睾酮水平低下的肾上腺嗜酸性细胞瘤一例报告并文献复习

Hypo-androgen adrenocortical oncocytoma： 1 case report and literature review

目的探讨合并睾酮水平低下的肾上腺嗜酸性细胞瘤的临床特点、诊治及预后。方法分析2016年3月12日收治的1例伴睾酮水平低下的肾上腺嗜酸性细胞瘤患者的临床资料。男，22岁。血压151／88mmHg（1mmHg=0．133kPa）。表现为双侧乳房发育明显，双侧睾丸质软、体积小，阴茎短小，阴毛稀疏。内分泌检查结果：雌二醇666pg／ml（正常值20—47pg／m1），泌乳素19．08ng／ml（正常值2．64～13．13ng／m1），睾酮0．18ng／ml（正常值1．75～7．81ng／m1），卵泡刺激素（FSH）〈0．2U／L（正常值1．27—19．26U／L）。精液常规检查未见精子。染色体核型为46，XY。肾上腺激素系列实验室检查：血、尿儿茶酚胺，血皮质醇，促肾上腺皮质激素，醛固酮，血管紧张素等未见异常。CT检查：左侧。肾上腺区可见团块状软组织密度影，直径7em，不均质强化，动脉期可见多支小血管穿行，肿块内血流丰富。临床诊断为左肾上腺嗜铬细胞瘤。全麻下行腹腔镜下左肾上腺肿物切除术。术中见左肾上腺肿物位于左肾上极，有独立的动脉供应。结果病理检查肿瘤重60g，直径7om，切面呈棕色，肿瘤细胞排列成实性巢索状或腺泡状，胞质内有较多嗜酸性颗粒，细胞核圆形，核仁位于中央。可有多形性和细胞簇状聚集。肿瘤被厚的纤维包膜包裹，主要成分是嗜酸性粒细胞肉芽组织，未见坏死、有丝分裂及血管侵犯。免疫组化染色检查：CD56、syn蛋白表达阳性。病理诊断：左肾上腺嗜酸性细胞瘤。术后4个月复查，患者血压恢复正常（118／75mmHg），开始出现胡须，乳房缩小，睾酮3．90ng／ml，雌二醇21pg／ml。无临床症状及影像学复发征象。随访16个月，血睾酮4．68ng／ml，血清雌二醇33pg／ml，肿瘤局部无复发及转移。精液常规化验未见精子。结论睾酮水平低下和高雌二醇的功能性肾上腺嗜酸性细胞瘤临床发病率低，病理成分主要是嗜酸性粒细胞肉芽组织。临床上功能性肾上腺嗜酸细胞瘤少见，术前不易诊断，需结合患者临床表现及影像学检查、肾上腺生化检查等给予定位及定性，微创手术是有效治疗手段，术后需密切随访。

Objective To analysis the clinical features, diagnosis, treatment and prognosis of adrenal eosinophilic tumor with low testosterone levels. Methods The clinical data of a 22 years old male patient with adrenal eosinophilic tumor and low testosterone levels was analyzed. Blood pressure was 151/88 mmHg. The patient got bilateral gynecomastia. His bilateral testicular was soft and became smaller, with short penisr. Endocrine examination results showed： Estradio] 666 pg/m], Prolactin 19.08 ng/m], Testosterone 0. 18 ng/ml,follicle stimulating hormone 〈 0. 2 U/L. The CT showed the mass density of soft tissue in the left adrenal region with diameter 7 era, which was inhomogeneous and enhanced. There were many small vessels enhanced in the CT arterial phase, and the blood flow in the tumor was abundant. Clinical diagnosis of left adrenal tumor was pheochromoeytoma. The patient underwent laparoscopic left adrenal tumor resection. The left adrenal gland was located in the superior pole of the left kidney, and there was an independent supply of the artery. Results Pathological result showed the tumor weigh was 60 g, 7 cm in diameter and brown in section. The tumor cells were arranged in solid nests or acini, with more eosinophilic granules in cytoplasm. The nuclei was round and the nucleoli was located in the center, had clusters of pleomorphic and clustered cells. The tumor was wrapped in a thick fibrous envelope, mainly consisted of eosinophils, granulation tissue. There was no necrosis,mitosis, and vascular invasion. Immunohistochemical staining showed that the expression of CD56 and syn protein was positive. Pathological diagnosis was left adrenal eosinophilic tumor. After 4 months, the blood testosterone levels rose to 3.90 ng/ml, the blood pressure returned to normal （ 118/75 mmhg）. The estradiol （ 21 pg/ml） was significantly inhibited. The patient began to appear beards and breasts became smaller. There were no signs of clinical or imaging recurrence. After 16 months follow-up, serum testosterone was 4. 68 ng/ml and serum estrogen levels dropped to 33 pg/ml. Semen routine showed no sperm. Conclusions The clinical morbidity of functional adrenocortical oncocytoma with low testosterone levels and high estradiol levels is low. The pathological components are mainly eosinophilic granulation tissue. The adrenocortical oncocytoma are rare and preoperative diagnosis is difficult. Clinical manifestation, imaging examination and adrenal biochemistry examination should be considered to determine the localization and qualitative of tumor. Minimally invasive surgery is an effective treatment. The close follow-up after operation is essential.