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Young Female Patient with Testosterone-Producing Adrenocortical Adenoma Also Showing Signs of Subclinical Cushing's Syndrome

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Young Female Patient with Testosterone-Producing Adrenocortical Adenoma Also Showing Signs of Subclinical Cushing's Syndrome Endocrine Journal 1995, 42 (2), 283-288 NOTE Young Female Patient with Testosterone-Producing Adrenocortical Adenoma Also Showing Signs of Subclinical Cushing's Syndrome YosHlo MURAKAMI, MIHARUSASAKI*, HIRONOBUSASANO***, TAKASHISUZUKI * * *, NoBUYASUKITAMURA* *, MASAHIROTOMOI**, HIROSHIYORIFUJI#, KuNIo KOSHIMURA ANDYUZURU KATO First Division, Department of Medicine, Shimane Medical University, Izumo 693, *Urology , **Radiology and #Gynecology and Obstetrics, Shizuoka City Hospital, Shizuoka 420 and ***Department of Pathology, Tohoku University School of Medicine, Sendai 980, Japan Abstract. A 28-year old female patient with virilization due to left adrenocortical adenoma was studied. The patient had clinical features of hyperandrogenism such as hirsutism and a low pitched voice, but not of hypercorticoidism. Plasma testosterone and dehydroepiandrosterone-sulfate (DHEA-S) were high. Although the basal plasma cortisol concentration and urinary excretion of 17-hydroxycorticosteroids (17- OHCS) were within the normal range, the absence of diurnal variation in plasma cortisol and loss of suppressibility by dexamethasone suggested constitutive secretion of cortisol by the tumor. Inappropri- ate cortisol secretion was also supported by blunted ACTH response to provocative stimuli. After successful removal of the left adrenal tumor, such endocrinological abnormalities were all normalized. Immunohistochemical analysis revealed that tumor cells were positively stained for C21 hydroxylase cytochrome P-450 (P-450C21) and P-45011 which convert 17-hydroxy (OH) progesterone to cortisol as well as P-450SCC,3/3-hydroxysteroid dehydrogenase and P-45017awhich are involved in testosterone bio- synthesis. These findings suggest that adrenocortical adenoma secretes predominantly testosterone and constitutively cortisol in a young woman patient with virilization. Key words: Adrenocortical virilization, Adenoma, Subclinical Cushing's syndrome, Immunohistochemistry (Endocrine Journal 42: 283-288,1995) VIRILIZING adrenocortical adenoma is a rare nied by hirsutism, plasma androgen concentrations cause of androgen excess in young women and including testosterone, androstenedione, dehydroe- there have been only forty cases reported [1]. Tes- piandrosterone (DHEA) and DHEA-sulfate tosterone is considered to be a major steroid (DHEA-S) are moderately increased [3]. In some hormone produced by these tumors [1, 2]. The patients with Cushing's syndrome, however, adre- plasma testosterone concentration was high in such nal androgen is rather decreased due to atrophic virilizing patients [1]. changes in the contralateral adrenal gland [4]. In patients with Cushing's syndrome accompa- There has been only one case report of a young woman with Cushing's syndrome associated with Received: August 1, 1994 a very high plasma testosterone concentration [5]. Accepted: November 25, 1994 In this report, we describe a young female pa- Correspondence to: Dr. Yoshio MURAKAMI, First tient with virilizing syndrome due to benign Division, Department of Medicine, Shimane Medical adrenocortical adenoma producing predominant- University, 89-1 Enya-cho, Izumo 693, Japan ly testosterone and constitutively cortisol, which 284 MURAKAMI et al. was evaluated immunohistochemically with spe- physical and neurological examinations. Fasting cific antibodies to enzymes involved in adrenal plasma glucose was 4.7 mmol/L. Serum Na, K steroidogenesis. and Cl concentrations were 137, 3.9 and 105 mmol/ L, respectively. Polycythemia was not noted. Steroid hormone concentrations are shown in Patient Table 1. Plasma testosterone, ll-deoxycortisol and DHEA-S levels were high. The basal plasma corti- A 28-year-old Japanese woman presented with sol level was within the normal range but the hirsutism and low pitched voice. She had been normal diurnal rhythm was absent. Urinary ex- oligomenorrheic since her menarche at the age of cretion of 17-ketosteroids (17-KS) and 17-OHCS 16. Five years ago she was noticed to have a low- was moderately increased but urinary cortisol was pitched voice and hirsutism in the perioral region. within the normal range. Plasma cortisol and During the last 4 years she was amenorrheic and DHEA-S were not suppressed by dexamethasone the low-pitched voice and hirsutism have been get- administration (2 mg/day for 7 days). Neither ting more prominent. urine 17-OHCS nor 17-KS was decreased during At physical examination, she was a well-nour- the administration of dexamethasone. Plasma es- ished woman with signs of virilization. Body tradiol was normal. Basal plasma ACTH was low weight was 43.5 Kg and body height was 154 