Glioma of the jugular foramen Case report

S~UNG H. LrE, M.D., MORRIS A. OSRORN, M.D.,

Ant) WILUAM A. BucnnEIT, M.D.

Departments of Radiology, Neurology, and Neurosurgery, Temple University Hospital, 3401 North Broad Street, Philadelphia, Pennsylvania v' A case is presented of a jugular foramen syndrome caused by an ectopic glioma. Treatment was by intracapsular removal through a suboccipital craniectomy. KE+WORDS 9 ectopicglioma

9 jugular foramen

E

CTOPIC glial tumors have been found Basilar skull radiographs showed in the leptomeninges, t,~ the nasal questionable cortical erosion of the left cavities/ the bridge of the nose and jugular foramen. This could not be confirmed face/ the soft palate, the pericranium, in- by tomography. Left brachial and left carotid tradural extramedullary space of the spinal arteriograms did not reveal any abnormality. canal, and extraspinal space of the lum- Pantopaque cisternogram revealed a smooth bosacral region? a filling defect on the left side of the clivus We believe that our case of ectopic glioma probably in the region of the left jugular of the jugular foramen is the first to be foramen. A retrograde jugular venogram by reported in the literature. percutaneous puncture of the left internal jugular vein demonstrated non-filling of the left jugular bulb, with good crossfilling to the Case Report contralateral right side by way of the caverThis 47-year-old man was admitted nous sinus (Fig. 1). because of increasing hoarseness and Operation. The patient underwent a subocprogressive dysphagia for 1 year. cipital craniectomy under general anesthesia Examination. There was bilateral sensory without complications. A small (1 to 11/2cm) neural loss of hearing. The uvula deviated to grayish mass was found adherent to the left the left and the tongue protruded in the 9th, 10th, and l lth cranial nerve roots midline. The gag reflex was diminished between the brain stem and the jugular bilaterally, particularly on the left. There was foramen. The tumor was decompressed by an weakness and atrophy of the left sterno- intracapsular removal. The capsule, which cleidomastoid muscle and partial loss of the was densely adherent to the nerve roots, was left trapezius muscle. No sensory impair- left. Microscopic examination of the tumor ment, long tract signs, or papilledema were tissue revealed low-grade glioma (Fig. 2). No noted. radiation therapy was given.

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S. H. Lee, M. A. O s b o r n a n d W. A. B u c h h e i t

Postoperative Course. The patient's postoperative course was complicated by persistent difficulty with swallowing and ultimately a gastrostomy was done to facilitate feeding. He was subsequently discharged, and when seen 1 year later there was no further progression of his neurological deficit. Discussion

F~c. 1. Left retrograde jugular venogram in the submentovertex projection demonstrates nonfilling of the left jugular bulb with irregular inferior border (L). Reflux into the left external jugular vein and crossfilling to the contralateral side reveals normal right jugular bulb (R).

FIG. 2. The tumor is basically formed by medium-sized glial cells with multiple cell processes. Fine glial fibers are seen within the tumor cells as well as in the stroma. The diagnosis was low-grade (I-II) glioma. H & E, • 369. 494

The jugular foramen is divided into the "pars nervosa" and "pars vascularis," and is traversed by the glossopharyngeal (9th), vagus (10th), and spinal accessory (llth) nerves, the inferior petrosal sinus, the posterior meningeal artery, and the internal jugular vein. The size and shape of the jugular foramen vary considerably. Asymmetry of the two sides is frequent and is attributed primarily to the dimensions of the pars vascularis, which is dependent upon the size of the transverse sinus. ',5 Erosion of the cortical margin and not asymmetry of the foramen should be considered as an abnormal finding. In our case, the submentovertex view of the skull in varying degrees with and without tomograms failed to demonstrate erosion of the left jugular foramen with certainty. Pantopaque cisternography revealed a smooth round defect on the left side of the clivus probably in the endocranial opening of the left jugular foramen. A pneumoencephalogram was not done in this case but may be helpful to determine intracranial extension. The most definitive study in the evaluation of a jugular foramen tumor and its extension is the retrograde jugular venogram in submentovertex view via either femoral vein catheterization or direct percutaneous puncture as was demonstrated in our case (Fig. 1). The clinical findings in our case most closely resemble the jugular foramen syndrome of Vernet which is caused by a lesion in the region of the jugular foramen characterized by ipsilateral involvement of the 9th, 10th, and 1lth nerves. This syndrome is usually of traumatic origin and follows a basal skull fracture. Vascular and neoplastic lesions, thrombosis of the jugular bulb, and aneurysm of the internal carotid artery may also be etiological factors. Absence of Horner's syndrome separates our case from the jugular foramen syndrome of Villaret. Tumors of the glomus jugulare show involvement of the 9th, 10th, and 1lth nerves, and

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Glioma of the jugular foramen there is usually some evidence of 7th and 8th nerve dysfunction as well. Our patient had longstanding bilateral 8th nerve dysfunction probably coincidental to the jugular foramen lesion. A progressive unilateral involvement of multiple lower cranial nerves should suggest possible neoplastic invasion along the floor of the cerebellar fossa. Acknowledgments We wish to express great appreciation to Dr. G. Tucker for his tremendous help in the management of this patient and Dr. G. DeLeon for his extensive review of the microscopic examination. Special appreciation to Dr. M. Scott for his critical review. References 1. Abbott KH: Intracranial extracerebral (leptomeningeal) glioma. Follow-up on previously reported case. Bull Los Angeles Neurol Soc 24:31, 1959 2. Bailey OT: Relation of glioma of the leptomeninges to neuroglia nests: report of case of astrocytoma of leptomeninges. Arch Pathol 21:584-600, 1936

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3. Cooper IS, Craig WMcK, Kernohan JW: Tumors of spinal cord: primary extramedullary gliomas. Surg Gynecol Obstet 92:183-190, 1951 4. DiChiro G, Fisher RL, Nelson KB: The jugular foramen. J Neurosurg 21:447-460, 1964 5. Gray H: Anatomy of the Human Body. Goss CM, editor. Philadelphia: Lea & Febiger, 1973 6. Kern WH, MacDonald I: Congenital glioma on the left side of the face. Calif Med 95:393-396, 1961 7. Rubinstein L J: Tumors of the central nervous system, in: Atlas of Tumor Pathology. Washington DC: Armed Forces Institute of Pathology, 1972, pp 296-297 8. Smith KR Jr, Schwartz HG, Luse SA, et al: Nasal gliomas: a report of five cases with electron microscopy of one. J Neurosurg 20: 968-982, 1963

Address reprint requests to: Seung H. Lee, M.D., Department of Radiology, Temple University, Health Sciences Center, Philadelphia, Pennsylvania 19140.

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Glioma of the jugular foramen. Case report.

A case is presented of a jugular foramen syndrome caused by an ectopic glioma. Treatment was by intracapsular removal through a suboccipital craniecto...
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