美国外科病理学杂志09年3月第20篇.pdfVIP

CASE REPORT Polypoid PEComa in the Rectum of a 15-year-old Girl Case Report and Review of PEComa in the Gastrointestinal Tract Paul Ryan, MD,*w Van-Hung Nguyen, MD,zy Suad Gholoum MD,Jy Lucia Carpineta, MD,zy Sharon Abish, MD,#y Najma N. Ahmed, MD, PhD, **y Jean-Martin Laberge, MD, Jy and Robert H. Riddell, MD*w he family of tumors first linked by Bonetti et al6 Abstract: PEComa of the gastrointestinal tract, composed of Tthrough being composed of perivascular epithelioid perivascular epithelioid cells with myomelanocytic differentia- cells (PECs) includes angiomyolipoma, clear cell ‘‘sugar’’ tion, is rare with previous literature limited to 16 case reports. tumor of lung, and lymphangioleiomyomatosis.43 These There is a marked female preponderance and approximately are rare tumors that are characterized by myomelanocytic one-third of the cases occur in the pediatric age group. We differentiation, for which neither a precursor lesion report PEComa with lymph node involvement occurring in the nor a cell of origin have yet been identified. The list of rectum of a 15-year-old girl, treated by surgical resection and anatomic sites where PEComa has been reported to adjuvant chemotherapy. The patient is well at 9 months follow- occur continues to lengthen,26,20 but data on PEComa up with neither radiologic nor endoscopic evidence of recur- arising in the bowel remain limited to isolated case rence. We review the differential diagnosis of intestinal reports,1,4,5,7,11,16,27,29,30,32,36,40,41