A Case of a Perivascular Epithelioid Cell Tumor Mimicking Colon Cancer

Abstract

Perivascular epithelioid cell tumor (PEComa) is extremely rare, which originated from mesenchymal cells in the intestine, and composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. We report here on a case of PEComa in the sigmoid colon. A 62-year-old woman presented with hematochzia 10 days ago. Her abdominal computed tomography scan showed a 5 cm sized intraluminal fungating heterogeneously enhanced, high density mass, which infiltrated pericolic tissue surrounding the sigmoid colon. Colonoscopy showed a purple colored polypoid mass with lobulating contour in the sigmoid colon. She underwent laparoscopic anterior resection. On the histologic examination, the tumor consisted of polygonal epithelioid cells with sheet-like growth of nests, which looked like alveolar tissues in lung. The tumor cells were strongly positive stained with human melanoma black-45 (HMB-45). Pathologic examination was compatible with PEComa. Sixteen months after surgery, she did well without tumor recurrence after surgery. We review the literatures concerning PEComa of the intestine focusing on endoscopic findings. (Korean J Gastroenterol 2012;60:377-381)

Pediatric case of colonic perivascular epithelioid cell tumor complicated with intussusception and anal incarceration: A case report

“…There was a significant sex difference among the adults but no significant sex difference among the children, consistent with the findings reported by Fadare[ 33 ], who proposed that PEComa may be a hormone-dependent tumor. PEComa can occur in all parts of the colon, although they occur more often in the left colon (9 cases in the sigmoid colon[ 11 , 12 , 15 , 16 , 18 , 21 , 22 , 26 , 31 ], 5 in the rectum[ 6 , 7 , 13 , 28 , 29 ], 3 in the descending colon[ 9 , 16 , 27 ], 4 in the ascending colon[ 16 , 19 , 30 ], 7 in the cecum[ 8 , 14 , 20 , 23 - 25 , 32 ], 1 in the transverse colon[ 10 ], and 1 in the right colon[ 17 ]).…”

Laparoscopically resected perivascular epithelioid cell tumor of the ascending colon demonstrating submucosal tumor morphology: a case report

“…We did a literature search using PubMed to investigate the clinicopathological features of colonic PEComa and found 21 primary cases, including that of our patient [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] . These are summarized in Table 1.…”