Intrahepatic peribiliary perivascular epithelioid cell tumor (PEComa) associated with heterotopic pancreas: A case repor
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CASE REPORT
Open Access
Intrahepatic peribiliary perivascular epithelioid cell tumor (PEComa) associated with heterotopic pancreas: A case report Yuka Kiriyama1, Tetsuya Tsukamoto1* , Yoshikazu Mizoguchi1, Shin Ishihara2, Akihiko Horiguchi2, Takamasa Tokoro2, Yutaro Kato2, Atsushi Sugioka2 and Makoto Kuroda1
Abstract 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 × 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. Keywords: Perivascular epithelioid cell tumor (PEComa), Liver, Heterotopic pancreas
Background Perivascular epithelioid cell tumors (PEComas) constitute a rare family of mesenchymal tumors that can occur in any part of the human body. Cases developing in the liver are, however, extremely rare [1]. PEComas arising in the pancreas are even rarer, and only 12 cases have been reported so far [2]. Heterotopic pancreatic tissue is usually found in the gastrointestinal tract; however, it is rarely detected in the liver and only 0.5–13.7 % have been identified in autopsies [3]. To our knowledge, no cases of a combination of PEComa and heterotopic pancreas have been previously described. We present here an incidentally found case of intrahepatic PEComa that was strongly associated with heterotopic pancreas tissue. * Correspondence: [email protected] 1 Department of Diagnostic Pathology, Fujita Health University School of Medicine, 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi 470-1192, Japan Full list of author information is available at the end of the article
Case presentation A 47-year-old Japanese man visited a local doctor complaining of low-back pain 5 months before admission to our hospital. The patient had no relevant past or family history. Abdominal computed tomography (CT) and ultrasonography (US) incidentally revealed a dilatation of the intrahepatic bile duct. The patient was referred to our hospital for further investigation
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