Adrenal Myelolipoma Associated with Congenital Adrenal 21-Hydroxylase Deficiency

Chika MURAKAMI, Miyuki ISHIBASHI, Masahiro KONDO, Sumako OHSHIRO, Mutsumi FUJITA, Shigenobu SATO, Makoto KAKO, Hisashi FURUE, Kunio MIZUGUCHI, Tohru YAMAJI
1992 Internal medicine (Tokyo. 1992)  
The occurrence of adrenal myelolipomas is reported in an untreated patient with congenital adrenal 21-hydroxylase deficiency. Laparotomy demonstrated the presence of two lesions, a large tumor which arose from an ectopic adrenal cortex and a smaller tumor in the left adrenal gland. Six cases of adrenal myelolipomas and congenital adrenal hyperplasia have been reported in the literature. All patients were associated with excessive ACTH secretion for a long period of time. The relative frequency
more » ... relative frequency of this association, coupled with the observation by Selye and Stone (Am J Pathol 26:211, 1950) that anterior pituitary extracts cause myelolipomatous changes in rats, may indicate a possible role for ACTH in the development of myelolipomas. (Internal Medicine 31: 803-806, 1992) Key words: extra-adrenal myelolipoma, congenital adrenal hyperplasia, ACTH 18.1 g/dl; erythrocyte count, 5,370,000//J; hematocrit, 53.3% ; platelet count, 260,000/^1; and leukocyte count, 9,900//il with 53% neutrophils, 9% eosinophils, 1% monocytes, and 37% lymphocytes. Urine evaluation did not show any abnormalities. Serum total protein was 7.3g/dl, blood urea nitrogen 8.0mg/dl, creatinine l.Omg/dl, total cholesterol 124mg/dl, glutamic oxalo-
doi:10.2169/internalmedicine.31.803 fatcat:6nf6gs4zefdjbo7kfi5756lfbu