Yeungnam Univ J Med.  2019 Jan;36(1):54-58. 10.12701/yujm.2019.00017.

Estrogen-secreting adrenocortical carcinoma

• Jeong Y
• Cho SC
• Cho HJ
• Song JS
• Kong JS
• Park JW
• Ku YH

• KMID: 2434100
• DOI: http://doi.org/10.12701/yujm.2019.00017

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 21×15.3×12 cm 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.

Keyword

Adrenal gland neoplasm; Adrenocortical carcinoma, Estrogen; Gynecomastia

MeSH Terms

Adrenal Gland Neoplasms
Adrenalectomy
Adrenocortical Carcinoma*
Aldosterone
Dehydroepiandrosterone Sulfate
Estradiol
Gynecomastia
Humans
Hydrocortisone
Incidence
Male
Middle Aged
Radiotherapy, Adjuvant
Recurrence
Aldosterone
Dehydroepiandrosterone Sulfate
Estradiol
Hydrocortisone

Figure

• 

  Fig. 1. Contrast-enhanced abdominal computed tomography reveals a right adrenal mass. Axial (A) and coronal (B) images of a well-defined, heterogeneously enhancing mass measuring 18×11×18 cm between the liver and right kidney.

• 

  Fig. 2. Gross appearance of the resected adrenal tumor. The mass was large, solitary, and circumscribed tumor (21×15.3×12 cm). The cut section is yellowish-tan in color, with a variegated appearance. Many areas of necrosis and hemorrhage are visible.

• 

  Fig. 3. Microscopic tumor findings. (A) The tumor exhibits a diffuse solid growth pattern with necrosis on the right side (hematoxylin and eosin [H&E] stain, ×40). (B) The tumor cells are round-to-oval in shape with high-nuclear-grade nucleoli. Mitoses are frequently observed (H&E stain, ×400).

• 

  Fig. 4. Immunohistochemical staining of the tumor. The Ki-67 index of the tumor cells is 20% (×400).

Reference

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.

2. Dackiw AP, Lee JE, Gagel RF, Evans DB. Adrenal cortical carcinoma. World J Surg. 2001; 25:914–26.

3. Allolio B, Hahner S, Weismann D, Fassnacht M. Management of adrenocortical carcinoma. Clin Endocrinol (Oxf). 2004; 60:273–87.

4. Libe R, Fratticci A, Bertherat J. Adrenocortical cancer: pathophysiology and clinical management. Endocr Relat Cancer. 2007; 14:13–28.

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.

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.

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.

8. Lanigan D, Choa RG, Evans J. A feminizing adrenocortical carcinoma presenting with gynaecomastia. Postgrad Med J. 1993; 69:481–3.

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.

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.

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.

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.

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.

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.

17. Lafemina J, Brennan MF. Adrenocortical carcinoma: past, present, and future. J Surg Oncol. 2012; 106:586–94.

18. Kirschner LS. The next generation of therapies for adrenocortical cancers. Trends Endocrinol Metab. 2012; 23:343–50.

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.

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.