儿童胰腺囊实性乳头状上皮性肿瘤9例并文献复习

目的探讨儿童胰腺囊实性乳头状上皮性肿瘤（sPTP）的发病率、病理特征和诊治特点。方法收集术后明确诊断的9例儿童SPTP，对组织标本进行常规HE、PAS染色及多种指标免疫组化染色，结合文献分析儿童SPrP临床表现、影像学检查、病理特点、手术处理方法及随访情况。结果9例SPTP，平均10．3岁，占同期儿童胰腺实体瘤的29．O％；临床多表现为腹痛（5例，55．56％）、腹部肿块（3例，33．33％）、黄疸（1例，11．11％）等症状；B超和cT显示：胰腺占位病变，边界清，呈囊实性；病理检查：肉眼观呈暗红色，包膜完整，剖面囊实性病变交替，瘤组织有片状出血、坏死和囊性变。免疫组化检查：d。一抗胰蛋白酶阳性率100％，NSE66．7％、S-10033．3％，PAS染色均呈阳性。随访4个月至10年，均存活，未见局部复发和远处转移。结论SPTP多发生于青春期前女孩，是低度恶性或交界性肿瘤，具有良性临床病程和潜在恶性的生物学行为，手术治愈率高，预后良好。

Solid-pseudopapillary tumor of the pancreas in children:report of nine cases and review of the literature

Objective To estimate the incidence,clinicopathological characteristics and therapeutic features in children with solid pseudopapillary tumors of the pancreas(SPTP).Methods Nine eases of children SPTP after diagnosis were collected in our hospital.Routine HE,PAS staining and immunohiatochemical staining of multiple indicators were analyzed,combined with literature analysis of clinical manifestations,imaging,pathological features,surgical treatment and follow-up case.Results The average age in nine cases of SPTP was 10.3 years.The SPTP patients in children in our hospital accounted for 29.0% of pancreas solid tumors.Many clinical manifestations were abdominal pain(5 cases,55.56%),abdominal mass(3 cases,33.33%),jaundice(1 cases,11.11%),and other symptoms.B-ultrasonography and CT showed pancreatic lesions,clear boundary,was solid and cystic,and some have a little calcification.Pathological features:tumor limitations,capsule integrity,and cross-section alternating solid and cystic lesions,tumors organizations sheet hemorrhage,necrosis and cystic change.Immunohistochemically,the positive rates were 100% for α1-AT,66.7% for NSE,33.3% for S-100 and 100% for PAS.The patients were followed-up for 4 months to 10 years and were alive postoperatively,but no local recurrence and distant metastasis.Histological examination showed solid with cystic areas and papillary protrusions.Conclusion SPTP should be the second most common pancreatic tumor in children.Girls were more frequently affected.The overall prognosis following surgical resection was good.