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一例罕见的以良性和恶性特征独特组合为特征的大型卵巢类固醇细胞瘤-其他特定型,导致 21 岁叙利亚女性出现月经稀少和多毛症:病例报告。

A large ovarian steroid cell tumor-not otherwise specified with a unique combination of benign and malignant features as a challenging cause of oligomenorrhea and hirsutism in a 21-year-old Syrian female: a case report.

机构信息

Department of Pathology, Cancer Research Center, Faculty of Medicine, Tishreen University, Lattakia, Syria.

Faculty of Medicine, Tishreen University, Lattakia, Syria.

出版信息

BMC Womens Health. 2021 Mar 4;21(1):95. doi: 10.1186/s12905-021-01244-1.

Abstract

BACKGROUND

Ovarian steroid cell tumors represent a rare category of sex cord-stromal tumors that constitute less than 0.1% of all ovarian tumors. These neoplasms are classified into three main subtypes according to the cell of origin: Leidyg cell tumors, stromal luteomas, and steroid cell tumors not otherwise specified (SCTs-NOS). The latter subtype is defined as a neoplasm of an uncertain lineage that mostly affects middle-aged women, whereas it's rare in younger ages.

CASE PRESENTATION

We report a case of a 21-year-old virgin female who presented to our hospital with complaints of mild abdominal pain, hirsutism, and oligomenorrhea for more than a year. Before her current admission, the patient had attended an external gynecologic clinic where she had been prescribed oral contraceptives to regulate her periods. Nevertheless, on presentation to our institution, physical examination revealed abdominal tenderness with a palpable pelvic mass and mild hirsutism in the thigh. Ultrasonography demonstrated a large left ovarian mass measuring 154 × 104 mm, and compressing the uterus. Therefore, a unilateral salpingo-oophorectomy was performed, and interestingly, pathologic examination of the large aforementioned mass alongside with immunohistochemical correlation revealed the diagnosis of a large ovarian steroid cell tumor-not otherwise specified with a unique combination of benign and malignant features.

CONCLUSIONS

Although ovarian steroid cell tumors represent a rare category, they must be considered in the differential diagnosis for mild virilization symptoms in young females due to the importance of early diagnosis and management. In this manuscript, we aimed to present the first case report from Syria that highlights the crucial role of detailed morphological examination for challenging cases despite the difficulties in differential diagnosis, and the absence of ancillary techniques. Furthermore, we managed to discuss a brief review of diagnostic methods, histological characteristics, and treatment recommendations.

摘要

背景

卵巢类固醇细胞瘤是一种罕见的性索-间质肿瘤,占所有卵巢肿瘤的比例不足 0.1%。这些肿瘤根据起源细胞的不同分为三个主要亚型:莱迪希细胞瘤、间质黄体瘤和未特指的类固醇细胞瘤(SCT-NOS)。后者被定义为一种不确定来源的肿瘤,主要影响中年女性,而在年轻女性中则较为罕见。

病例介绍

我们报告了一例 21 岁未婚女性的病例,她因腹痛、多毛症和月经稀发超过一年而就诊于我院。在本次入院前,该患者曾在一家外妇科诊所就诊,当时她被开处口服避孕药来调整月经周期。然而,在我院就诊时,体格检查发现腹部有压痛,可触及盆腔肿块,大腿部有轻度多毛症。超声检查显示左侧卵巢有一个 154×104mm 的巨大肿块,压迫子宫。因此,行单侧输卵管卵巢切除术,有趣的是,对上述大型肿块的病理检查以及免疫组化相关性研究提示诊断为一种未特指的大型卵巢类固醇细胞瘤,具有独特的良性和恶性特征的组合。

结论

尽管卵巢类固醇细胞瘤较为罕见,但由于早期诊断和管理的重要性,对于年轻女性出现轻度男性化症状时,必须考虑将其作为鉴别诊断的一种可能性。在本研究中,我们旨在报告来自叙利亚的首例病例报告,强调了在存在鉴别诊断困难和缺乏辅助技术的情况下,详细的形态学检查对于疑难病例的重要性。此外,我们还成功地讨论了诊断方法、组织学特征和治疗建议的简要综述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c7dc/7934245/7de65f57d931/12905_2021_1244_Fig1_HTML.jpg

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