Virilizing adrenal ganglioneuroma in a woman with subclinical Cushing syndrome

Dima L. Diab, Charles Faiman, Allan E. Siperstein, Ming Zhou, Robert S. Zimmerman

Research output: Contribution to journalArticlepeer-review

17 Scopus citations


Objective: To describe a patient with a virilizing adrenal ganglioneuroma and subclinical Cushing syndrome. Methods: Detailed clinical, laboratory, radiologic, and pathologic findings are presented, and the pertinent literature is reviewed. Results: A 56-year-old postmenopausal woman was referred for evaluation of a 3.6- by 3.0-cm right adrenal mass, which had been diagnosed during a work-up for hirsutism. A bilateral oophorectomy done 2 months before the presentation failed to correct the elevated testosterone levels. On examination, she had severe hirsutism on her face, chest, back, and extremities, as well as male pattern baldness and clitoromegaly. Biochemical evaluation showed elevated total and free serum testosterone levels of 319 ng/dL (reference range, 20 to 70) and 78 pg/mL (reference range, 1 to 9), respectively, values in the adult male range. The serum dehydroepiandrosterone sulfate level was 117 μg/dL (reference range, 10 to 152), and the urine free cortisol was 10.4 μg/24 h (reference range, <45). A laparoscopic adrenalectomy revealed a 5.0-cm adrenal ganglioneuroma containing nests of adrenocortical cells. On the first day postoperatively, the serum cortisol level was <1.0 μg/dL. At 1 month after adrenalectomy, the total and free testosterone levels had declined to 16 ng/dL and 3.1 pg/mL, respectively. At 2 months postoperatively, normal results of a cosyntropin stimulation test (basal and peak cortisol levels of 13.6 and 20.0 μg/dL, respectively) indicated recovery of the hypothalamic-pituitary-adrenal axis. Conclusion: To our knowledge, this is the first case report of a virilizing adrenal ganglioneuroma with this unique pathologic finding and concomitant subclinical Cushing syndrome.

Original languageEnglish (US)
Pages (from-to)584-587
Number of pages4
JournalEndocrine Practice
Issue number5
StatePublished - Jan 1 2008

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology


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