胰腺实性-假乳头状瘤的临床病理学及免疫组织化学观察

SOLID-PSEUDOPAPILLARY NEOPLASM OF THE PANCREAS: A CLINICOPATHOLOGIC AND IMMUNOHISTOCHEMICAL OBSERVATION OF 15 CASES

目的观察胰腺实性-假乳头状瘤（SPN）的临床病理学特点，探讨其分化表型及组织起源。方法应用苏木精-伊红染色法观察15例SPN的组织学特征。采用免疫组化PV6000法检测15例SPN中多种蛋白的表达。结果15例病人均为女性，年龄18～51岁，平均27．5岁。5例位于胰头，5例位于胰体，4例位于胰尾，1例位于腹膜后。肿瘤直径3．0～17．3cm，平均9．3cm。14例有完整包膜。CT检查发现15例肿瘤由不同比例实性与囊性成分混合组成，呈不均匀密度的软组织包块影，14例周边有高密度纤维包膜，1例边界不清。随访3月～10年14例均未见肿瘤复发，1例有肝脏转移者术后随访13月未见复发。SPN肿瘤细胞大小形态较一致，核圆形或卵圆形，核异性不明显，核分裂象罕见。肿瘤细胞围绕纤维血管复层排列形成假乳头突起，常见肿瘤细胞的退变、出血、泡沫细胞和胆固醇裂隙。1例见肝脏内肿瘤组织转移。免疫组织化学染色显示，15例α-1-抗胰蛋白酶（＋），13例波形蛋白（＋），10例神经元特异性烯醇酶（＋），5例嗜铬素A（＋），8例孕激素受体（＋），4例肌动蛋白（＋）；雌激素受体、突触素、上皮膜抗原、胰高血糖素、胃泌素、胰岛素均为阴性。结论SPN多发生于年轻女性，具有良性临床病程和较高的手术治愈率。SPN可能起源于胰腺多潜能于细胞，具有导管、腺泡和内分泌细胞的分化表型。

Objective To observe the clinicopathologic characteristics of the solid-pseudopapillary neoplasm of the pancreas （SPN） and to probe into its differentiation patterns and histogenesis. Methods The histological characteristics of 15 cases of SPN were observed by HE method. Immunohistochemical PV 6000 was used to test some protein expression in 15 cases of SPN. Results Clinical and imageological findings were： all the 15 cases were females, mean age （range） was 27.5 years （18-51）. Of the 15 tumors, 5 located in the head of the pancreas, 5 in the body, 4 in the tail and 1 in retroperitoneum. The mean （range） diameter of the tumors was 9.3 cm （3.0-17.3）, in which, 14 with intact capsule. CT revealed variable combinations of solid and cystic areas in all 15 cases, in which, 14 had a higher-density fibrous capsule, no clear margin was seen in one. Follow-up of from 3 months to 10 years, no recurrence was found, another one with hepatic metastasis 13 months later. Histology showed a uniform population of tumor cells. The nuclei were oval or round without atypia, and mitotic activity was absent. Psudopapillary structure with a fibrovascular core was remarkable. Hemorrhage, foam cells and cholesterol crystals were often found. Immunohistochemically, 15 cases had positive for α-1-AT, 13 had vimentin, 10 had NSE, 5 had CgA, 8 had PR and 4 had Actin; but ER, Syn, EMA, Glucagon, Gastrin and Insulin were negative for all the cases. Conclusion SPN is most commonly seen in young female with a benign clinical course and a higher surgically-cure rate, which develops from primitive pancreatic cells,with the potentiality of developing into ducts, acinus, and endocrine cells.