一家族にみられた2例の頸動脈小体腫瘍

抄録

Carotid body tumor is a rare neoplasm, occasionally familial in occurence, is difficult to establish its malignancy histopathologically. In this paper, we report two cases seen in the same family. As far as our survey concerned present report on the familial incidence is the second in Japan.
Case 1: I. K., 13 years old girl.
She visited a hospital with complaints of the neck mass and low grade fever in Oct. 1985. Open biopsy of the neck mass was performed and the histopathological diagnosis was chemodectoma originated from the carotid body. She was referred to the ENT department of Kitasato University Hospital in Jan. 1986. Our initial impression was that the tumor was so large that in order to remove the tumor completely, it was neccessary to make a by-pass of the carotid artery, and the aurgery was deferred until her grow-up completed.
Operation was done in May 1987, because the tumor began to grow rapidly. During operation, the electro-encephalogram was monitored and the by-pass of the carotid artery by using the autograft of the jugular vein was performed. The removed tumor size was 70×50×35mm and histopathological study revealed benign type of carotid body tumor.
Case 2: I. T., 15 years old boy (the elder brother of the case 1)
At his sister's visit to the hospital, he happened to find out a neck mass in the left side of his neck.
The operation was done under general anetshesia with controlled hypothermia. Electroencephalogram was also monitored. The removed tumor was 40×35×25mm. Histopathological find was similar to his sister's case.
No regional cervical lymphnode metastasis was seen in both cases. Neither autosomal analysis nor HLA typing showed particular abnormal result. Since it has been reported that even the histopathologically benign carotid body tumor show late metastasis in recurrence, it is firmly suggested that follow-up study is important especially for young patient with familial incidence.