Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma: Three case reports

Abstract

BACKGROUND

Hepatic perivascular epithelioid cell neoplasms (PEComas) are rare. Diagnostic and treatment experience with hepatic PEComa remains insufficient.

CASE SUMMARY

Three hepatic PEComa cases are reported in this paper: One case of primary malignant hepatic PEComa, one case of benign hepatic PEComa, and one case of hepatic PEComa with an ovarian mature cystic teratoma. During preoperative imaging and pathological assessment of intraoperative frozen samples, patients were diagnosed with hepatocellular carcinoma (HCC), while postoperative pathology and immunohistochemistry subsequently revealed hepatic PEComa. Patients with hepatic PEComa which is misdiagnosed as HCC often require a wider surgical resection. It is easy to mistake them for distant metastases of hepatic PEComa and misdiagnosed as HCC, especially when it's combined with tumors in other organs. Three patients eventually underwent partial hepatectomy. After 1-4 years of follow-up, none of the patients experienced recurrence or metastases.

CONCLUSION

A clear preoperative diagnosis of hepatic PEComa can reduce the scope of resection and prevent unnecessary injuries during surgery.

Keywords: Perivascular epithelioid cell neoplasms; Hepatocellular carcinoma; Case report; Diagnoses; Coexistence

Core Tip: Herein, we present three cases of hepatic perivascular epithelioid cell neoplasms (PEComas): One case of primary malignant PEComa, one case of benign PEComa, and one case of PEComa with ovarian mature cystic teratoma. The first case of PEComa cooccurred with an ovarian mature cystic teratoma. All three cases were misdiagnosed as liver cancer before surgery. A high rate of misdiagnosis of hepatocellular carcinoma is noted among patients with PEComa. A clear preoperative diagnosis of hepatic PEComa is crucial before deciding on a treatment plan, especially with the extent of surgical treatment in hepatocellular carcinoma.