子宫性索样肿瘤临床病理学特征

Clinicopathologic Features of Uterine Tumors Resembling Ovarian Sex Cord Tumors

目的:探讨子宫性索样肿瘤的临床病理学特征。方法:对3例子宫性索样肿瘤的临床资料、病理形态学和免疫组织化学染色进行观察;采用免疫组织化学En Vision法检测肿瘤细胞中calretinin、alpha-inhibin、Melan A、CD99、CK7、ER、PR、CD10、SMA、EMA、desmin、Calponin、h-caldesmon和HMB45的表达情况;对3例患者均进行了随访观察。结果:均为女性患者,年龄37～48岁,临床主要症状是阴道不规则出血;肿瘤细胞排列成小巢状、梁索状、丛状或实性分布,呈明显的性索或上皮样分化;肿瘤细胞无明显异型性,大小较为一致,圆形、卵圆形、短梭形或多边形。胞质丰富,嗜伊红色,呈上皮样分化,或泡沫状胞浆,类似卵巢性索间质肿瘤中的黄素化细胞。胞核较小,罕见病理性核分裂象。3例肿瘤细胞均表达calretinin和alpha-inhibin;1例表达MelanA,1例表达Melan A和CD99;肿瘤细胞灶性表达CK7、EMA、ER和PR,均不表达CDl0、SMA、desmin、Calponin、h-caldesmon和HMB45。3例随访结果目前均无复发。结论:子宫性索样肿瘤是一种非常少见的子宫肿瘤,组织病理学特征结合性索间质免疫标记可提高诊断的正确率;生物学行为应被视为潜在恶性,临床处理上应依据患者年龄以及对生育的要求综合考虑,长期随访是必须的。

Objective： To explore the clinicopathologic features of uterine tumor resembling ovarian sex cord tumor （ UTROSCT ）. Methods： Clinical data, light microscopy and immunohistochemistry results from 3 UTROSCT cases were analyzed, lmmunohistochemical staining was performed to detect calretinin, alpha-inhibin, Melan A, CD99, CK7, ER, PR, CD10, SMA, EMA, desmin, Calponin, h-caldesmon and HMB45 using the En Vision method. Results： Cords, nests, trabecular ribbons, and solid architecture were seen. Tumor cells were small with bland nuclei and abundant eosinophilic or foamy cytoplasm, rare mitotic activity and necrosis. Immunohistochemically, tumor cells were positive for calretinin and alpha-inhibin, negative for CD10, SMA, desmin, Calponin, h-caldesmon and HMB45, and partially positive for Melan A, CD99, CK7, EMA, ER and PR. Follow-up data were available for all 3 patients and none had local recurrence after treatment. Conclusion： UTROSCT is a rare neoplasm that should be considered a tumor of low-grade malignant potential. Combined morphological and immunophenotypical analyses can be used to confirm the diagnosis. Clinical procedures should be performed based on the age of the patient and in consideration of the patient＇s plans for future children. Long-term follow-up is necessary.