SUN-376 Isolated DHEA-S Production by an Adrenal Neoplasm: Clinical, Biochemical, and Pathologic Findings

Abstract

Background. Pure androgen secreting adrenal neoplasms are rare and present with virilization, including hirsutism (face, chest, and back), male pattern baldness, acne, deepening of the voice and clitoral enlargement. Serum concentrations of testosterone, dehydroepiandrosterone (DHEA), dehydroepiandrosterone sulfate (DHEA-S), and androstenedione are elevated (1,2). To our knowledge, an adrenal neoplasm with isolated DHEA-S production and otherwise normal androgens has not been reported. We report the clinical, biochemical, and pathologic features of a pure DHEA-S secreting adrenal neoplasm occurring in a post-menopausal woman. Clinical case. A 57 y/o post-menopausal Indian female presented with a 4 cm right adrenal mass found on CT imaging for colitis. She denied any dietary supplements containing DHEA or DHEA-S. She reported generalized hair thinning. She denied deepening of voice, excessive hair growth, or acne. Physical examination confirmed these reports; there was no clitoromegaly. Biochemical testing revealed an elevated (mean of 2 measurements) serum DHEA-S concentration of 706 mcg/dL (nl = 26-200). Other serum androgen concentrations were normal and included free testosterone = 2.3 pg/mL (nl = 0.6-3.8 ); DHEA = 2.41 ng/mL(nl = 0.60-5.73); and androstenedione = 0.529 ng/mL (nl = 0.130-0.820 ). Other circulating adrenal hormone concentrations including cortisol, aldosterone, estrone, estradiol, and metanephrines were normal. ACTH was not suppressed, and FSH and LH were appropriately elevated for the post-menopausal state. CT imaging revealed a heterogeneous 4 cm right adrenal mass that contained several areas measuring <10 HU. The mass was excised by uncomplicated laparoscopic adrenalectomy. The tumor size was 5.0 x 4.5 x 2.5 cm. Pathology revealed an adrenal cortical neoplasm that was likely benign, since the mitotic rate was low. At 24 h post adrenalectomy, serum DHEA-S concentration had decreased to 274 mcg/dL. Three weeks post adrenalectomy, DHEA-S was normal at 91 mcg/dL. Other serum androgen concentrations decreased slightly post-operatively. Summary: The rapid decrease in the elevated serum DHEA-S concentration following adrenalectomy confirmed DHEA-S production by the pathologically benign 5.0 cm adrenal adenoma. Biochemical testing was negative for production of other adrenal hormones, and the only clinical feature was nonspecific generalized hair thinning. Conclusion: We describe the clinical, biochemical, and pathologic findings of an exceedingly rare adrenal neoplasm with isolated DHEA-S production. References. (1)F. Cordera, et al., Surgery, 134 (6): 874-880, 2003. (2)Moreno, S., et al, Surgery, 136(6): 1192-1198, 2004.