cm. and did not respond to pharmacological stimuli Blood pressure was 130/72 mmHg. Such Cushin- (Table 2). Other pituitary hormones concentrations goid appearances as moon face, buffalo hump, skin including GH, prolactin, TSH, LH and FSH were striae and central obesity were not present. Her within the normal range (data not shown). A voice was low-pitched and pubic hair was of the venous catheterization study revealed that plasma male type. Body hair was predominant in the pen- testosterone, ll-deoxycortisol and cortisol were in- oral region, limbs and lower abdominal wall. The creased in the left adrenal vein. breasts were normally developed and not atro- CT scan detected a round tumor with a diame- phied. At gynecological examination, the uterus ter of 3 cm in the left adrenal region. The tumor was slightly small and mild clitomegaly was not- was homogeneous and its margin was regular. ed. There were no other abnormal findings at There were no abnormal findings in the ovaries or Table 1. Plasma and urine steroid concentrations in a young female patient with viri lism due to left-adrenal adenoma before and after surgery CORTISOL IN VIRILIZING ADENOMA 285 the uterus. also positive especially in compact cells. The ad- The left adrenal tumor and the attaching left ad- renal cortex attached to the adenoma was atrophic, renal gland were surgically resected. After and immunoreactivity of P-45017a was detectable successful surgery, the plasma testosterone and only in scattered cortical cells in the reticular zone DHEA-S concentrations promptly dropped into the (Fig. 2d). normal range. Plasma cortisol was low for several days after the surgery and then returned to the normal range. Two weeks after the surgery, the Discussion normal diurnal rhythm of plasma cortisol was ob- tained and ACTH responses to provocative stimuli In this report, we have described a young fe- were recovered (Table 2). Replacement therapy male case with virilization due to left-adrenal with hydrocortisone was not required. adenoma. The 2.8 cm diameter, the homogenous Concentrations in plasma steroids and pituitary image and the clear margin in CT scanning, the hormones were determined with commercial RIA absence of metastasis and the histlogy indicated kits. the benign nature of the tumor. Although both plasma testosterone and DHEA-S were increased, the main steroid hormone secreted from the tumor Pathology and Immunohistochemistry was considered to be testosterone. Levine et al. [10] reported that plasma testosterone as high as The tumor was 2.8 x 2.8 cm in size, dark colored in normal men were found in females with adre- and well circumscribed. Hematoxylin-Eosin stain- nal carcinoma but not with adrenal adenoma, but ing revealed that it was histologically composed Schteingart et al. [5] reported the case of a young dominantly of compact and of clear cells with tra- woman with Cushing's syndrome due to adreno- becular and acinal arrangements (Fig. 1). There was no sign of malignancy. The presence of enzymes involved in steroid hor- mone biosynthesis was analyzed by an immunohistochemical method as described previ- ously [6-9]. The tumor cells possessed all the three enzymes involved in testosterone biosynthesis, P- 450scc (not shown), 3/3-hydroxysteroid dehydro- genase (not shown) and P-45017a (Fig. 2a). Stain- ing of P-450C21 (Fig. 2b) and P-45011 (Fig. 2c), which are involved in cortisol biosynthesis, was Table 2. Basal and stimulated plasma ACTH concentrations in a young female case with virilism due to left- adrenal adenoma before and after surgery Fig. 1. Histology of the adrenocortical tumor (Hematoxylin-Eosin stain) (x 50). The tumor is composed of compact and clear cells. There were no signs of malignancy such as capsular or vascular invasion, increased mitotic activity, cellular atypism or pleomorphism. 286 MURAKAMI et al. cortical adenoma which was associated with a very of a normal diurnal cortisol rhythm and the loss of high plasma testosterone concentration. suppressibility by dexamethasone as well as low The basal plasma cortisol concentration and dai- plasma ACTH suggested autonomous constitutive ly urinary cortisol excretion remained within the cortisol secretion by the tumor. Plasma ACTH re- normal range in the present case, but the absence sponses to insulin-induced hypoglycemia, Fig. 2, Immunostaining of the tumor and the adjacent adrenal gland. The tumor cells are immunorectively positive for P-45017 (a), P-450C21(b) and P-4501111(c)(x 50). Only faint immunoreactivity of P-45017(,is detectable in some cells in the zona reticularis of the adjacent adrenal gland (d) (x 33). CORTISOL IN VIRILIZING ADENOMA 287 methyrapone and LVP-CRF were all blunted, but was cortisol- and testosterone-producing but the not completely suppressed preoperatively. After activity of P-45011 was low. surgery, plasma ACTH response to provocative In the adrenal cortex attaching to the adenoma, stimuli
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