Clinicopathological study of solid and pseudopapillary tumor of pancreas: Emphasis on magnetic resonance imaging findings

AIM: To report the clinicopathological features and magnetic resonance imaging (MRI) findings of solid and pseudopapillary tumor (SPT) of pancreas.METHODS: From 1981 to 2005, 26 surgically treated cases of SPT were retrospectively reviewed. MRI findings of the latest 11 consecutive SPT cases were investigated.RESULTS: There were 25 women and one man having SPT (median age: 23 year) with a median tumor size of 7.5 cm. Among them, nine patients developed solid pseudopapillary carcinoma. During the median follow-up period of 66 mo, the 5-year survival rate of the 26 SPT patients was 96.2%. Three MRI features were proposed including Type 1 image, displaying SPT with completely solid part. All SPT patients with type 1 image were detected incidentally. Type 2 image displays of SPT with solid mass hemorrhage and type 3 image with massive hemorrhage. All the eight SPT patients with type 2 and 3 images suffered abdominal pain due to hemorrhage from SPT.CONCLUSION: SPT had a favorable survival rate irrespective of surgical procedures, malignancy, and MRI findings, however, MRI could reliably correlate with its clinicopathological features.

4羟基丁酸尿症6例临床及实验室研究

目的探讨4羟基丁酸尿症患儿临床特征及实验室诊断方法。方法2012年6月至2013年6月天津市儿童医院收治的6例4羟基丁酸尿症患儿，应用头磁共振成像（MRI）、尿气相色谱一质谱（GC／MS）半定量检测及ALDH5A1基因突变分析多层面分析。结果6例均存在精神运动发育落后。3例存在癫痫发作。5例起病年龄〈1岁。4例头MRI示双侧对称性苍白球病变。6例尿GC／MS半定量检测示尿4羟基丁酸与内标比值增高（1252．4—9059．8）；ALDH5A1基因突变分析示不同位点均可出现突变（2例为c．1568C〉T纯合突变，余4例分别为c．691G〉A，c．1568C〉T；IVS9—2delA，C．1568C〉T；c．527G〉A，c．691G〉A；c．904G〉A，O．1022C〉A复合杂合突变）。结论头MRI双侧对称性苍白球病变是本病影像学特征。尿GC／MS为本病诊断依据。ALDH5A1基因不同位点均可发生突变，为本病基因诊断依据。