp, cholesterol granuloma (serous otitis media), dehiscent jugular bulb, aberrant internal carotid artery, and otosclerosis with a prominent Schwartze sign. 3. There are other benign tumors that arise from structures in the jugular foramen (nerves, dura, blood vessels) which so closely resemble the glomus jugulare tumor clinically and radiologically, that the diagnosis can only be made after careful histological evaluation of the tumor tissue. This report describes two patients having tumors closely mimicking glomus jugulare tumor with regard to both clinical and radiological presentation. Only after histological examination of the excised tumor mass could the diagnosis of schwannoma of the jugular foramen be made. Since the ultimate management of these tumors is different, the necessity for accurate tissue diagnosis is emphasized. CASE REPORTS
Case 1. P.L. is a 15-year-old female patient who was seen by a local otolaryngologist because she had failed a school hearing test. The patient also noted a pulsating tinnitus in the right ear. A reddish mass behind the inferior half of the right tympanic membrane was noted and a sensorineural hearing loss was documented in the right ear. Radiographic
From the Department of Otolaryngology, University of Massachusetts Medical School, Worcester, Massachusetts. 215
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Fig. 1. Base view tomogram of the skull showing the enlargement of the right jugular foramen (arrows) in Case 1. evaluation of the base of the skull showed a destructive lesion of the right jugular foramen typical of a glomus jugulare tumor. The patient was then referred to the author on July 26, 1974 for management. Examination revealed a reddish purple mass partially displacing the lower half of the tympanic membrane laterally. There was a questionable pulsatile appearance to this tumor mass. The remainder of the ear, nose and throat examination, and the cranial nerve evaluation was negative except for a partial right facial weakness and a moderately severe sensorineural hearing loss in the right ear. Conventional x-rays and poly tomes of the temporal bones revealed a large area of destruction in the jugular foramen which extended laterally to the tympanic cavity and medially to the hypoglossal canal (Fig. 1). Erosion of the undersurface of the petrous segment of the temporal bone and the inferior surface of the external auditory canal was also noted. The caroticojugular spine had been destroyed by the osteolytic lesion, and the tumor was noted to be in close apposition to the vertical portion of the facial nerve canal.
On September 24, 1974 surgical management of the tumor mass was carried out with six units of blood available. Following a postauricular incision which was extended down into the upper neck along the anterior border of the sternocleidomastoid muscle, the external carotid artery was identified and ligated just inferior to the ascending pharyngeal artery. At this time it was noted that the internal jugular vein was extremely small, measuring approximately 3 mm in diameter and flattened in appearance. Very little blood flow was present in this vein.
Audiometric evaluation confirmed normal hearing in the left ear and a severe sensorineural hearing loss in the right ear. Vestibular testing showed minimal depression of the caloric response in the right ear. A presumptive diagnosis of glomus jugulare tumor on the right was made and the patient was admitted to the hospital for further evaluation. A left vertebral and a right internal carotid arteriogram were normal. The right external carotid arteriogram demonstrated a 1.5 to 2 em vascular blush with small irregular vessels feeding from the ascending pharyngeal artery and located in the area of destruction of the jugular fossa (Fig. 2). This was felt to be most compatible with a glomus jugulare tumor.
Fig. 2. Right external carotid anteriogram in Case 1 showed a circular vascular mass (arrows) supplied by the ascending pharyngeal artery.
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Fig. 3. Drawing of the surgical exposure of the tumor mass in Case 1. A radical mastoidectomy was carried out and extended by removal of the tym.panic bone anteriorly and inferiorly around the tumor mass in the hypotympanum. After removal of the tympanic membrane, malleus and incus, a reddish purple lobulated tumor mass was seen filling the hypotympanum up to the level of the round window niche and extending from the carotid canal anteriorly to the vertical facial nerve segment posteriorly (Fig. 3 ). The tumor displaced the vertical portion of the facial nerve laterally and extended posteriorly to the dura of the posterior cranial fossa . The facial nerve at this point was noted to be flattened against the tumor. Delineation of the bony semicircular canals and the dura of the posterior cranial fossa and sigmoid sinus was carried out to establish the safe limits of maximum exposure before removal of the tumor mass.
