Intrahepatic peribiliary perivascular epithelioid cell tumor (PEComa) associated with heterotopic pancreas: A case report

被引：9

作者：

Kiriyama, Yuka ^{[1
]}

Tsukamoto, Tetsuya ^{[1
]}

Mizoguchi, Yoshikazu ^{[1
]}

Ishihara, Shin ^{[2
]}

Horiguchi, Akihiko ^{[2
]}

Tokoro, Takamasa ^{[2
]}

Kato, Yutaro ^{[2
]}

Sugioka, Atsushi ^{[2
]}

Kuroda, Makoto ^{[1
]}

机构：

[1] Fujita Hlth Univ, Sch Med, Dept Diagnost Pathol, 1-98 Dengakugakubo,Kutsukake Cho, Toyoake, Aichi 4701192, Japan

[2] Fujita Hlth Univ, Sch Med, Dept Surg, Toyoake, Aichi, Japan

来源：

DIAGNOSTIC PATHOLOGY | 2016年 / 11卷

关键词：

Perivascular epithelioid cell tumor (PEComa); Liver; Heterotopic pancreas; HEPATIC ANGIOMYOLIPOMA; ADENOCARCINOMA; TISSUE;

D O I：

10.1186/s13000-016-0528-9

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

Background: Perivascular epithelioid-cell tumor (PEComa) is a group of rare mesenchymal neoplasms that express myomelanocytic-cell markers and exhibit a wide variety of histopathological features. Although heterotopic pancreas has been reported to occur in the gastrointestinal tract, intrahepatic heterotopic pancreas has been reported only rarely. Case presentation: We present a case of intrahepatic PEComa that showed a strong regional correlation with the presence of heterotopic pancreas. An intrahepatic tumor and biliary dilatation was incidentally discovered during a diagnostic evaluation to investigate low-back pain in a 47-year-old Japanese male. Cholangiocarcinoma was suspected and a left hemihepatectomy performed. Histological examination revealed a 3 x 3.8-mm tumor in the neighboring B2 bile duct. Histological and immunohistochemical investigations revealed the presence of a PEComa and pancreatic acini within the tumor mass. PEComa in the hepatobiliary and pancreatic regions are extremely rare. The presence of heterotopic pancreas is also relatively uncommon. Conclusion: The strong regional association of these 2 lesions raises the possibility of a PEComa originating from heterotopic pancreas or from an irritable response caused by heterotopic pancreas.

引用

页数：5