Published online Apr 7, 2016. doi: 10.3748/wjg.v22.i13.3693
Peer-review started: November 12, 2015
First decision: December 11, 2015
Revised: December 27, 2015
Accepted: January 11, 2016
Article in press: January 11, 2016
Published online: April 7, 2016
Perivascular epithelioid cell tumor (PEComa) of the pancreas is an unusual tumor deriving from mesenchyma. This paper described a case of pancreatic PEComa, which was initially suspected as neuroendocrine carcinoma by biopsy, and therefore surgical treatment was recommended due to undetermined diagnosis. Examination of the surgical specimen under a microscope showed that the tumor cell’s morphology was epithelioid or spindle-shaped, and ranged in a nested pattern. Additionally, these cells had a large extent of acidophilic cytoplasm, no mitotic figures, and expressed HMB-45, melan-p, and smooth muscle actin immunohistochemically. Pathological examination indicated that PEComa originated from the pancreas, but symptoms related to tuberous sclerosis were absent. Since PEComa is extremely rare in the pancreas, it is likely to be ignored in differential diagnosis. In conclusion, our article highlighted the clinicopathological features of PEComa, and we conducted a literature review focusing on PEComa so as to deepen the understanding of this tumor type.
Core tip: Perivascular epithelioid cell tumor (PEComa) of the pancreas is an unusual tumor deriving from mesenchyma. Via describing a rare case of pancreatic PEComa, we highlight the clinicopathological features of PEComa and conduct a literature review focusing on this tumor type so as to deepen the understanding of the subject. We also reviewed the biological behavior, prognosis, and therapeutic strategy of PEComa.