类似卵巢性索肿瘤的子宫肿瘤的临床病理特征和免疫表型

Clinicopathological features and immunophenotype of uterine tumor resembling ovarian sex cord tumor

目的 探讨类似卵巢性索肿瘤的子宫肿瘤(uterine tumor resembling ovarian sex cord tumor, UTROSCT)的临床病理特征和免疫表型以及预后。方法 收集9例UTROSCT患者的临床病理资料及随访资料,进行光镜观察、免疫组化染色及相关文献复习,提炼和总结诊断和鉴别诊断的要点。结果 6例为绝经前患者,3例为绝经后患者,年龄27~69岁。8例表现为子宫异常出血,1例为体检时发现。肿瘤均为单发,直径2.5~8.0 cm。6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、管状或条索样排列。细胞无明显异型性,无明确坏死及核分裂象。所有病例均表达一种以上的性索标记(α-inhibin, WT-1,CD56,Calretinin),ER、PR、CK和Vimentin几乎均为阳性表达,3例表达肌源性标记(SMA,Desmin)。2例不同程度表达神经内分泌标记物(Syn、NSE、CgA)。本研究收集的9例UTROSCT患者有1例子宫全切术后26个月复发,1例患者死于胃癌,其余7例均无病生存。结论 UTROSCT是一种非常罕见的低度恶性妇科肿瘤,临床症状缺乏特异性,目前主要依靠组织病理、免疫表型和分子病理分型进行诊断及鉴别诊断,分子病理检测有助于判断患者的预后。

Objective To explore the clinicopathological characteristics, immunohistochemical expression and prognosis of uterine tumor resembling ovarian sex cord tumor(UTROSCT). Methods The data of 9 patients with UTROSCT were analyzed retrospectively. Histopathologic analysis was performed on tissue sections after HE staining, and immunohistochemical staining. The literatures were reviewed and the diagnosis and differential diagnosis summarized. Results Six patients were premenopausal women and three patients were postmenopausal women aged 27-69 years. Eight patients presented with abnormal uterine bleeding and one without any symptoms. Six tumors were located in the uterine muscle wall and three cases were located under the mucosa with the diameter from 2.5 to 8.0 cm. Tumors were completely composed of epithelioid cells or sex cord-like cells, with few or without mitotic figures, and necrosis was absent. By immunohistochemistry, all cases expressed more than one sex cord markers(α-inhibin, WT-1, CD56, or Calretinin). Vimentin, ER, PR and CK were almost all positive. Three cases expressed myogenic markers including smooth muscle actin(SMA) and Desmin. Two cases expressed neuroendocrine markers(Syn, NSE, CgA). The follow-up data showed that one died of gastric cancer, one patient relapsed after having accepted a total hysterectomy 26 months later, and others lived without recurrence or metastases. Conclusion UTROSCT is a rare low-grade gynecological tumor. Due to lack of specific clinical symptoms, the diagnosis of UTROSCT mainly relies on morphological characteristics, immunophenotype and molecular pathology;moreover, molecular pathological subtyping is helpful to judge the prognosis of patients.