胰腺实性-假乳头状瘤与内分泌肿瘤的病理鉴别诊断。
Pathological differential diagnosis of solid-pseudopapillary neoplasm and endocrine tumors of the pancreas.
机构信息
Department of Pathology, Xiangya Basic Medical School, Central-south University, 110# Tongzipo Road, Changsha 410013, Hunan Province, China.
出版信息
World J Gastroenterol. 2010 Feb 28;16(8):1025-30. doi: 10.3748/wjg.v16.i8.1025.
AIM
To investigate differential points of solid-pseudopapillary neoplasm (SPN) of the pancreas and pancreatic endocrine tumor (PET).
METHODS
Ten cases of SPN and fourteen cases of PET were studied in this retrospective study. Clinical and pathologic features, immunostaining reactions and beta-catenin gene mutations were analyzed.
RESULTS
The mean age of SPN patients was 25.6 years and these patients had no specific symptoms. The mean diameter of the tumors was 11.0 cm, 9/10 cases were cystic or a mixture of solid and cystic structures, and there was hemorrhage and necrosis on the cut surface in 8/10 (80%) cases. Characteristic pseudopapillary structure and discohesive appearance of the neoplastic cells were observed in all 10 (100%) cases. The results of immunostaining showed that nuclear expression of beta-catenin and loss of E-cadherin in all the cases, was only seen in SPN. Molecular studies discovered that 9/10 (90%) cases harbored a point mutation of exon 3 in beta-catenin gene. On the other hand, the mean age of PET patients was 43.1 years. Eight of 14 cases presented with symptoms caused by hypoglycemia, and the other 6 cases presented with symptoms similar to those of SPN. The mean size of the tumors was 2.9 cm, most of the tumors were solid, only 3/14 (21%) were a mixture of solid and cystic structures, and macroscopic hemorrhage and necrosis were much less common (3/14, 21%). Histologically, tumor cells were arranged in trabecular, acinar or solid patterns and demonstrated no pseudopapillary structure and discohesive appearance in all 14 (100%) cases. The results of immunostaining and mutation detection were completely different with SPN that membrane and cytoplastic expression of beta-catenin without loss of E-cadherin, as well as no mutation in beta-catenin gene in all the cases.
CONCLUSION
Both macroscopic and microscopic features of SPN are quite characteristic. It is not difficult to distinguish it from PET. If necessary, immunostaining of beta-catenin and E-cadherin is quite helpful to make the differential diagnosis.
目的
探讨胰腺实性假乳头状瘤（SPN）和胰腺内分泌肿瘤（PET）的鉴别要点。
方法
回顾性分析 10 例 SPN 和 14 例 PET 患者的临床和病理特征、免疫组化反应和β-连环蛋白基因突变情况。
结果
SPN 患者的平均年龄为 25.6 岁，无特异性症状。肿瘤平均直径为 11.0cm，9/10 例为囊实性或囊实性混合结构，10/10（80%）例肿瘤切面可见出血坏死。所有 10 例（100%）均可见特征性假乳头状结构和瘤细胞离散排列。免疫组化结果显示，所有病例均有核β-连环蛋白表达和 E-钙黏蛋白丢失，仅见于 SPN。分子研究发现，9/10（90%）例β-连环蛋白基因外显子 3 点突变。另一方面，PET 患者的平均年龄为 43.1 岁。14 例中有 8 例因低血糖引起症状，6 例有类似 SPN 的症状。肿瘤平均大小为 2.9cm，大部分为实性，14/14（100%）例为实性和囊性混合结构，宏观出血坏死少见（3/14，21%）。组织学上，肿瘤细胞呈小梁状、腺泡状或实性排列，14 例（100%）均未见假乳头状结构和离散排列，均有膜和细胞质β-连环蛋白表达，E-钙黏蛋白无丢失，β-连环蛋白基因无突变。
结论
SPN 的大体和镜下特征均具有特征性，与 PET 不难鉴别。必要时免疫组化β-连环蛋白和 E-钙黏蛋白有助于鉴别诊断。
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