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

Abstract

BackgroundPerivascular 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 presentationWe 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.ConclusionThe 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.

Pancreatic Perivascular Epithelial Cell Tumor (PEComa) with Liver Metastasis: a Case Report and Literature Review

“…Размер опухолей варьировал в пределах от 0,38 до 11,5 см. Следует отметить, что размеры опухоли 0,38 см были выявлены в опухоли, локализованной в гетеротопической ПЖ [37]. Чаще всего опухоль локализовалась в головке ПЖ (16 пациентов, 48,5 %).…”

Unusual paediatric sigmoid perivascular epithelioid cell tumour with regional lymph node metastasis treated using gemcitabine and docetaxel: a case report and literature review

“…Review of an article by Chen et al, 3 and a PubMed search of articles published up to July 2020, using the following search terms: ("perivascular epithelioid cell tumor" or "PEComa") and ("gastrointestinal tract" or "GI" or "oral " or "mouth" or "esophagus" or "gullet" or "gastric" or "stomach" or "duodenum" or "jejunum" or "ileum" or "cecum" or "colon" or "colorectal" or "sigmoid" or "rectum" or "anus" or "mesentery"), revealed 62 cases of gastrointestinal PEComa-NOS in the literature. 3,12,[15][16][17][18][19][20][21][22][23] the colon was the most commonly affected part of the abdominal cavity, followed by the mesentery, rectum, stomach, duodenum, ileum, cecum, and other locations. 3 The GI tract is the second most frequent location of PEComa, preceded only by the gynaecological tract.…”

Primary Liver Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review

“…The most common locations of PEComas are the ovaries, uterus, gastrointestinal tract, bladder, abdominal wall, heart, and pancreas [ 5 ]. Cases of primary liver PEComas are extremely rare [ 6 , 7 , 8 ].…”