胰腺实性假乳头状瘤临床病理特点及其诊治

Clinicopathologic features,diagnosis and treatment of solid-pseudopapillary tumor of the pancreas

目的探讨胰腺实性假乳头状瘤临床病理特点及其诊治。方法回顾性分析19例胰腺实性假乳头状瘤患者的临床病理资料。结果上腹疼痛14例,腹部肿块12例,影像学均能发现腹部肿块但不能明确诊断。肿块平均直经10.3 cm,包膜完整10例,包膜不完整9例,质地呈实性8例,呈囊实性11例。快速冰冻病理10例,6例获得确诊。病理特征为肿瘤细胞围绕纤细血管轴心形成特征性的假乳头状结构,细胞形态一致,异型不明显。4例证实有胰腺和血管浸润。11例免疫组化结果是:波形蛋白(Vimentin)、神经元特异性烯醇化酶(NSE)、α_1抗胰蛋白酶(α_1-AT)、突触素(Syn)、孕激素(PR)为阳性,上皮膜抗原(EMA)、雌激素(ER)、嗜铬颗粒素 A(CgA)、S-100蛋白为阴性。6例行胰十二指肠切除术,7例行肿瘤局部切除术,5例行胰体尾加脾切除术,1例胰体部肿瘤行胰腺阶段切除术。18例获随访,中位随访时间41(2～103)个月,肿瘤无复发,除1例出现营养不良外,余患者生存满意。结论胰腺实性假乳头状瘤为低度恶性肿瘤,确诊需依赖病理组织学检查。手术切除是治疗本病的良好方法。

Objective To study the clinicopathologic features, diagnosis and treatment of solid- pseudopapillary tumor of the pancreas. Methods A retrospective clinicopathologic analysis was made on 19 cases of solid-pseudopapillary tumor of the pancreas. Results The main complains included abdominal pain in 14 cases and abdominal mass in 12 cases. Imaging examination was not suggestive of tumor differentiation. The mean diameter of tumor was 10. 3 cm with complete capsule in 10 cases and incornplete capsule in 9 cases. Tumor was solid in 8cases and cysto- solid in 11 cases. Fast frozen pathology was definite in 6 out of 10 cases. Histologically, tumors were composed of papillary and microcystic solid structures, with uniformed population of cells without atypia. Pseudopapilla with fibro-vascular core was remarkable. The pancreas and blood vessels invasion were identified in 4 cases. Enzyme labeling examinations （ 11 cases） were positive for Vimention, NSE,a1 - AT,Syn,PR but negative for EMA,ER,CgA, S-100. 6 cases underwent Whipple＇s procedure, 7 cases underwent body and tail resection of the pancreas plus splenectomy, 5 cases underwent tumor enucleations, one case underwent body resection of the pancreas. 18 cases were followed up with a median period of 41 （2 - 103） months without tumor recurrence. Conclusions Solid - pseudopapillary tumor of the pancreas is a tumor of low- grade malignancy. The correct diagnosis depends on histopathology examination. Surgical resection is a therapy of cure for these patients.