Leydig Cell Tumor and Malignant Lymphoma in a Patient with Nonclassical 21-Hydroxylase Deficiency

• Published in: Internal Medicine Vol. 48; no. 8; pp. 601 - 605
• Main Authors: Inaba, Hidefumi, Suzuki, Satoru, Shigematsu, Satoshi, Shinomiya, Ken, Ohfusa, Hirokazu, Shimojo, Yasuyo, Uehara, Takeshi, Hashizume, Kiyoshi
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.01.2009
• Subjects: 21-OHD; Adrenal Gland Neoplasms - etiology; Adrenal Gland Neoplasms - surgery; Adrenal Hyperplasia, Congenital - complications; Adrenal Hyperplasia, Congenital - diagnosis; Adrenocortical Adenoma - etiology; Adrenocortical Adenoma - surgery; CAH; Humans; hypoglycemia; Hypoglycemia - diagnosis; Hypoglycemia - etiology; Leydig cell tumor; Leydig Cell Tumor - etiology; Leydig Cell Tumor - surgery; Lymphoma - etiology; Lymphoma - surgery; Male; malignant lymphoma; Middle Aged; Testicular Neoplasms - etiology; Testicular Neoplasms - surgery
• ISSN: 0918-2918; 1349-7235; 1349-7235
• DOI: 10.2169/internalmedicine.48.1733

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 11β-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.