Abstract：
Sex cord tumor with annular tubules (SCTAT) is a rare ovarian tumor. It belongs to sex cord and stromal tumor of the ovary and represents less than 1% of cases. It includes two forms: the first one associated with Peuz-Jeghers syndrome and the second sporadic. We report 4 cases of SCTAT collected at the department of pathology of Salah Azaiez Institute of Tunis over the 12 last years. The age ranged from 10 to 32 years. Symptoms were non specific except for one case revealed by precocious puberty. One patient had Peutz-Jeghers syndrome associated. Tumors were unilateral. Gross findings showed often a solid tumor with yellow cut surface. Their size ranged from 0.5cm to 28cm. Their morphological features were characteristic. Immunohistochemistry showed that tumor cells expressed inhibin and claretinin. The treatment was surgical, often conservative. The diagnosis of malignancy wasn\'t focused on histological features, but on tumor extension, clinical course, and presence of metastases. Evolution was often favorable. We also performed a systematic review of the literature that identified 166 cases. Features of these cases were studied. We also compared these features between sporadic and syndromic forms and between benign and malignant forms. In conclusion, SCTAT is a rare tumor, usually benign. Its diagnosis is based on histological examination. There is a malignant potential especially in sporadic forms, estimated at 20%. Treatment is most often conservative, based on oophorectomy.
摘要：
性索环形小管肿瘤（SCTAT）是一种罕见的卵巢肿瘤。它属于性索和卵巢间质瘤，占不到1％的病例。它包括两种形式：第一种与Peuz-Jeghers综合征有关，第二种是零星的。我们报告了过去12年在突尼斯SalahAzaiez研究所病理科收集的4例SCTAT病例。年龄从10到32岁不等。除一例因性早熟而出现症状外，症状无特异性。一名患者患有Peutz-Jeghers综合征。肿瘤是单方面的。总体发现通常显示实体瘤，切面为黄色。它们的大小范围从0.5cm到28cm。它们的形态特征是特征性的。免疫组化显示肿瘤细胞表达抑制素和克拉汀。治疗是手术，往往是保守的。恶性肿瘤的诊断不集中在组织学特征上，但是在肿瘤扩展方面，临床课程，和转移的存在。进化往往是有利的。我们还对发现166例病例的文献进行了系统回顾。研究了这些病例的特点。我们还比较了散发性和综合症形式以及良性和恶性形式之间的这些特征。总之,SCTAT是一种罕见的肿瘤,通常是良性的。其诊断基于组织学检查。有一种恶性潜能，特别是在零星形式中，估计为20%。治疗通常是保守的，基于卵巢切除术。
