Glomus jugularis tumor of the middle ear; a case report.

Abstract

Primary tumors of the middle ear are extremely rare. Schall reported the incidence as 1 in 6,000 cases of disease of the ear. Marx collected 155 cases, of which the majority were squamous-cell carcinomas. His series included, also, 15 cases which he described as endothelioma, and among this number may well be some examples of what has more recently come to be known as “glomus jugularis tumor.” Most of the symptoms of these tumors are non-specific. Deafness, headache, facial paralysis, dizziness, discharge from the ear, and soft, dark, purplish masses in the external canal are the signs and symptoms. Progressive expansive growth leads to destruction of the temporal bone, as a result of pressure atrophy, and extension into the cranial cavity with compression of cranial nerves. Hemorrhage makes operative removal rather difficult. The microscopic picture is generally comparable to that of tumors of the carotid body. The tumor is vascular, with “epithelioid cells” lining the blood spaces. The terms “glomus jugularis tumor” and “carotid body-like tumor” have come into use in the last five or six years, following a report by Rosenwasser in 1945 describing what he called a carotid body tumor in the middle ear, extending into the mastoid process, in the absence of a demonstrable tumor of the carotid body in the neck. The origin was believed to have been in the paraganglion structure in the adventitia of the bulb of the jugular vein or along the ninth cranial nerve. In a tentative explanation of the occurrence of a carotid body tumor at this site, Rosenwasser went back to a communication by Guild in 1942, describing the glomus jugularis in the temporal bone. “Human temporal bone sections,” wrote Guild, “reveal structures in several respects like the carotid body, for which the name glomus jugularis is proposed. Usually they are found in the adventitia of the dome of the jugular bulb, immediately below the bony floor of the middle ear and near the ramus tympanicus of the glossopharyngeal nerve… Presumably it has functions like the carotid body, perhaps limited to a smaller circulatory region.” It was suggested that like structures might be found along other portions of the circulatory system. Glomus tumors, which are tumors of highly specialized glomic arteriovenous anastomoses, can develop not only at the junction of skin and subcutaneous tissues but may also occur in deeper structures. Murray states that glomus tumors may be infiltrative, indicating that not all are thoroughly encapsulated. The epithelioid cell is the same as the so-called “pericyte” of Zimmermann, which is a type of cell transitional between an endothelial capillary cell and a smooth muscle cell. This identification offers a satisfactory explanation for the occurrence of glomus tumors in sites where normal glomera have not been found. Rosenwasser in reporting his case listed the x-ray findings as revealing good pneumatization on both the right and left side in the mastoid region.