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.

Hepatic perivascular epithelioid cell tumors: The importance of preoperative diagnosis

“…Preoperative laboratory tests may only meet the requirements for excluding certain diseases. For example, in the three patients reported by Kou et al [ 1 ], except for the increase in CA-125 in Patient 3 with an ovarian tumor, the patients did not have abnormal serum tumor marker levels, which was consistent with previous findings that PEComas were not accompanied by abnormal serological results[ 7 , 8 ]. This approach may help clinicians rule out the diagnosis of some common tumors or simply make them doubt the proposed diagnosis.…”

“…We read the recently published papers of Kou et al [ 1 ] and express our satisfaction and congratulations on their excellent work. This is a well-written case report.…”

“…By introducing three rare cases of hepatic perivascular epithelioid cell tumors (PEComas), they further noted the practical challenges faced by clinicians in the face of hepatic PEComas (HPEComas). We fully understand Kou et al ’s concerns about the preoperative misdiagnosis of HPEComas[ 1 ]. Although the accuracy of a single examination may be insufficient to meet diagnostic requirements, combined examination of multiple imaging and immunohistochemical markers may be an effective method for improving accuracy.…”

“…In conclusion, Kou et al ’s concern about the preoperative misdiagnosis of liver PEComas is entirely reasonable and necessary[ 1 ]. The preoperative diagnosis of HPEComas is very important.…”

“…Imaging and serological tests are the first step, followed by biopsy. However, we also need to point out that the clinical reality is often more complex than theoretical accounts, as in the three cases reported by Kou et al [ 1 ]. Although all the patients were subjected to ultrasound, three-phase enhanced tomography, enhanced MRI, and intraoperative frozen pathology, the results still suggested HCC.…”