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产生睾酮和皮质醇的卵巢附属类固醇细胞瘤:一例报告

Accessory ovarian steroid cell tumor producing testosterone and cortisol: A case report.

作者信息

Zang Li, Ye Mingxia, Yang Guoqing, Li Jinlong, Liu Mei, Du Jin, Gu Weijun, Jin Nan, Yang Lijuan, Ba Jianming, Dou Jingtao, Fan Wensheng, Mu Yiming, Meng Yuanguang, Lyu Zhaohui

机构信息

Department of Endocrinology Department of Gynecology Department of Pathology, Chinese PLA General Hospital, Beijing, China.

出版信息

Medicine (Baltimore). 2017 Sep;96(37):e7998. doi: 10.1097/MD.0000000000007998.

Abstract

RATIONALE

An accessory ovary is a rare structure containing normal ovarian tissue, which has a direct or ligamentous connection with a normal and eutopic ovary.

PATIENT CONCERNS

In the study, we reported a 46-year-old woman presented with secondary amenorrhea and virilization symptoms for 1 year.

DIAGNOSES

Endocrine evaluation revealed slightly elevated serum cortisol, extremely elevated 24-hour urinary-free cortisol and serum testosterone. Clinical assessment exhibited a large solid mass with heterogeneous enhancement in the left adnexauteri compounded with hypercortisolism and hyperandrogenemia. An accessory ovarian tumor attached to the infundibulum of the left fallopian tube was found, and a separate normal ovary was present on the same side.

INTERVENTIONS

The patient underwent a left adnexectomy.

OUTCOMES

During surgery, a 12 cm × 8 cm, gray-red, and well-circumscribed solid mass was be identified. The tumor had ligamentous attachment with the infundibulum of left fallopian tube. The sectioned surface was gray-brown, lobulated and did not exhibit either significant necrosis or hemorrhage. Pathological findings demonstrated that tumor cells had small round nuclei, mild atypia, no mitosis were arranged in a diffuse pattern of columns or nests separated by a rich vascular network and no crystals of Reinke were found. It was diagnosis ovarian steroid cell tumor (NOS) without malignant behavior by immunohistochemical staining. The patient was finally diagnosed as accessory ovarian steroid. The patient was discharged from the hospital on the seventeenth day after surgery. During postoperative follow-up, the first postoperative menstrual flow recovered and blood pressure regained 1 month after surgery. Furthermore, her Cushing syndrome regressed and hirsutism disappeared completely 4 months after surgery cell tumor.

LESSONS

It is vitally important to establish a final diagnosis according to the clinical manifestations and laboratory values in addition to imaging studies and laparoscopic examination of a rare coexistence of hyperandrogenemia and Cushing syndrome based on the accessory ovarian pathology.

摘要

理论依据

副卵巢是一种罕见的结构,包含正常卵巢组织,与正常的原位卵巢有直接或韧带连接。

患者情况

在本研究中,我们报告了一名46岁女性,出现继发性闭经和男性化症状1年。

诊断

内分泌评估显示血清皮质醇轻度升高,24小时尿游离皮质醇极度升高,血清睾酮升高。临床评估显示左附件区有一个大的实性肿块,强化不均匀,合并皮质醇增多症和高雄激素血症。发现一个附着于左侧输卵管漏斗部的副卵巢肿瘤,同侧还有一个单独的正常卵巢。

干预措施

患者接受了左侧附件切除术。

结果

手术中发现一个12厘米×8厘米、灰红色、边界清晰的实性肿块。肿瘤与左侧输卵管漏斗部有韧带相连。切面呈灰棕色,分叶状,无明显坏死或出血。病理结果显示肿瘤细胞有小圆形核,轻度异型性,无有丝分裂,呈弥漫性柱状或巢状排列,被丰富的血管网分隔,未发现Reinke晶体。免疫组化染色诊断为无恶性行为的卵巢类固醇细胞瘤(NOS)。患者最终被诊断为副卵巢类固醇细胞瘤。患者术后第17天出院。术后随访期间,术后第1个月月经恢复,血压恢复正常。此外,术后4个月库欣综合征消退,多毛症完全消失。

经验教训

除了影像学检查和腹腔镜检查外,根据临床表现、实验室检查结果以及基于副卵巢病理的罕见高雄激素血症和库欣综合征并存情况来做出最终诊断至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/dfe6/5604648/f64a9abfada4/medi-96-e7998-g002.jpg

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