Rich Joseph M, Duddalwar Vinay, Cheng Phillip M, Aron Manju, Daneshmand Siamak
MD/PhD Program, USC-Caltech MD/PhD Program, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA.
Department of Radiology, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA.
Case Rep Oncol. 2023 Sep 26;16(1):1033-1040. doi: 10.1159/000533835. eCollection 2023 Jan-Dec.
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.
女性化肾上腺皮质肿瘤(FATs)是极为罕见的原发性肾上腺肿瘤,可导致雌激素水平升高和睾酮水平降低。它们在成年男性中最为常见,通常表现为男性乳房发育、性腺功能减退和体重减轻。它们几乎总是恶性的,预后较差且复发率高。我们报告一例35岁男性患有肾上腺FAT,其雌激素水平高(181 pg/mL)、睾酮水平低(37 ng/dL),表现为男性乳房发育、勃起功能障碍、亚临床库欣综合征以及躯干不同部位疼痛。计算机断层扫描显示边界清晰的肿块,最初未发现转移病灶迹象。对FAT进行了手术切除,肿块被证实为低级别肿瘤。术后临床症状得到缓解。尽管手术切缘阴性且完整切除,但患者在几年内随后出现了两次独立的局部转移复发,接受了进一步手术和药物干预相结合的治疗。该病例突出了这种极其罕见的肾上腺肿瘤的独特特征,并强调了由于复发率高,早期检测和切除后密切监测的重要性。