Long-term effects of radiation therapy for a catecholamine-producing glomus jugulare tumor

Case report

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✓ A 42-year-old woman presented with otorrhea 22 years after extracranial resection of a norepinephrinesecreting glomus jugulare tumor with intravascular embolization and radiation therapy to the intracranial portion of the tumor. Tumor growth was arrested and was associated with a decrease in blood and urine norepinephrine levels. Extensive evaluation of the otorrhea, including computerized tomography-cisternography, gadoliniumenhanced magnetic resonance imaging, and arteriography showed marked diffuse necrosis of the temporal bone and skull base with limited tumor vascularity. Cerebrospinal fluid (CSF) collected from the right ear showed norepinephrine levels of 2975 pg/ml; plasma norepinephrine levels were normal. The precise site of CSF leakage could not be delineated. Exploration of the posterior fossa revealed a large dural defect at the anteromedial aspect of the petrous bone through which CSF flowed over the surface of the residual extradural glomus tumor. The defect was successfully sealed with a fascial patch. Postoperatively, CSF norepinephrine levels were normal and no further leakage was observed.

Article Information

Address reprint requests to: Ryszard M. Pluta, M.D., Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, Building 10, Room 5D37, National Institutes of Health, Bethesda, Maryland 20892.

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    Right carotid arteriograms of the glomus jugulare tumor obtained in 1970. Left: Lateral arteriogram showing intracranial and extracranial tumor blush. The main tumor-feeding artery (arrow) is a branch of the middle meningeal artery and the vessel targeted for embolization. Right: Postembolization arteriogram demonstrating diminished intracranial tumor blush.

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    Studies performed in July, 1992. Left: Iopamidol-enhanced computerized tomography-cisternogram showing the intracranial portion of the tumor (small arrows), bone erosion of the right pyramid, and the possible site of cerebrospinal fluid leakage directly above the posterior part of the tumor (curved arrow). Contrast material is present in the mastoid air cells. Center: Contrast-enhanced magnetic resonance image demonstrating an enhancing mass in the same location (arrows) as shown left. Right: Right carotid arteriogram showing tumor blush similar to that shown in the postembolization study (Fig. 1 right) and permanent occlusion of the main tumor-feeding artery.

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