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目的探讨淋巴管内乳头状血管内皮瘤(PILA)的临床病理学特点、诊断与鉴别诊断、起源及预后。方法对1例PILA的临床表现、组织形态、组织化学及免疫组化特点进行分析,并复习相关文献。结果患儿女性,1岁11个月。出生时即发现右前臂红色肿物。镜下见肿瘤组织位于真皮,由不规则的扩张的薄壁管腔构成,类似海绵状淋巴管瘤,部分管腔内见玻璃样轴心的乳头状皱褶,管壁与乳头衬覆鞋钉状内皮细胞。免疫组化:管壁与乳头衬覆鞋钉状内皮细胞CD34、VEGFR-3、CD31、FⅧ因子均呈(+),海绵状管腔内皮细胞及乳头衬覆鞋钉状内皮细胞D2-40(-);组织化学:VG染色显示乳头玻璃样轴心呈红色,Masson染色显示乳头玻璃样轴心呈绿色。结论淋巴管内乳头状血管内皮细胞瘤罕见,成人与儿童均可发生,确诊主要依靠组织病理学,组织化学及免疫组化染色对诊断有帮助。其可能来源于淋巴管,预后大多良好。
Objective To investigate the clinicopathological features, diagnosis, differential diagnosis, origin and prognosis of lymphatic vessel papillary hemangioendothelioma (PILA). Methods The clinical manifestations, histomorphology, histochemistry and immunohistochemical features of one case of PILA were analyzed and the related literatures were reviewed. Results Children with children, 1 year and 11 months. Right forearm red tumor found at birth. Microscope, the tumor tissue located in the dermis, composed of irregular expansion of thin-walled lumens, similar to cavernous lymphangioma, part of the lumen see the glass-like axis of the papillary folds, wall and nipple lined spike Like endothelial cells. Immunohistochemistry showed that the number of CD34, VEGFR-3, CD31 and FⅧ were (+), spongiform endothelial cells and papillary endothelial cells D2-40 (P < -); histochemistry: VG staining showed a nipple glass axis was red, Masson staining showed nipple glass axis was green. Conclusions Lymphatic vessel papillary hemangioendothelioma is rare in both adults and children. The diagnosis depends mainly on histopathology, histochemistry and immunohistochemical staining. It may come from lymphatic vessels, the prognosis is mostly good.