A generous portion of the tumor was removed from the hypotympanum and sent for frozen section. Minimal bleeding was produced following this biopsy procedure and the tumor mass appeared to be more friable than glomus jugulare tumor tissue. The frozen section report indicated no evidence of glomus jugulare tumor; however, a definite diagnosis could not be made. Tumor removal was then carried out in piecemeal fashion with cup forceps and blunt dissectors until the dural surface of the undersurface of the temporal bone and the internal carotid artery wall was seen anteriorly. A small amount of tumor tissue was left posteroinferiorly in the region
of the jugular formen, because severe venous bleeding followed attempted tumor removal in this area. Approximately 75 to 80 percent of the tumor was successfully removed. The radical mastoidectomy cavity was maintained and the defect in the hypotympanum packed open. Two units of whole blood were replaced at surgery. The patient followed a benign postoperative course and left the hospital after three days. Pathological examination of the 'permanent sections of the tumor specimen indicated a neurolemmoma (schwannoma) (Figs. 4 and 5). The patient's ear healed rapidly within six weeks. She has a profound right-sided sensorineural hearing loss but has completely recovered facial nerve function within two months. Case 2. E.N. is a 59-year-old female patient who was first seen by her local otolaryngologist in December 1972 for progressive hoarseness. Examination at that time revealed paralysis of the left vocal cord, the left side of the tongue and the left sternocleidomastoid and trapezius muscles. A purplish discoloration in the anterior hypotympanum of the left ear was also noted on otoscopic examination. A presumptive diagnosis of glomus [ugulare tumor of the left side was made, and an exploratory tympanotomy was carried out. Very little material was available for biopsy in the middle ear; that tissue which was obtained showed only hypervascularity but no diagnostic abnormality. Since facilities were not
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Fig. 4. A) Photomicrograph of the tumor mass removed from Case 1 demonstrates the tumor cells somewhat loosely dispersed among blood vessels as in the Antoni B type schwannoma. (500X). B) Higher power photomicrograph of the center of the field in 4A shows the streaming tendency of cell nuclei. (1250X)
Fig. 5. Photomicrograph of a different area in the tumor removed from Case 1 showing a more typical histologic appearance of schwannoma. (500X)
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Fig. 6. Frontal tomograms through the temporal bones in Case 2. The cuts from top to bottom represent anterior to posterior levels through the bone. The large radiolucent defect (arrows) on the left appears typical for a glomus jugulare tumor. available for retrograde jugular venography, the patient was treated for a glomus jugulare tumor on the basis of clinical and radiological findings. Radiation therapy totalling 4,880 rads on the Cobalt unit were delivered in January 1973. Audiometric evaluation in February 1972 and January 1974 reportedly showed a mild high-tone loss in the left ear with normal hearing in the right ear.
Fig. 7. Left external carotid arteriogram in Case 2. The diffuse vascular flush (arrows) is supplied br the ascending pharyngeal and occipita arteries.
The patient had no change in signs or symptoms following radiation therapy until the Fall of 1974, when she was admitted to the hospital for left hemifacial spasm. At this time a neurosurgical consultant felt that the facial nerve finding indicated progression of the tumor, and a decision to operate was entertained. However, since the unchanged otologic findings and cranial nerve deficits pointed toward little or no tumor enlargement, surgical management was delayed and the patient was referred to the Massachusetts Eye and Ear Infirmary for management. Examination of this patient on October 1, 1974 at the Massachusetts Eye and Ear Infirmary revealed a normal right ear drum and a purplish discoloration behind the anteroinferior portion of an intact tympanic membrane in the left ear. The cranial nerve deficits were confirmed. These included atrophy and paralysis of the left side of the palate, the left
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Fig. 8. Drawing of the surgical exposure of the tumor mass in Case 2. half of the tongue, the left sternocleidomastoid and trapezius muscles, and the left vocal cord. In addition, 75% paralysis of the left side of the face was also noted. Audiometric evaluation revealed a total sensorineural hearing loss in the left ear and normal hearing in the right. Electronystagmography demonstrated a markedly diminished response on the left side. X-rays obtained on October 2, 1974 included both conventional and polytomes of the mastoid bones. A large destructive lesion of the left jugular foramen was noted. The area of destruction extended into the middle ear, the inferior part of the petrous apex, the adjacent portion of the occipital bone, and the region of the hypoglossal foramen (Fig. 6). This osteolytic defect was compatible with a large glomus jugulare tumor." The patient was admitted to the hospital on October 15, 1974 for additional diagnostic studies. Neurological evaluation confirmed deficits of the seventh through twelfth cranial nerves. A left external carotid arteriogram indicated a vascular mass in the left jugular foramen with feeding vessels arising from the ascending pharyngeal artery and branches of the occipital artery (Fig. 7). It was noted that this vascular blush was somewhat less pronounced than is usually seen with glomus iugulare tumors. Following discharge after the diagnostic evaluation, the patient was readmitted for surgical management of the vascular tumor in the left ear. With six units of whole blood available, the operation was carried out on
