Feminizing Adrenocortical Tumor with Multiple Recurrences: A Case Report

Abstract

Feminizing adrenocortical tumors (FATs) are exceptionally rare primary adrenal neoplasms that cause high estrogen and low testosterone levels. They are most common in adult males, typically presenting with gynecomastia, hypogonadism, and weight loss. They are almost always malignant, with a poor prognosis and a high recurrence rate. We report a case of a 35-year-old man with an adrenal FAT with high estrogen (181 pg/mL) and low testosterone (37 ng/dL) who presented with gynecomastia, erectile dysfunction, subclinical Cushing syndrome, and pain localizing to different regions of the torso. There was no evidence of metastatic disease initially as seen by visualization of a well-marginated mass on computed tomography scan. Surgical resection of the FAT was performed, and the mass was confirmed to be a low-grade tumor. Clinical symptoms were resolved after surgery. Despite complete resection with negative margins, the patient subsequently had two separate local metastatic recurrences within a few years, treated with a combination of further surgery and medical intervention. This case highlights the unique features of an exceedingly rare adrenal tumor and stresses the importance of early detection and vigilant surveillance following resection due to high recurrence rates.

Keywords: Estrogen-secreting tumor; Feminizing adrenocortical tumor; Gynecomastia.

Fig. 1.

Axial (a) and coronal (b) contrast-enhanced CT scans upon diagnosis. There is a large, heterogeneous, predominantly soft tissue mass replacing the right adrenal and displacing the kidney and liver. The mass (blue arrows) is well-marginated with no obvious infiltration into surrounding tissues and no intra-venous extension. A few intratumoral streak-like linear densities are from neovascularity. Axial post-contrast T1-weighted MR image (c) depicts the heterogeneous nature of the well-marginated right adrenal tumor (blue arrow).

Fig. 2.

Pathological findings of the primary tumor specimen. a Gross tumor demonstrating predominantly yellow color with areas of hemorrhage and necrosis. b–c Light microscopy of the tumor showing adrenal cortical carcinoma with necrosis ×20 (b) and mitosis (c).

Fig. 3.

Axial CT scan 18 months after surgery revealing a growing perinephric nodule (blue arrow) consistent with recurrent/locally metastatic disease (the inferior tip of the right hepatic lobe is seen lateral to the right kidney).