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November 13, 1987, Vol 258, No. 18 >
ARTICLE | November 13, 1987

Testosterone-Secreting Adrenal Adenoma in a Peripubertal Girl FREE

Themis C. Kamilaris, MD, PhD; C. Rowan DeBold, MD, PhD; Kostas J. Manolas, MD, PhD; Anestis Hoursanidis, MD; Sotirios Panageas, MD; John Yiannatos, MD, PhD
[+] Author Affiliations

Reprint requests to AA-4206, Medical Center North, Vanderbilt University School of Medicine, Nashville, TN 37232 (Dr Kamilaris).


JAMA. 1987;258(18):2558-2561. doi:10.1001/jama.1987.03400180092034
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References

A 15-year-old girl who presented with primary amenorrhea and virilization had an adrenocortical adenoma that secreted predominantly testosterone. To our knowledge, she is the first peripubertal and second youngest patient with a testosterone-secreting adrenal tumor described. Serum dehydroepiandrosterone sulfate and urinary 17-ketosteroid and 17-hydroxycorticosteroid levels were normal. A tumor was located by a computed tomographic (CT) scan and by uptake of 6-β-[75Se] selenomethylnorcholesterol. Microscopic examination of the tumor showed typical features of an adrenocortical adenoma with no histologic features characteristic of Leydig cells. Postoperatively, her hirsutism regressed, she rapidly went through puberty, and regular monthly menstruation started four months later. Finding the source of testosterone in a virilized patient can be difficult. Eleven of the 14 previously described patients with testosterone-secreting adrenal tumors initially underwent misdirected surgery on the ovaries. Review of these cases revealed that results of hormone stimulation and suppression tests are unreliable and that these tumors are usually large. Therefore, CT scanning of the adrenal glands is recommended in all patients suspected of having a testosterone-secreting tumor. If the adrenal glands on CT scan are normal, then surgery directed at the ovaries can be undertaken. Adrenal and ovarian vein catheterization is rarely necessary.

(JAMA 1987;258:2558-2561)

