类似卵巢性索肿瘤的子宫肿瘤8例临床病理特征分析
Clinicopathologic study of 8 cases of uterine tumors resembling ovarian sex cord tumors

目的:探讨类似卵巢性索肿瘤的子宫肿瘤(UTROSCT)的临床病理特征。方法:收集8例UTROSCT患者的临床病理资料及随访资料,进行光镜观察及免疫组化染色。结果:8例均为绝经前患者,年龄27～52岁。肿瘤均为单发,直径2.1～14.0 cm。5例表现为子宫出血,2例表现为腹痛,1例在体检时发现。5例位于子宫肌壁间,3例位于黏膜下。镜下肿瘤均由上皮样或性索样细胞构成,呈小梁状、巢片状或性索样排列。细胞无明显异型性,无明确坏死及核分裂。肿瘤细胞同时表达性索标记(α-inhibin,WT-1,CD56,CR)、肌源性标记(SMA,desmin)及上皮性标记(CK、CAM5.2、EMA),ER与PR均为阳性表达,但H-caldesmon常为阴性表达。4例不同程度表达神经内分泌标记物(SyN、NSE、CgA)。1例子宫全切术后30个月复发,1例子宫及双附件切除术后26个月后死于肺癌,其余6例均无病生存。结论:UTROSCT确诊主要依靠特征性的形态及免疫表型,多呈良性临床经过,但可复发或转移,患者需长期随访。
Aim: To describe the clinicopathologic features of uterine tumors resembling ovarian sex cord tumors(UTROSCT).Methods: Clinicopathological data and follow-up data of 8 patients with UTROSCT were retrospectively reviewed. Histopathologic analysis was performed on sections after HE-staining and immunohistochemical staining.Results: All patients were premenopausal women, with age ranged from 27-52 years. Five patients presented with abnormal uterine bleeding and two with abdominal pain, while another one without any symptoms, the tumor was found during physical examination. Five tumors were located in the uterine muscle wall and three cases were located under the mucosa with the diameter from 2.1 cm to 14.0 cm. Microscopic examination revealed that tumors were completely composed of sex cord-like cells or epithelioid cells, with few or without mitosis, and necrosis was not seen. By immunhistochemistry, all cases expressed more than 2 sex cord markers(α-inhibin, WT-1, CD56 or CR), epithelial markers(CK, CAM5.2, EMA)and myogenic markers including smooth muscle actin(SMA)and desmin. However, H-caldesmon was negative in all cases. Interestingly, four cases showed neuroendocrine markers(NSE, SyN, CgA)expression. The follow-up data suggested that one patient died of lung cancer 26 months after complete hysterectomy and bilateral adnexectomy, and one case relapsed after having accepted a total hysterectomy 30 months later, while the other 6 patients had no recurrence or metastases.Conclusion: The diagnosis of UTROSCT mainly relies on morphological character and immunophenotype, though the tumor generally behaves in a benign clinical course, it can relapse or metastasis, therefore all patient need long-term follow-up