November 20, 1974. The postauricular incision was extended into the neck along the anterior border of the sternocleidomastoid, and the left external carotid artery was identified and ligated. The internal jugular vein could not be found in the carotid sheath and was thought to be completely atrophic. A radical mastoidectomy was carried out and extended to remove the tympanic bone anteriorly and inferiorly in such a manner as to skirt the perimeter of the tumor mass which filled the hypotympanum (Fig. 8). The bony labyrinth was skeletonized for surgical landmarks, and the bone over the vertical portion of the facial nerve was thinned down to a bony plate. The tumor in the hypotympanum appeared as a smooth dome-like mass with a pale tan color. It reached superiorly to the level of the round window niche and anteriorly through the carotid canal to make contact with the internal carotid artery wall. The posterior limit of the tumor in the jugular foramen was reached by removing the bone from the sigmoid sinus and the posterior fossa dura. A large segment of tissue from the hypotympanic mass was excised and sent for frozen section. The report indicated fibrous tissue but no evidence of glomus jugulare tumor. Approximately 90 percent removal of the tumor mass was accomplished in piecemeal fashion until the dural surfaces at the depth of the cavity could be seen. The blood loss was minimal and did not require replacement. Several myelinated nerves could be identified following the curvature of the surgical defect.
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Fig. 9. Photomicrograph of the tumor specimen removed from Case 2. A more distinct tendency for palisading of nuclei and the presence of abundant collagenous tissue represents the Antoni A pattern of schwannoma. (500X) The cavity was packed with Gelfoam®o as in a radical mastoidectomy. The postoperative course was uneventful and the patient was discharged on November 25, 1974. Permanent sections of the pathological specimen indicated schwannoma (Fig. 9). The out-patient follow up was characterized by rapid healing of the mastoid cavity in a three to four week period and a noticeable return of facial nerve function. The patient now has approximately 60% facial nerve function. No improvement in the other cranial nerve deficits has been noted. No further surgery is planned in this patient except for possible TeHon®oo injection of the left vocal cord after a 6 to 12 month period if there is no return of function. DISCUSSION
Diagnosis. Virtually no description of neurogenic tumors limited to the jugular foramen can be found in the English literature. This conclusion is supported by reports which reviewed glomus jugulare tumors,3.4.6 those which reviewed all neoplasms of the middle ear and mastoid.t" and those which summarized neurogenic tumors of the head and neck,lO-14 Simpson and Dallachy" and Alford and Cuilford! included neurofibroma of IX through XII
cranial nerves in the differential diagnosis of glomus jugulare tumor but could not cite documentation of this entity. Rosenwasser," Bradley and Maxwell," and Coodman" could only find schwannoma of the facial nerve presenting as a middle ear neoplasm. Conley.l" Putney et al,ll Katz et al,12 and Batsakis'" referred to the well known presentation of neurogenic tumors of IX through XII cranial nerves as tumors of the parapharyngeal space with or without extension into the base of the skull. Das Gupta et al 14 reviewed 303 cases of solitary schwannomas and found 136 occurred in the head and neck area. Although there were over 20 different locations for these tumors in the head and neck, the jugular foramen and middle ear were not included. This void may be due to the fact that these tumors are rare in this location or may suggest that they have not been recognized as neurogenic tumors but possibly called variants of glomus jugulare tumor. The clinical similarities of this tumor to glomus body tumors of the
° The Upjohn 00
Co., Kalamazoo, Mich. E.!. DuPont de Nemours & Co., Inc., Wilmington, DE
