First Description of a PEComa (Perivascular Epithelioid Cell Tumor) of the Colon: Report of a Case and Review of the Literature

Abstract

We describe a young female patient suffering from a PEComa (perivascular epithelioid cell tumor) of the cecum, incidentally found at an examination made by her family physician. The perivascular epithelioid cell tumor is a very rare tumor, until today reported in a few cases in falciform ligament, uterus, jejunum, terminal ileum, rectum, liver, kidney, lung, pancreas, prostate, and soft tissue of the thigh. This tumor is part of a new group of tumors, comprised of angiomyolipoma, lymphangiomyolipoma, and clear-cell myomelanocytic "sugar" tumor. Defined by coexpression of melanocytic (HMB-45) and muscle markers (smooth muscle actin and desmin) the perivascular epithelioid cell tumor does not have predictable histopathologic behavior. Some cases of metastasis are described, comorbidities such as tuberous sclerosis of the brain "Bourneville" and lymphangioleiomyomatosis have to be excluded. The therapy consists of the radical resection. An adjuvant therapy is not known. Recommended is a close and long-term follow-up clinically and by CT scan.

Malignant hepatic perivascular epithelioid cell tumor (PEComa) – Case report and a brief review

“…They have in common the co-expression of immunohistochemical markers for smooth muscles and melanocytes [4]. The histogenesis and pathogenesis of PECs are still unclear because physiological counterpart of a PEC has yet to be determined.…”

Perivascular epithelioid cell tumor of the ascending colon mesentery in a child: case report and review of the literature

“…Two of their patients (both female, one 9 years old and the other 40 years old) presented with rectal tumors. Birkhaeuser et al [9] described what they believed to be the first case of PEComa of the colon in a 35-year-old woman.…”

Ectopic hyperprolactinaemia in a woman with a mesocolic perivascular epithelioid cell tumor (“PEComa”)

“…This renal tumor can be assimilated to a PEComa, since it contained a perivascular epithelioid cells contingent. PEComas are rare mesenchymal neoplasms, principally described in women over 40 years old [11][12][13]. They appear to arise most commonly in the pelvis (described in the falciform ligament, the uterus, the prostate), the digestive tract (described in the terminal ileum, the colon, the rectum), the liver, the pancreas, the kidney, the lung or soft tissue of the thigh [12].…”