Benign Feminizing Adrenal Tumor in an Adult Male

摘要

Background: Feminizing adrenal tumors are rare and generally malignant tumors usually seen in male adults and children. We report the case of a benign feminizing adrenal tumor in a male patient. To our knowledge, only 2 other cases of benign, estrogen-only–secreting adrenal tumors have been reported.Case Report: A 44-year-old male with a history of hypertension presented to his primary care physician with chest tenderness, fatigue, and erectile dysfunction. Hormonal workup and imaging identified an estrogen-only–secreting adrenal adenoma. The adenoma was removed via laparoscopic adrenalectomy, and the patient had a normal postoperative course. Pathologic findings were an adrenal cortical adenoma with a Weiss score of 0 and a Ki-67 score of 0%. At 6-month follow-up, the patient's symptoms had significantly improved, and his previously abnormal sex hormone levels were within normal limits.Conclusion: Given the ambiguity in distinguishing between malignant and benign feminizing adrenal tumors, we suggest that radiologic (via Hounsfield units), clinical (via trending hormone levels), and histopathologic (via Weiss and Ki-67 scores) findings are sufficient to confirm the benign nature of this commonly malignant tumor. Keywords: Adrenocortical adenoma , estrogen-secreting tumor , feminizing adrenal tumor INTRODUCTIONFeminizing adrenal tumors are extremely rare adrenal neoplasms. Chentli et al identified 50 cases reported between 1979 and 2014.~(1) Forty-one patients were males, 33 were adults, and all but 1 case were malignant. In a study conducted by Moreno et al, feminizing adrenal tumors accounted for 0.37% of 801 adrenalectomies between 1970 and 2003, and all of them were malignant.~(2) The most common initial presenting symptoms in males are gynecomastia, erectile dysfunction, and fatigue.~(1) Gynecomastia is usually bilateral and painful, and the size varies significantly among patients. This breast development is related to the high levels of estrogen being secreted from the tumor via increased activity of aromatase and the peripheral conversion of androstenedione to estrone.~(3) Estrogen production can be solitary but is more commonly accompanied by the production of other adrenocortical hormones.The prognosis of patients with malignant tumors varies in the literature, with Chentli et al reporting a survival of 4 years, Moreno et al reporting a survival of 7 years, and Lanigan et al reporting a median survival of 2.9 months.~(1,2,4) All of the tumors in these 3 studies showed aggressive pathology and were considered carcinomas according to the consensus guidelines (requiring a Weiss score >2).~(5)We report a case of benign feminizing adrenal tumor in a male patient. To our knowledge, only 2 other cases of benign estrogen-only–secreting adrenal tumors have been reported.~(6,7)CASE REPORTA 44-year-old male with a history of hypertension presented to his primary care physician in January 2017 for chest tenderness, predominantly on the left, for the prior several months. He had also noticed an increase in the size of both breasts. The patient was an active individual with a regimen of weightlifting and basketball 3 times per week, but he reported weight gain (principally central adiposity) and feeling fatigued. Additionally, he had had erectile dysfunction symptoms since 2016 for which he had seen a urologist. Mammogram revealed benign bilateral gynecomastia that was managed conservatively.In January 2018, he presented again to his primary care physician with spontaneous improvement of the previously reported chest pain but continued symptoms of fatigue and erectile dysfunction. He was referred to an endocrinologist who determined that his breasts were still enlarged but overall had not changed since his previous mammogram. Hormonal workup showed an elevated estrone level of 192 pg/mL (male reference, <68 pg/mL), elevated estradiol of 44 pg/mL (male reference, 50%, with calculation of absolute and relative washout consistent with an adenoma. Adrenal intermediaries checked to evaluate for malignancy were also normal, as was a workup for hypercortisolism (Cushing syndrome) (Tables 2 and ?and33). Table?1. Pituitary Hormonal Workup Test Result Reference Range Prolactin, ng/mL10.32.0-18.0Luteinizing hormone, mIU/mL3.11.5-9.3Follicle-stimulating hormone, mIU/mL0.81.6-8.0Thyroid-stimulating hormone, mIU/L2.080.4-4.5Thyroxine, ng/dL1.30.8-1.8Plasma renin, ng/mL/h0.750.6-4.3Open in a separate window Open in a separate windowFigure?1. Computed tomography scan of abdomen and pelvis with contrast shows interval development of a 2.8-cm left adrenal nodule with imaging characteristics consistent with an adenoma (arrow).