REFERENCES

1 +
Freeman DA:  Steroid hormone-producing tumors in man . Endocr Rev 1986;;7:208-220.
2 +
Werk EE, Sholiton LJ, Kalejs L:  Testosterone-secreting adrenal adenoma under gonadotropin control . N Engl J Med 1973;;289:767-770.
3 +
Givens JR, Andersen RN, Wiser WL, et al:  A gonadotropin-responsive adrenocortical adenoma . J Clin Endocrinol Metab 1974;;38:126-133.
4 +
DeLange WE, Pratt JJ, Doorenbos H:  A gonadotrophin responsive testosterone producing adrenocortical adenoma and high gonadotrophin levels in an elderly woman . Clin Endocrinol 1980;; 12:21-28.
5 +
Louros NC, Batrinos ML, Carcatzoulis S:  Individual 17-KS excretion in a case of arrhenoblastoma and its response to ACTH and hCG stimulation and to dexamethasone inhibition . J Clin Endocrinol Metab 1966;;26:645-650.
6 +
Gallagher TF, Spencer H, Bradlow L, et al:  Steroid production and metabolism in metastatic arrhenoblastoma . J Clin Endocrinol Metab 1962;; 22:970-977.
7 +
Tucci JR, Zah W, Kalderon AE:  Endocrine studies in an arrhenoblastoma responsive to dexamethasone, ACTH, and human chorionic gonadotropin . Am J Med 1973;;55:687-694.
8 +
Vasiloff J, Chideckel EW, Boyd CB, et al:  Testosterone-secreting adrenal adenoma containing crystalloids characteristic of Leydig cells . Am J Med 1985;;79:772-776.
9 +
Burr IM, Sullivan J, Graham T, et al:  A testosterone-secreting tumour of the adrenal producing virilisation in a female infant . Lancet 1973;;2:643-644.
10 +
Drekter IJ, Heisler A, Scism GR, et al:  The determination of urinary steroids: I. The preparation of pigment-free extracts and a simplified procedure for the estimation of total 17-ketosteroids . J Clin Endocrinol Metab 1952;;12:55-65.
11 +
Silber RH, Porter CC:  The determination of 17,21-dihydroxy-20-ketosteroids in urine and plasma . J Biol Chem 1954;;210:923-932.
12 +
Page DL, DeLellis RA, Hough AJ Jr: Tumors of the Adrenal . Washington, DC, Armed Forces Institute of Pathology, 1986;, pp 81-114.
13 +
Takahashi H, Yoshizaki K, Kato H, et al:  A gonadotrophin-responsive virilizing adrenal tumour identified as a mixed ganglioneuroma and adrenocortical adenoma . Acta Endocrinol 1978;; 89:701-709.
14 +
Mack E, Sarto GE, Crummy AB, et al:  Virilizing adrenal ganglioneuroma . JAMA 1978;;239: 2273-2274.
15 +
Kable WT, Yussman MA:  Testosterone-secreting adrenal adenoma . Fertil Steril 1979;;32:610-611.
16 +
Kelly TR, Mayors DJ, Boutsicaris PS:  Adrenal adenoma: Isolated testosterone secretion . Am Surg 1982;;48:604-606.
17 +
Smith HC, Posen S, Clifton-Bligh P, et al:  A testosterone-secreting adrenal cortical adenoma . Aust NZ J Med 1978;;8:171-175.
18 +
Trost BN, Koenig MP, Zimmermann A, et al:  Virilization of a post-menopausal woman by a testosterone-secreting Leydig cell type adrenal adenoma . Acta Endocrinol 1981;;98:274-282.
19 +
Dolinar R, Burch WM:  Testosterone-producing adrenal adenoma in a woman with normal urinary 17-ketosteroid levels . JAMA 1983;;250:2504-2505.
20 +
Aguirre P, Scully RE:  Testosterone-secreting adrenal ganglioneuroma containing Leydig cells . Am J Surg Pathol 1983;;7:699-705.
21 +
Larson BA, Vanderlaan WP, Judd HL, et al:  A testosterone-producing adrenal cortical adenoma in an elderly woman . J Clin Endocrinol Metab 1976;;42:882-887.
22 +
Spaulding SW, Masuda T, Osawa Y:  Increased 17β-hydroxysteroid dehydrogenase activity in a masculinizing adrenal adenoma in a patient with isolated testosterone overproduction . J Clin Endocrinol Metab 1980;;50:537-540.
23 +
Bayliss RIS, Edwards OM, Starer F:  Complications of adrenal venography . Br J Radiol 1970;; 43:531-533.
24 +
Printz RA, Brooks MH, Churchill R, et al:  Incidental asymptomatic adrenal masses detected by computed tomographic scanning . JAMA 1982;; 248:701-704.
25 +
Copeland PM:  The incidentally discovered adrenal mass . Ann Intern Med 1983;;98:940-945.
26 +
Bertagna C, Orth DN:  Clinical and laboratory findings and results of therapy in 58 patients with adrenocortical tumors admitted to a single medical center (1951 to 1978) . Am J Med 1981;;71:855-874.
27 +
Sternberg WH, Segaloff A, Gaskill CJ:  Influence of chorionic gonadotrophin on human ovarian hilus cells (Leydig-like cells) . J Clin Endocrinol Metab 1953;;13:139-153.
28 +
Mostofi FK, Price EB Jr: Tumors of the Male Genital System . Washington, DC, Armed Forces Institute of Pathology, 1973;, pp 85-114.
29 +
Suwa S, Maesaka H, Matsui I:  Serum LH and FSH responses to synthetic LH-RH in normal infants, children, and patients with Turner's syndrome . Pediatrics 1974;;54:470-475.
30 +
Reiter EO, Root AW, Duckett GE:  The response of pituitary gonadotropes to a constant infusion of luteinizing hormone-releasing hormone (LHRH) in normal prepubertal and pubertal children and in children with abnormalities of sexual development . J Clin Endocrinol Metab 1976;;43: 400-411.

