17-ketosteroids and Primary-Myelofibrosis

The occurrence of a giant myelolipoma of the adrenal gland reported in a patient with congenital adrenal hyperplasia (21-hydroxylase deficiency). Associated significant findings include a massive proliferation of adrenocortical cells as an integral part of the myelolipoma and coincidental tumor of the interstitial cells of the testis. The clinical, radiologic, endocrinologic, and pathologic features of this case are correlated with a review of the literature. The additional myelolipomas are also reported here for the first time. Similar lesions have been induced experimentally in rats and provide further evidence suggesting a hyperplastic rather than a neoplastic nature for this complex lesion, at least in its earlier stages.

Topics: 17-Ketosteroids; Adrenal Cortex; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Adrenocorticotropic Hormone; Adult; Age Factors; Chronic Disease; Diagnosis, Differential; Female; Humans; Male; Microscopy, Electron; Middle Aged; Primary Myelofibrosis; Testicular Neoplasms

Topics: 17-Ketosteroids; Adrenal Gland Neoplasms; Adrenal Hyperplasia, Congenital; Female; Hirsutism; Humans; Hydrocortisone; Lipid Metabolism, Inborn Errors; Middle Aged; Primary Myelofibrosis

Topics: 17-Hydroxycorticosteroids; 17-Ketosteroids; Adrenal Insufficiency; Adrenocorticotropic Hormone; Aged; Busulfan; Female; Humans; Male; Metyrapone; Middle Aged; Pituitary Diseases; Pituitary-Adrenal System; Polycythemia Vera; Primary Myelofibrosis; Splenectomy; Thyroid Function Tests