Arafat Hasan, Khaldy Marah, Abu Munshar Ahmad, Zughayyer Amer
Cancer Care Center Augusta Victoria Hospital Jerusalem Palestine.
Clin Case Rep. 2023 Oct 18;11(10):e7915. doi: 10.1002/ccr3.7915. eCollection 2023 Oct.
Ovarian steroid cell tumors are a rare subtype of sex-cord stromal cell tumors. Overall, these tumors make <0.1% of all ovarian tumors. These neoplasms can be divided according to the cell of origin into stromal luteomas, Leydig cell tumors, and steroid cell tumors not otherwise specified. These tumors can be benign, malignant, or borderline, with variable presentation. We report a case of 24-year-old virgin female who was referred to our hospital after being diagnosed with steroid cell tumor-not otherwise specified. Prior to her admission, the patient had been treated unsuccessfully with oral contraceptive pills due to male-pattern facial hair growth, abdominal cramps, and irregular menstrual cycle. Lack of improvement warranted further investigations. Hormonal studies showed an elevated total testosterone, dehydroepiandrosterone sulfate, and morning fasting cortisol. Ultrasonography and computed tomography confirmed the presence of a large pelvic mass with mixed solid and cystic component. Therefore, unilateral salpingo-oophorectomy was performed. Pathological and immunohistochemical examination suggested the presence of a large ovarian steroid cell tumor-not otherwise specified with malignant behavior. The patient did not receive adjuvant therapy and developed metastatic disease. She received four cycles of BEP protocol with no improvement, so she was referred to our center to continue oncological management. Case revision confirmed the presence of steroid cell tumor, but of a different subtype: Leydig cell. She received six cycles of carboplatin-paclitaxel, but her assessment showed disease progression. We report this case with review of literature regarding the appropriate approach to these rare tumors. Although rare, ovarian steroid cell tumors should be included in the differential diagnosis of virilization in young females, especially those refractory to hormonal therapy. In our study, we aimed to present the first reported Palestinian case, which highlights the importance of detailed morphological examination in addition to the difficulties encountered to reach a proper diagnosis. We also provided a review of the existing literature regarding chemotherapeutic lines used in such cases and the response to each.
卵巢类固醇细胞瘤是性索间质细胞瘤的一种罕见亚型。总体而言，这些肿瘤在所有卵巢肿瘤中占比不到0.1%。这些肿瘤可根据起源细胞分为间质黄体瘤、莱迪希细胞瘤和未另行分类的类固醇细胞瘤。这些肿瘤可以是良性、恶性或交界性的，表现各异。我们报告一例24岁处女女性病例，该患者在被诊断为未另行分类的类固醇细胞瘤后转诊至我院。入院前，患者因男性化面部毛发增多、腹部绞痛和月经周期不规律接受口服避孕药治疗，但未成功。病情无改善促使进一步检查。激素研究显示总睾酮、硫酸脱氢表雄酮和晨空腹皮质醇升高。超声检查和计算机断层扫描证实盆腔有一个大肿块，具有实性和囊性混合成分。因此，实施了单侧输卵管卵巢切除术。病理和免疫组化检查提示存在一个未另行分类的大的具有恶性行为的卵巢类固醇细胞瘤。患者未接受辅助治疗并发生了转移。她接受了四个周期的BEP方案治疗但无改善，因此被转诊至我们中心继续肿瘤管理。病例复查证实存在类固醇细胞瘤，但为不同亚型：莱迪希细胞瘤。她接受了六个周期的卡铂 - 紫杉醇治疗，但评估显示疾病进展。我们报告此病例并回顾关于这些罕见肿瘤的适当处理方法的文献。尽管罕见，但卵巢类固醇细胞瘤应纳入年轻女性男性化的鉴别诊断中，尤其是那些对激素治疗难治的患者。在我们的研究中，我们旨在呈现首例报道的巴勒斯坦病例，该病例强调了详细形态学检查的重要性以及在做出正确诊断时遇到的困难。我们还提供了关于此类病例使用的化疗方案及其反应的现有文献综述。
