First description of a PEComa (perivascular epithelioid cell tumor) of the colon: report of a case and review of the literature.
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.
Birkhaeuser
Frederic
F
Department of Surgery, St. Claraspital, Basel, Switzerland.
Ackermann
Christoph
C
Flueckiger
Tobias
T
Guenin
Marc-Olivier
MO
Kern
Beatrice
B
Tondelli
Peter
P
Peterli
Ralph
R
D000328:Adult; D000367:Age Factors; D002277:Carcinoma / Q000473:pathology* / Q000601:surgery*; D002432:Cecum / Q000473:pathology*; D003110:Colonic Neoplasms / Q000473:pathology* / Q000601:surgery*; D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D006801:Humans