Endocrine studies in a patient with functioning adrenal rest tumor of the liver

We report a case of a calcified liver tumor in a 23 year old female patient who presented with virilization and a mild degree of Cushing's syndrome. Androgen levels were elevated; there was loss of cortisol circadian rhythm and marked increase in urinary 17-ketogenic and 17-ketosteroids which failed to suppress with administration of dexamethasone. Venous sampling by inferior vena cava catheterization showed that the highest steroid hormone levels were in blood from the right hepatic vein. After death, in vitro studies revealed that the tumor contained testosterone and cortisol as determined by immunofluorescence techniques. The adrenals and ovaries were atrophic. Results of metyrapone testing indicated dyshormonogenesis. To our knowledge, this is the first case of an adrenal rest tumor of the liver proved to be functionally active.