Journal of Yeungnam Medical Science

Yeungnam University College of Medicine, Yeungnam University Institute Medical Science (영남대 의대 학술지 편집위원회)
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Estrogen-secreting adrenocortical carcinoma

  • Jeong, You (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences) ;
  • Cho, Sung Chul (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences) ;
  • Cho, Hee Joon (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences) ;
  • Song, Ji Soo (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences) ;
  • Kong, Joon Seog (Department of Pathology, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences) ;
  • Park, Jong Wook (Department of Urology, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences) ;
  • Ku, Yun Hyi (Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences)
  • Received : 2018.06.19
  • Accepted : 2018.09.11
  • Published : 2019.01.31
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Abstract

Adrenocortical carcinoma is a rare type of endocrine malignancy with an annual incidence of approximately 1-2 cases per million. The majority of these tumors secrete cortisol, and a few secrete aldosterone or androgen. Estrogen-secreting adrenocortical carcinomas are extremely rare, irrespective of the secretion status of other adrenocortical hormones. Here, we report the case of a 53-year-old man with a cortisol and estrogen-secreting adrenocortical carcinoma. The patient presented with gynecomastia and abdominal discomfort. Radiological assessment revealed a tumor measuring $21{\times}15.3{\times}12cm$ localized to the retroperitoneum. A hormonal evaluation revealed increased levels of estradiol, dehydroepiandrosterone sulfate, and cortisol. The patient underwent a right adrenalectomy, and the pathological examination revealed an adrenocortical carcinoma with a Weiss' score of 6. After surgery, he was treated with adjuvant radiotherapy. Twenty-one months after treatment, the patient remains alive with no evidence of recurrence.

Keywords

References

  1. Wajchenberg BL, Albergaria Pereira MA, Medonca BB, Latronico AC, Campos Carneiro P, Alves VA, et al. Adrenocortical carcinoma: clinical and laboratory observations. Cancer 2000;88:711-36. https://doi.org/10.1002/(SICI)1097-0142(20000215)88:4<711::AID-CNCR1>3.0.CO;2-W
  2. Dackiw AP, Lee JE, Gagel RF, Evans DB. Adrenal cortical carcinoma. World J Surg 2001;25:914-26. https://doi.org/10.1007/s00268-001-0030-7
  3. Allolio B, Hahner S, Weismann D, Fassnacht M. Management of adrenocortical carcinoma. Clin Endocrinol (Oxf) 2004;60:273-87. https://doi.org/10.1046/j.1365-2265.2003.01881.x
  4. Libe R, Fratticci A, Bertherat J. Adrenocortical cancer: pathophysiology and clinical management. Endocr Relat Cancer 2007;14:13-28. https://doi.org/10.1677/erc.1.01130
  5. Moreno S, Guillermo M, Decoulx M, Dewailly D, Bresson R, Proye C. Feminizing adreno-cortical carcinomas in male adults. A dire prognosis. Three cases in a series of 801 adrenalectomies and review of the literature. Ann Endocrinol (Paris) 2006;67:32-8. https://doi.org/10.1016/S0003-4266(06)72537-9
  6. Zayed A, Stock JL, Liepman MK, Wollin M, Longcope C. Feminization as a result of both peripheral conversion of androgens and direct estrogen production from an adrenocortical carcinoma. J Endocrinol Invest 1994;17:275-8. https://doi.org/10.1007/BF03348975
  7. Abiven G, Coste J, Groussin L, Anract P, Tissier F, Legmann P, et al. 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;91:2650-5. https://doi.org/10.1210/jc.2005-2730
  8. Lanigan D, Choa RG, Evans J. A feminizing adrenocortical carcinoma presenting with gynaecomastia. Postgrad Med J 1993;69:481-3. https://doi.org/10.1136/pgmj.69.812.481
  9. Shen WT, Sturgeon C, Duh QY. From incidentaloma to adrenocortical carcinoma: the surgical management of adrenal tumors. J Surg Oncol 2005;89:186-92. https://doi.org/10.1002/jso.20180
  10. Advani A, Johnson SJ, Nicol MR, Papacleovoulou G, Evans DB, Vaikkakara S, et al. Adult-onset hypogonadotropic hypogonadism caused by aberrant expression of aromatase in an adrenocortical adenocarcinoma. Endocr J 2010;57:651-6. https://doi.org/10.1507/endocrj.K10E-046
  11. Gupta N, Bansal U, Mahajan N, Yadav MS. Abdominal wall metastasis in scar after open resection of an adrenocortical carcinoma. Clin Pract 2012;2:e9.
  12. Weiss LM, Medeiros LJ, Vickery AL. Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol 1989;13:202-6. https://doi.org/10.1097/00000478-198903000-00004
  13. Schulick RD, Brennan MF. Long-term survival after complete resection and repeat resection in patients with adrenocortical carcinoma. Ann Surg Oncol 1999;6:719-26. https://doi.org/10.1007/s10434-999-0719-7
  14. Bilimoria KY, Shen WT, Elaraj D, Bentrem DJ, Winchester DJ, Kebebew E, et al. Adrenocortical carcinoma in the United States: treatment utilization and prognostic factors. Cancer 2008;113:3130-6. https://doi.org/10.1002/cncr.23886
  15. Patalano A, Brancato V, Mantero F. Adrenocortical cancer treatment. Horm Res 2009;71(Suppl 1):99-104.
  16. Berruti A, Baudin E, Gelderblom H, Haak HR, Porpiglia F, Fassnacht M, et al. Adrenal cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2012;23(Suppl 7):vii131-8. https://doi.org/10.1093/annonc/mds231
  17. Lafemina J, Brennan MF. Adrenocortical carcinoma: past, present, and future. J Surg Oncol 2012;106:586-94. https://doi.org/10.1002/jso.23112
  18. Kirschner LS. The next generation of therapies for adrenocortical cancers. Trends Endocrinol Metab 2012;23:343-50. https://doi.org/10.1016/j.tem.2012.04.001
  19. Nigri G, Rosman AS, Petrucciani N, Fancellu A, Pisano M, Zorcolo L, et al. Meta-analysis of trials comparing laparoscopic transperitoneal and retroperitoneal adrenalectomy. Surgery 2013;153:111-9. https://doi.org/10.1016/j.surg.2012.05.042
  20. Fassnacht M, Hahner S, Polat B, Koschker AC, Kenn W, Flentje M, et al. Efficacy of adjuvant radiotherapy of the tumor bed on local recurrence of adrenocortical carcinoma. J Clin Endocrinol Metab 2006;91:4501-4. https://doi.org/10.1210/jc.2006-1007

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