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jugular foramen are: 1) They occur onstrated after removal of a biopsy more commonly in female patients; 2) specimen. They may present clinically with pulsating tinnitus and hearing loss; 3) Oto- . The histological features of glomus scopic examination may reveal a red- jugulare tumors are unique and quite dish tumor mass in the hypotympanum; characteristic. The tumor cells are large, 4) The most commonly involved cranial polyhedral chief cells with small nuclei nerves are seven and eight, but the which are not hyperchromatic. Frenerves of the jugular foramen (nine, quently the cytoplasm of these cells has ten and eleven) and the hypoglossal a vacuolated appearance. These cells nerve may be affected in extensive tu- are characteristically arranged in groups mors; 5) Conventional x-rays and poly- (Zellbahlen) separated by fibrous tissue tomes of the jugular foramen reveal an septa containing blood vessels. enlargement of the jugular foramen; 6) Neurogenic tumors arise from proExternal carotid arteriography may liferation of the neurolemmal or demonstrate a vascular lesion supplied 13 15 by vessels from the ascending pharyn- schwann cells of myelinated nerves. • They are appropriately called neurogeal artery. lemmoma or schwannoma. These tumor Clinically no differentiation between cells are elongate spindle cells, usually these two tumors can be made. Even with large nuclei which can range from with conventional x-ray films of the a cigar shape to round. They are acbase of the skull and polytomes of the companied by thin fibrous strands jugular foramen, the osteolytic enlarge- which are usually arranged into mergment of the jugular foramen is indis- ing streams. Characteristically the nutinguishable in both types of tumors. clei tend to align themselves in a paliWhile arteriography shows a vascular sade pattern as in the Antoni A type flush with both types of tumor, it may (Fig. 9). However, there may be a wide be diagnostically significant that the degree of cell pleomorphism with irpattern in a neurofibroma is not as regular cell types dispersed in rather prominent as in the glomus jugulare loose connective tissue and no clear evidence of palisading (Antoni type B) tumor. (Fig. 4). These wide differences may Although a definitive diagnosis can- make the diagnosis of neurolemmoma not be made after clinical or radi- difficult. It is possible that such cases ological evaluation, the initial step in may be misclassified on the basis of management is the same. Surgical ex- clinical features. ploration of the middle ear and mastoid Although these tumors may arise from cavity must be carried out with sufficient blood available to replace the any of the cranial nerves passing blood loss usually associated with re- through the jugular foramen, it is not moval of glomus jugulare tumors. After always possible to identify the site of wide exposure of the tumor has been origin. When a functional nerve deficit achieved, tissue diagnosis from a frozen heralds the presentation of such a tusection is helpful. While the final histo- mor, it may point to the origin of the pathological diagnosis may not be ob- tumor as in Case 2. However, it is well tained at this time, it is usually possible known that these benign tumors may in the to determine the presence or absence of not produce a functional deficit 10 the characteristic chief cells of glomus nerve of origin as in Case 1. ,1 4 tumors. Other features of the tumor at Treatment. The most effective treatsurgery may provide clues to the dif- ment of both glomus tumors and neuroferentiation of these two types of tu- fibroma of the jugular foramen is surmors. The neurofibroma will have a gical removal. While radiation therapy pale tan appearance and its significantly may play a limited role in the control lower propensity to bleed will be dem- of glomus jugulare tumors that are un-
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resectable, this mode of therapy has no effect on neurogenic tumors. Accurate diagnosis is essential to avoid the administration of unnecessary radiation therapy to residual neurogenic tumor tissue on the assumption that it represents glomus body tumor.
with regard to subtotal removal of the tumor, it would appear that the otological approach is the preferred choice because of the low morbidity associated with it, and the exteriorization of the tumor capsule into an area that can be observed (mastoid cavity).
There are two surgical approaches to the jugular foramen: 1) The neurosurgical approach via the posterior cranial fossa carries a high incidence of morbidity and probably would not result in complete removal of tumor since the tumor extends inferiorly through the jugular foramen and laterally into the mastoid and middle ear. 2) The otological approach carries a low morbidity to the patient and may not result in complete removal of the tumor, since the medial extent of a large tumor in this region may not be reached. However, the tumor area may be considered exteriorized and lend itself to follow up observation. Since slow growth is generally characteristic of this type of tummor, long survival may be associated with complete or subtotal tumor removal.
CONCLUSION
Since both surgical approaches achieve essentially the same end result
These two case reports illustrate the close similarity which schwannoma of the jugular foramen may have to glomus jugulare tumors. While some indication as to the nature of the tumor may be provided by close study of the angiographic pattern, only surgical exploration and a tissue diagnosis can produce an accurate diagnosis. Even in cases where tissue has been obtained for histological evaluation, the wide range of histological patterns which these neurogenic tumors may assume makes it important to study closely all areas of the tumor tissue. It is possible that neurogenic tumors of the jugular foramen have been interpreted both clinically and histologically as glomus jugulare tumors. Such may be the case with those glomus jugulare tumors which have been considered atypical in their relative lack of vascularity and histological pattern.!"
