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

Estrogen-secreting adrenocortical carcinoma

Affiliations
  • 1Department of Internal Medicine, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea. kyh@kirams.re.kr
  • 2Department of Pathology, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea.
  • 3Department of Urology, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea.

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

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