Perivascular epithelioid cell tumor (PEComa) in the transverse colon of an adolescent: a case report

Abstract

Perivascular epithelioid cell tumor (PEComa) is defined as a very rare mesenchymal tumor of histologically and immuno-histochemically distinctive perivascular epithelioid cells. PEComa in the colon is very rare, with only a few reported cases so far. Because of its rarity, the clinical features and biological behavior of PEComa in the colon have yet to be established. A 16-year-old female patient with PEComa in the transverse colon was referred to our hospital for rectal bleeding. Laboratory data showed a hemoglobin level of 6.6 g/dL, WBC of 8,800/mm(3), and platelet count of 191,000/mm(3). Colonoscopy, barium enema, and abdominal computed tomography revealed a 2-cm, smooth-surfaced, round tumor with focal ulceration in the proximal transverse colon. The patient complained of abdominal pain one day after endoscopic polypectomy. She underwent a segmental resection for a perforated transverse colon. Immunohistochemically, the tumor cells showed strong diffuse positivity for HMB-45 while they were negative for c-kit, smooth muscle actin, cytokeratin, S-100, vimentin, desmin, chromogranin, synaptophysin, EMA, and CD-34. The diagnosis of PEComa was based on histological and immunohistochemical staining. The patient did not receive any adjuvant therapy and was discharged on postoperative day 11 without complications. Whole-body fluorine-18 fluorodeoxyglucose fusion positron emission tomography performed 2 months after surgery showed no signs of recurrence or metastasis. There was also no recurrence or metastasis at 24 months' follow-up.