Perivascular epithelioid cell neoplasms (PEComas) are a tumor family defined as such just a couple of decades ago. They make an unusual group of neoplasms, which can appear in different locations of the organism. PEComas are usually considered to be benign tumors, but there are some histological features that make some subgroups suspicious of malignancy. The treatment of these tumors consist in their surgical resection, with no current effective complementary oncological treatment known. We present the clinical case of a woman that underwent surgery for a resection of a hepatic lesion labeled afterwards as a PEComa with malignant features.
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