Pancreatic Perivascular Epithelial Cell Tumor (PEComa) with Liver Metastasis: a Case Report and Literature Review

Aim : to present a clinical case of pancreatic PEComa with liver metastases an emphasis on morphological features, radiology diagnostic methods and features treatment tactics, allowing practitioners get idea about this rare mesenchymal tumors composed “perivascular epithelioid cells”, review literature data PEComa, including 32 author's cases. General statements . A 22-year-old woman who was surgically treated for head infiltration the duct Wirsung, common bile duct, duodenal wall, focal invasion into blood lymphatic vessels, perineural space infiltration. Synchronously, were detected in both lobes, which she took Everolimus 6 years under control dose adjustment frequency administration. At A.V. Vishnevsky National Medical Research Center Surgery, patient underwent right-sided hemihepatectomy atypical resection II-III segments. Conclusion To determine clearer criteria diagnosis differential identify malignancy these tumors, develop tactics further dynamic monitoring, set statistical significant group randomized trials are needed. Pancreatic PEComas extremely often have benign course. The presented demonstrates most malignant form tumor localization pancreas metastases.