Abstract

This paper referred to primary immunodeficiency diseases (PID)-malignancy association in autopsy cases in Japan. The occurrence of malignant neoplasms almost centered upon ataxia-telangiectasia among PID in Japan. It seems to be due to extremely shorter life span in Japanese patients with PID except for in those with ataxia-telangiectasia, compared with that in European and American patients. Most of the malignant neoplasms seen in Japanese patients with PID were epithelial and were seen mostly in older patients, while lymphoreticular tumors were rare. Gastric cancer was the most frequent of the epithelial tumors.

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