淋巴管内乳头状血管内皮瘤临床病理观察

Papillary intralymphatic angioendothelioma: a clinicopathologic analysis

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 study the elinieopathologic features, differential diagnosis and origin of papillary intralymphatie angioendothelioma. Methods One case of papillary intralymphatic angioendothelioma was reported and the related literatures were reviewed. Results The patient was a 1-year and 11-month girl presenting a red tumor in the right forearm at birth. Microscopically, the main features showed a subcutaneous vascular tumor composed of irregular vascular channels forming papillae in some areas. These papillae had hyalinized cores and were lined by atypical cuboidal endothelial cells with focal hobnailed appearance. VG, Masson and PAS were all positive for hyaline cores. Immunohistochemically, the tumor cells showed strongly positive for CD34, VEGFR-3, CD31, and factor-Ⅷ, but D2-40 was also positive for the endothelial cells of spongy lumina and negative for papillae. Conclusion Papillary intralymphatic angioendothehoma is a rare type of tumor, and diagnosis depends on pathological examinations with assistance of histochemical and immunohistochemical stainings. The tumor may be origined from lymphangioma and the prognosis is good.