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目的探讨胎儿骶尾部先天性未成熟畸胎瘤的临床病理特征、诊断及鉴别诊断。方法分析1例胎儿骶尾部先天性未成熟畸胎瘤的临床资料、尸检病理结果,并结合文献复习。结果 B超示胎儿脊柱骶尾部囊、实性混合回声。尸检发现胎儿骶尾部可见5.5 cm×5 cm×4 cm大小肿物,切面呈囊实性,囊内含淡黄色液体。光镜下肿瘤由多个胚层组成,可见角化的鳞状上皮、纤毛上皮、软骨、平滑肌、肠管、内胚窦样结构、神经胶质细胞和原始神经管。免疫组化:小管样结构细胞nestin、vimentin和CD99(+),GFAP、NF和Neu N(-);周围神经胶质细胞GFAP和NF(+),Neu N(-);上皮细胞CK(+),内胚窦样结构S-100(+);Ki-67阳性率为20%,p53(-)。结论胎儿骶尾部先天性未成熟畸胎瘤罕见,确诊依赖于B超、组织学形态和免疫组化结果,需要与成熟性畸胎瘤和原始神经外胚层肿瘤鉴别。
Objective To investigate the clinical and pathological features, diagnosis and differential diagnosis of fetal sacrococcygeal immature teratoma. Methods One case of fetal sacrococcygeal immature teratoma clinical data, autopsy pathology results, combined with the literature review. B ultrasound showed fetal sacrococcygeal sac, solid mixed echo. Autopsy found that fetal sacrococcygeal visible 5.5 cm × 5 cm × 4 cm size of the tumor, the section was cystic, cysts containing a light yellow liquid. Under light microscopy, the tumor consists of multiple germ layers, showing keratinized squamous epithelium, ciliated epithelium, cartilage, smooth muscle, intestine, endoderm-like structure, glial cells and primitive neural tube. Immunohistochemistry: Nestin, vimentin and CD99 (+), GFAP, NF and Neu N (-) were detected in tubular epithelial cells. GFAP and NF (+) and Neu N ), Endodermal sinusoid S-100 (+); Ki-67 positive rate of 20%, p53 (-). Conclusions Fetal sacrococcygeal innate immature teratoma is rare and the diagnosis depends on the results of ultrasonography, histological examination and immunohistochemistry. It needs to be differentiated from mature teratoma and primitive neuroectodermal tumor.