Clinicopathological Features of Two Kinds of Liver Epithelioid Soft Tissue Tumors Prone to Misdiagnosis and Missed Diagnosis

WANG Lu; CHEN Simin; LI Yuan; SI Haipeng

doi:10.3969/j.issn.1002-3429.2025.07.006

Abstract：Objective To investigate the clinicopathological features and causes of misdiagnosis and missed diagnosis of two rare hepatic epithelioid soft tissue tumors.Methods From July 2022 to May 2024,8 cases of liver biopsy specimens were collected,including 4 cases of hepatic perivascular epithelioid cell tumor(PEComa)and 4 cases of hepatic epithelioid hemangioendothelioma(HEHE).The clinical data,pathological morphology and immunohistochemical staining markers of the patients were retrospectively analyzed.Results Four patients with hepatic PEComa were admitted to the hospital for abdomi-nal pain(n=1),fatigue and weight loss(n=1),and incidental diagnosis(n=2).Among the 4 HEHE patients,3 were admitted due to abdominal pain and 1 was incidentally found by physical examination.All the 8 patients had epithelioid tumor cells,which were similar to epithelioid hepatocytes.It is difficult to make diagnosis based on HE staining,which usually leads to misdiagnosis and missed diagnosis at the initial hospital visit.Hepatic PEComa was misdiagnosed as hepatic epithelial tumors,while HEHE was missed because no tumor cells were found,and finally diagnosed based on immunohistochemistry ex-amination.Microscopically,the tumor cells were radially arranged along the blood vessel wall,while immunohistochemically,the tumor cells were characteristically positive for melanocytes and smooth muscle markers.Microscopically,HEHE was com-posed of infiltrating epithelioid or spindle cells with a structure similar to primitive vascular lumen.Immunohistochemically,the tumor cells were positive for vascular endothelial markers.Four cases of hepatic PEComa did not undergo surgery,of which 1 patient did not progress after palliative treatment,2 patients had a good prognosis after interventional embolization,and 1 patient of suspected malignant tumor had a poor prognosis after interventional embolization and immunotherapy.Four pa-tients with HEHE did not undergo surgery,3 patients underwent microwave ablation and interventional embolization,and 1 pa-tient was treated with Everolimus and Interferon after microwave ablation.The follow-up prognosis was good.Conclusion PEComa and HEHE,the two rare soft tissue tumors in liver biopsy specimens,have epithelioid cells,and the differential diag-nosis is difficult.However,the definite diagnosis can be made according to the characteristic histological morphology and im-munohistochemical markers,which is helpful for clinical accurate treatment and prognosis evaluation.