Leydig cell tumor and malignant lymphoma in a patient with nonclassical 21-hydroxylase deficiency.

A 46-year-old man was admitted in our hospital with hypoglycemia; his FPG was 43 mg/mL. Five years earlier, he underwent simultaneous surgeries for an adrenal adenoma, a benign Leydig cell tumor (LCT), and a malignant lymphoma. Based on the laboratory results, he was diagnosed as congenital adrenal hyperplasia (CAH) due to nonclassical 21-hydroxylase deficiency (21-OHD). On immunohistochemistry analysis using the antibody against adrenal-specific 11beta-hydroxylase antibody, the LCT showed both properties as a testicular cell and as an adrenal cell. The genetic background of 21-OHD might contribute to the development of malignant lymphoma. Such as a case of LCT and malignant lymphoma in a patient with 21-OHD seems to be rare.