Abstract
PEComas are a collection of generally rare tumors, defined by the World Health Organization as ‘mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epitheloid cells’. We describe the case of retroperitoneal PEComa with a liposarcoma-like appearance on cross-sectional imaging, but distinctive immunohistochemistry revealing the correct diagnosis.
Highlights
Perivascular Epitheloid Cell tumors (PEComas) are rare mesenchymal tumors that can appear at various sites in the body and can be associated with the tuberous sclerosis complex
We present the case of a large lipid-rich Perivascular epitheloid cell tumor (PEComa) primarily misdiagnosed as a liposarcoma on computed tomography and magnetic resonance imaging
The degree of malignancy is highly variable in PEComas, but as with other sarcomas, the size of the primary tumor as well as the mitotic rate seem to be the most reliable prognostic factors associated with recurrence after surgical resection
Summary
Perivascular Epitheloid Cell tumors (PEComas) are rare mesenchymal tumors that can appear at various sites in the body and can be associated with the tuberous sclerosis complex. We present the case of a large lipid-rich PEComa primarily misdiagnosed as a liposarcoma on computed tomography and magnetic resonance imaging. Histology revealed a lipid-rich PEComa without any signs of malignancy, which required no further treatment following surgical resection. The contrast-enhanced CT scan showed an 8 × 11 × 15 cm large retroperitoneal mass in the lower left abdomen adjacent to the sigmoid colon and the left psoas muscle (Figure 1A). Due to the imaging findings, a retroperitoneal liposarcoma was suspected and the patient underwent surgery with resection of the mass and the adjacent sigmoid colon. A fluorescence in situ hybridization (FISH) for MDM2 gene amplification was negative, a liposarcoma was ruled out and the final diagnosis was lipid-rich perivascular epitheloid cell tumor (PEComa) without any signs of malignancy. The patient recovered well from surgery and did not require any further adjuvant treatment
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