Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor

Abstract

Approximately 10% of patients with gastrointestinal stromal tumors (GIST) develop other neoplasms, either synchronously or metachronously. In this report we describe coexistence of a gastrointestinal stromal tumor and a hepatic perivascular epithelioid cell tumor (PEComa) in a 51-year-old woman with no evidence of tuberous sclerosis. A subcapsular hepatic nodule (0.8 cm in diameter) was found during surgery for symptomatic gastric neoplasm (15 cm in diameter) arising from the lesser curvature. Both tumors revealed histomorphological and immunohistochemical features confirming a diagnosis of a small incidental hepatic PEComa and a high risky extramural gastric GIST, respectively. The patient remained disease-free 25 mo after surgery with no evidence of tumor recurrence or new neoplasms. To our knowledge, this is the first report of PEComa in a patient with GIST. Hepatic lesions detected synchronously or metachronously in patients with GISTs may represent histogenetically distinct lesions and should be sampled to confirm or exclude metastatic GISTs.

Primary perivascular epithelioid cell tumor (PEComa) of the liver: Report of a case

“…9 Mesenchymal neoplasms of the liver are very uncommon. 10 A review of the pertinent literature revealed only a few cases of PEComa of the liver; [11][12][13][14][15][16][17] most of them were referred to as angiomyolipoma. The authors herein present a case report of extremely rare histological tumor type of the liver, namely primary clear cell "sugar" tumor with myomelanocytic differentiation, and demonstrate its clinical, imaging, pathological, and immunohistochemical characteristics.…”

Contrast-enhanced ct and angiographic findings in hepatic perivascular epithelioid cell tumor

“…Carlos et al (8) reported a case of PEComa coexisting with GIST. Loss of heterozygosity (LOH) in chromosome 16p (harboring the TSC2 locus) has been detected in some PEComas, but it has not been investigated in GISTs (1, 3).…”

“…Four patients had normal levels of serum AFP and were negative for hepatitis B surface antigen and anti-hepatitis C virus antigen (6,10,12,15); reports of the other 8 cases did not mention this. Two patients had a history of melanoma (13,14), and 1 had a history of GIST (8). Four had no history of tuberous sclerosis.…”

“…The resections of these 12 tumors ranged from tumorectomy to hemihepatectomy (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15). In most cases, PEComas are benign, and patients have good postoperative prognosis and no recurrence after several months of follow-up.…”

“…Generally, indigestion, loss of appetite, nausea, body weight loss, and intermittent colic pain can occur, and on physical examination tenderness to palpation and liver enlargement may be observed. These tumors have occurred in female patients aged from 13 to 70 years (median age: 55) (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15). Of the reported 12 cases (5-15), 6 cases occurred in the left lobe (7-9, 11, 14, 15), 4 in the right lobe (5,6,10,12), 1 in the caudate lobe (7), and 1 in the ligamentum teres (13).…”