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1 +
Freeman DA:  Steroid hormone-producing tumors in man . Endocr Rev 1986;;7:208-220.
2 +
Werk EE, Sholiton LJ, Kalejs L:  Testosterone-secreting adrenal adenoma under gonadotropin control . N Engl J Med 1973;;289:767-770.
3 +
Givens JR, Andersen RN, Wiser WL, et al:  A gonadotropin-responsive adrenocortical adenoma . J Clin Endocrinol Metab 1974;;38:126-133.
4 +
DeLange WE, Pratt JJ, Doorenbos H:  A gonadotrophin responsive testosterone producing adrenocortical adenoma and high gonadotrophin levels in an elderly woman . Clin Endocrinol 1980;; 12:21-28.
5 +
Louros NC, Batrinos ML, Carcatzoulis S:  Individual 17-KS excretion in a case of arrhenoblastoma and its response to ACTH and hCG stimulation and to dexamethasone inhibition . J Clin Endocrinol Metab 1966;;26:645-650.
6 +
Gallagher TF, Spencer H, Bradlow L, et al:  Steroid production and metabolism in metastatic arrhenoblastoma . J Clin Endocrinol Metab 1962;; 22:970-977.
7 +
Tucci JR, Zah W, Kalderon AE:  Endocrine studies in an arrhenoblastoma responsive to dexamethasone, ACTH, and human chorionic gonadotropin . Am J Med 1973;;55:687-694.
8 +
Vasiloff J, Chideckel EW, Boyd CB, et al:  Testosterone-secreting adrenal adenoma containing crystalloids characteristic of Leydig cells . Am J Med 1985;;79:772-776.
9 +
Burr IM, Sullivan J, Graham T, et al:  A testosterone-secreting tumour of the adrenal producing virilisation in a female infant . Lancet 1973;;2:643-644.
10 +
Drekter IJ, Heisler A, Scism GR, et al:  The determination of urinary steroids: I. The preparation of pigment-free extracts and a simplified procedure for the estimation of total 17-ketosteroids . J Clin Endocrinol Metab 1952;;12:55-65.
11 +
Silber RH, Porter CC:  The determination of 17,21-dihydroxy-20-ketosteroids in urine and plasma . J Biol Chem 1954;;210:923-932.
12 +
Page DL, DeLellis RA, Hough AJ Jr: Tumors of the Adrenal . Washington, DC, Armed Forces Institute of Pathology, 1986;, pp 81-114.
13 +
Takahashi H, Yoshizaki K, Kato H, et al:  A gonadotrophin-responsive virilizing adrenal tumour identified as a mixed ganglioneuroma and adrenocortical adenoma . Acta Endocrinol 1978;; 89:701-709.
14 +
Mack E, Sarto GE, Crummy AB, et al:  Virilizing adrenal ganglioneuroma . JAMA 1978;;239: 2273-2274.
15 +
Kable WT, Yussman MA:  Testosterone-secreting adrenal adenoma . Fertil Steril 1979;;32:610-611.
16 +
Kelly TR, Mayors DJ, Boutsicaris PS:  Adrenal adenoma: Isolated testosterone secretion . Am Surg 1982;;48:604-606.
17 +
Smith HC, Posen S, Clifton-Bligh P, et al:  A testosterone-secreting adrenal cortical adenoma . Aust NZ J Med 1978;;8:171-175.
18 +
Trost BN, Koenig MP, Zimmermann A, et al:  Virilization of a post-menopausal woman by a testosterone-secreting Leydig cell type adrenal adenoma . Acta Endocrinol 1981;;98:274-282.
19 +
Dolinar R, Burch WM:  Testosterone-producing adrenal adenoma in a woman with normal urinary 17-ketosteroid levels . JAMA 1983;;250:2504-2505.
20 +
Aguirre P, Scully RE:  Testosterone-secreting adrenal ganglioneuroma containing Leydig cells . Am J Surg Pathol 1983;;7:699-705.
21 +
Larson BA, Vanderlaan WP, Judd HL, et al:  A testosterone-producing adrenal cortical adenoma in an elderly woman . J Clin Endocrinol Metab 1976;;42:882-887.
22 +
Spaulding SW, Masuda T, Osawa Y:  Increased 17β-hydroxysteroid dehydrogenase activity in a masculinizing adrenal adenoma in a patient with isolated testosterone overproduction . J Clin Endocrinol Metab 1980;;50:537-540.
23 +
Bayliss RIS, Edwards OM, Starer F:  Complications of adrenal venography . Br J Radiol 1970;; 43:531-533.
24 +
Printz RA, Brooks MH, Churchill R, et al:  Incidental asymptomatic adrenal masses detected by computed tomographic scanning . JAMA 1982;; 248:701-704.
25 +
Copeland PM:  The incidentally discovered adrenal mass . Ann Intern Med 1983;;98:940-945.
26 +
Bertagna C, Orth DN:  Clinical and laboratory findings and results of therapy in 58 patients with adrenocortical tumors admitted to a single medical center (1951 to 1978) . Am J Med 1981;;71:855-874.
27 +
Sternberg WH, Segaloff A, Gaskill CJ:  Influence of chorionic gonadotrophin on human ovarian hilus cells (Leydig-like cells) . J Clin Endocrinol Metab 1953;;13:139-153.
28 +
Mostofi FK, Price EB Jr: Tumors of the Male Genital System . Washington, DC, Armed Forces Institute of Pathology, 1973;, pp 85-114.
29 +
Suwa S, Maesaka H, Matsui I:  Serum LH and FSH responses to synthetic LH-RH in normal infants, children, and patients with Turner's syndrome . Pediatrics 1974;;54:470-475.
30 +
Reiter EO, Root AW, Duckett GE:  The response of pituitary gonadotropes to a constant infusion of luteinizing hormone-releasing hormone (LHRH) in normal prepubertal and pubertal children and in children with abnormalities of sexual development . J Clin Endocrinol Metab 1976;;43: 400-411.

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