骶尾部卵黄囊瘤9例临床病理分析

Sacrococcygeal yolk sac tumor: clinicopathologic analyses of 9 cases

目的探讨骶尾部卵黄囊瘤（YST）的临床病理学特征及诊断与鉴别诊断。方法对9例骶尾部卵黄囊瘤进行临床病理分析及免疫组化染色,同时复习相关文献。结果 8例为女童,1例为男童,发病年龄16个月～44个月。以臀部或骶尾部包块为主要临床表现。组织病理学检查均可见到典型的内胚窦结构以及腺样、乳头状和疏松网状结构。免疫组化显示9例AFP（＋）,其中5例呈灶状（＋）,4例胞质呈弥漫性强（＋）;2例PLAP灶状（＋）;8例CK呈强（＋）,1例（-）;9例CD30、HCG、EMA、CD15和CD117均（-）。5例确诊后及时进行化疗,3例未化疗者短期内复发死亡,1例失访。结论骶尾部卵黄囊瘤多见于女婴,恶性度高,化疗敏感,诊断主要依靠组织形态与免疫组化染色。

Objective To investigate the clinicopathological features, diagnosis,and differential diagnosis of sacrococcygeal yolk sac tumor. Methods The samples of nine patients with sacral yolk sac tumor were processed with clinical pathological analysis and immunohistochemical staining,and the references about this clinical data were reviewed.Results Eight cases were girls,and one case was boys,whose ages were from 16 months to 44 months. The mass in buttocks or sacral region was the main clinical manifestations. For histopathological examination,typical endodermal sinus structures,and adenoid,papillary and loose network structures were found. Immunohistochemistry showed the expression of AFP with different levels in nine cases,of which five cases were spotty expression,and four cases were strongly positive diffuse cytoplasm. The spotty expression of PLAP was showed in two cases. CK was strongly positive in eight cases,and one case was negative. CD30,HCG,EMA,CD15,and CD117 were not expressed in nine cases. Five cases were promptly performed with chemotherapy after they were diagnosed. While three cases were relapsed and transformed without chemotherapy in the result of death. One case lost follow-up. Conclusion Sacrococcygeal yolk sac tumor with highgrade malignancy,is sensitive to chemotherapy,and more commonly occurs in girls. Diagnosis of sacrococcygeal yolk sac tumor is mainly depended on morphology and immunohistochemical staining.