MALIGNANT PARAGANGLIOMA PRESENTING AS CUSHING SYNDROME WITH VIRILISM IN CHILDHOOD - PRODUCTION OF CORTISOL, ANDROGENS, AND ADRENOCORTICOTROPIC HORMONE BY THE TUMOR

Background. A 12-year-old girl with intractable retroperitoneal paraga nglioma experienced increased appetite, acne, obesity, ''moon face,'' and enlargement of the clitoris during the course of the tumor. Plasma cortisol, serum testosterone, and dehydroepiandrosterone sulfate (DHE A-S) levels were increased to 34.1 mug/dl, 2.0 ng/ml, and 6.628 ng/ml, respectively. Adrenocorticotrophic hormone (ACTH) levels were not inc reased, and results of dexamethasone suppression tests were negative. Her condition was diagnosed as Cushing syndrome with virilism. Plasma cortisol levels were increased to a level of 107.1 mug/dl before death . Methods. Tumor samples were obtained at the time of autopsy. The con centrations of cortisol, androgens, ACTH, and catecholamines were assa yed in the tumor extracts. The indirect immunoperoxidase procedure was performed on fixed tissues for cortisol, DHEA-S, testosterone, and AC TH. Results. Extracts of the tumor masses contained steroid hormones: the amount of immunoreactive cortisol was 1.64 mug/g wet weight; the a mount of immunoreactive testosterone was 25.60 ng/g wet weight; immuno reactive DHEA-S, 579.00 ng/g wet weight; and immunoreactive ACTH, 891. 00 pg/g wet weight in the metastatic mass of the lung. Immunohistochem ically, immunoreactive cortisol, testosterone, and DHEA-S were detecta ble in the tumor cells. The adrenal gland was atrophic. Conclusions. T he patient is the first reported with malignant paraganglioma with the capacity to produce cortisol, androgens, and ACTH.