腹膜后具有血管周上皮样细胞分化的肿瘤1例报道及文献复习

目的探讨具有血管周上皮样细胞分化的肿瘤临床病理特点。方法对1例腹膜后具有血管周上皮样细胞分化的肿瘤的临床病理组织学及免疫表型进行观察并结合文献复习。结果患者体检发现盆腔包块,术中见腹膜后偏左相当于髂内外动脉分叉下方有一10×8×6cm^3大小不规则包块。镜下见上皮样肿瘤细胞环绕血管生长排列,大部分细胞呈圆形或多边形,也可见少数梭形细胞,胞浆呈透明至细颗粒状,核位于中央,呈圆形或椭圆形,染色质正常,核仁小,细胞有明显异型性,免疫组化标记HMB45（＋）,SMA（＋）,CK（-）,S-100（-）。结论腹膜后PEComas非常罕见,其有独特的细胞组织学特征和明确的免疫组化表型,但其良恶性标准及预后仍待进一步探讨。

Retroperitoneal with perivascular epithelioid cell differentiation of the tumor a case report and literature review

Objective To study the clinicopathological featuresof tumoursshowing perivascular epithelioid cell differentiation（ PEComas）. Methods Observe the clinicopathological and immunohistochemical features of a patient with PEComa in retroperitoneum and review literatures. Results Physical examination was found in patients with pelvic mass. Intraoperative peritoneal see below left internal and external iliac artery bi-furcation is equivalent to the bottom of a 10 × 8 × 6 cm^3 size of the irregular mass. Microscopically,epithelioid tumor cells arranged around the blood vessel growth,most cells were round or polygonal,can also be found a small number of spindle cells,clear to finely granular cytoplasm,nuclear is located in the central,round or oval,chromatin normal,small nucleoli,cell marked atypia. Immunohistochemical markers HMB45 （ ＋ ） ,SMA （ ＋ ） ,CK （ -） ,S-100 （ -）. Conclusion Retroperitoneal PEComas is very rare. It has unique tissue characteristics and specific immunohistochemical phenotype. Its standards and prognosis of benign or malignant remains to be further explored.