PSAT022 A Rare Case of Androgen Secreting Adrenal Cortical Carcinoma in a Young Woman

Abstract

Abstract Introduction/Background Adrenal cortical carcinoma (ACC) is a rare disease occurring in 1 to 2 per million per year. (1) The pure androgen secreting carcinomas are even more rare and occur in only 6% of the cases of ACC. (2) These tumors have an occurrence of age which is usually bimodal either in the first or fifth decade of life. (3) Herein, we report an unusual case of malignant pure androgen secreting adrenal tumor in a woman in her second decade. Clinical Case A 19 year old female presented to the ED with abdominal pain and was found to have incidental adrenal tumor and therefore referred to endocrinology. She complained of hirsutism in the upper lip, chest and abdomen, weight gain and amenorrhea for 18 months?. Hormonal work-up revealed elevated DHEAS 755 mcg/dL (normal 27-240), serum testosterone level 306 ng/dL (normal 10-75), whereas cortisol and metanephrines levels were within normal limits. A diagnostic MRI showed a large left 10.5cm adrenal tumor and patient subsequently underwent laparoscopic left total adrenalectomy. Final pathological report revealed low grade adrenocortical carcinoma confined to the adrenal cortex. Oncology was consulted and the patient was started on adjuvant cytotoxic chemotherapy with cisplatin and etoposide followed by radiation, and mitotane therapy. Postoperatively at two months testosterone and DHEAS levels were normalized. Conclusion This case illustrates how ACC may present with atypical symptoms of hirsutism, amenorrhea, and hyperandrogenic state. Hypersecretion of both androgen and cortisol is more common occurring approximately 46% of patients with ACC but the presence of hypersecretion of isolated androgens in adrenal tumors has been associated with an increased likelihood of malignancy. (3) Physicians of different specialties should have a high degree of suspicion for adrenal involvement in the evaluation of patients with hirsutism and hyperandrogenism. Work-up should include laboratory testing along with radiographical images to help establish the diagnosis. References Angelousi A, Kassi E, Kaltsas GA. Adrenocortical Carcinoma. [Updated 2018 Oct 2]. In: Feingold KR, Anawalt B, Boyce A, et al., editors. Endotext [Internet]. South Dartmouth (MA): MDText.com, Inc.; 2000-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK278924/ Abiven G, Coste J, Groussin L, Anract P, Tissier F, Legmann P, Dousset B, Bertagna X, Bertherat J. Clinical and biological features in the prognosis of adrenocortical cancer: poor outcome of cortisol-secreting tumors in a series of 202 consecutive patients. J Clin Endocrinol Metab. 2006 Jul;91(7): 2650-5. doi: 10.1210/jc.2005-2730. Epub 2006 May 2. PMID: 16670169. Cavlan D, Bharwani N, Grossman A. Androgen- and estrogen-secreting adrenal cancers. Semin Oncol. 2010 Dec;37(6): 638-48. doi: 10.1053/j.seminoncol.2010.10.016. PMID: 21167382. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.