Reprints: Richard R. Gacek, M.D., Dept. of Otolaryngology, Univ, of Massachusetts Medical School, 55 Lake Ave. North, Worester, MA 63110. REFERENCES I. Riemenschneider PA, Hoople GD, Brewer D, et al: Roentgeongraphic diagnosis of the glomus [ugulare, Am J Roentgenol Radium Ther Nucl Med 69 :59-65, 1953 2. Holesh S: Diagnosis of tumors of the glomus jugulare. Lancet 268:169-170, 1955 3. Simpson IC, Dallachy R: A review of tumors of the glomus jugulare with reports of three further cases. J Laryngol Otol 72: 194 226, 1958 4. Alford BR, Guilford FR: A comprehensive study of tumors of the glomus [ugulare. Laryngoscope 72:765-787, 1962
mors, Arch Otolaryngol 88:29-66, 1968 7. Rosenwasser H: Neoplasms involving the middle ear. Arch Otolaryngol 32:38-53, 1940 8. Bradley WH, Maxwell JH: Neoplasms of the middle ear and mastoid. Report of fifty-four cases. Laryngoscope 64:533-556, 1954 9. Goodman ML: Middle ear and mastoid neoplasms. Ann Otol Rhinol Laryngol 80:419-424, 1971 10. Conley JJ: Neurogenous tumors in the neck. Arch Otolaryngol 61: 167-180, 1955
5. Valvasorri GE, Buckingham RA: Middle ear masses mimicking glomus tumors: Radiographic and otoscopic recognition. Ann Otol Rhinol Laryngol 83:606-612, 1974
II. Putney FJ, Moran JJ, Thomas GK: Neurogenic tumors of the head and neck. Laryngoscope 74:1037-1059, 1964
6. Rosenwasser H: Glomus jugulare tu-
12. Katz AD, Passy V, Kaplan L: Neuro-
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genous neoplasms of major nerves of face and neck. Arch Surg 103:51-56, 1971 13. Batsakis ]G: Tumors of the peripheral nervous system, in Tumors of the Head and Neck. Baltimore, The Williams and Wilkins Company, 1974 chap 16 14. Das Gupta TK, Brasfield RD, Strong
WE, et al: Benign solitary schwannomas (Neurilemomas). Cancer 24:355-366, 1969 15. Dykstra PC: The pathology of acoustic neuromas. Arch OtolaryngoI80:605-616, 1964 16. Mattick WL, Burke EM: Glomus [ugulare tumors. Laryngoscope 62:311-322, 1952
PSYCHOSOCIAL CARE OF THE DYING PATIENT The first national training conference for physicians on Psychosocial Care of the Dying Patient: Doctor-Patient Relationships in Terminal Illness, will be held April 29-May 1, 1976 at the St. Francis Hotel, San Francisco, California. This program will be presented by the Cancer Research Institute and Extended Programs in Medical Education, University of California School of Medicine, San Francisco, California.
CONFERENCE The Second International Conference on the Diagnosis and Treatment of Craniofacial Anomalies will be held at the New York University Medical Center, New York, New York on May 3-5, 1976. The Conference is organized under the auspices of the Educational Foundation of the American Society of Plastic and Reconstructive Surgeons. The Conference will focus on four major subject areas: orbitocephalic malformations, including orbital clefts and hypertelorism, craniofacial synostosis syndromes, mandibulofacial dyostosis (Treacher-Collins Syndrome) and craniofacial microsomia (maldevelopment of the first and second branchial arches). Emphasis will be placed on etiology, embryology, genetics, classification, pathology and the role of the different clinical specialties in the diagnosis and treatment of these anomalies. Special sessions will be devoted to associated speech and airway problems, helical deformities, audiology and middle ear surgery. For detailed information please write: John Marquis Converse, M.D., Institute of Reconstructive Plastic Surgery, 550 First Avenue, New York, New York 10016.
EUROPEAN SYMPOSIUM A European Symposium, Neurophysiology of Hearing: Interpretation of Human Electrophysiological Data, sponsored by the European Economic Community (Medical Research Committee: Ad hoc Working Group on Deafness) and organized by M. Portmann and J. M. Aran, will be held May 20-22, 1976, in Bordeaux, France. Registration fees: 350.00 F.F. For further information write: M. Portmann, 114 Avenue d'Ares, 33000 Bordeaux, France.
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