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胰腺实性假乳头状瘤合并胰腺分裂症。病例报告。

Solid pseudopapillary tumor of the pancreas with concomitant pancreas divisum. A case report.

作者信息

Watanabe Daisuke, Miura Kouichi, Goto Takashi, Nanjo Hiroshi, Yamamoto Yuzo, Ohnishi Hirohide

机构信息

Department of Gastroenterology, Noshiroyamamoto Medical Association Hospital, Noshiro, Japan.

出版信息

JOP. 2010 Jan 8;11(1):45-8.

PMID:20065552
Abstract

CONTEXT

Solid pseudopapillary tumor of the pancreas is a rare neoplasm which affects young women. On the other hand, pancreas divisum is an anomaly which develops at 7 weeks of gestation. Here, we report a case of a solid pseudopapillary tumor of the pancreas with concomitant pancreas divisum.

CASE REPORT

A 26-year-old woman was diagnosed as having a pancreatic tumor with solid and cystic components in the pancreatic head. Pancreatograms obtained by ERCP and MRCP showed no communication between the ventral and dorsal pancreatic ducts, indicating that pancreas divisum was present. Microscopically, the resected tumor had solid and cystic components. Immunohistochemical study demonstrated that the tumor cells were positive for alpha-1-antitrypsine, vimentin and progesterone receptors but negative for estrogen receptors, NSE, insulin or glucagon. The tumor was diagnosed as a solid pseudopapillary tumor of the pancreas. Although more than 700 cases of solid pseudopapillary tumors of the pancreas have been reported in the English literature, a search of PubMed turned up no reports of concomitant solid pseudopapillary tumor and pancreas divisum.

CONCLUSION

Solid pseudopapillary tumors of the pancreas with concomitant pancreas divisum are extremely rare.

摘要

背景

胰腺实性假乳头状瘤是一种罕见的肿瘤,好发于年轻女性。另一方面,胰腺分裂是一种在妊娠7周时出现的异常情况。在此,我们报告一例胰腺实性假乳头状瘤合并胰腺分裂的病例。

病例报告

一名26岁女性被诊断为胰头部有一个具有实性和囊性成分的胰腺肿瘤。通过内镜逆行胰胆管造影(ERCP)和磁共振胰胆管造影(MRCP)获得的胰管造影显示腹侧和背侧胰管之间无交通,提示存在胰腺分裂。显微镜下,切除的肿瘤具有实性和囊性成分。免疫组织化学研究表明,肿瘤细胞α1抗胰蛋白酶、波形蛋白和孕激素受体呈阳性,但雌激素受体、神经元特异性烯醇化酶(NSE)、胰岛素或胰高血糖素呈阴性。该肿瘤被诊断为胰腺实性假乳头状瘤。尽管英文文献中已报道了700多例胰腺实性假乳头状瘤,但在PubMed上搜索未发现胰腺实性假乳头状瘤合并胰腺分裂的报道。

结论

胰腺实性假乳头状瘤合并胰腺分裂极为罕见。

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