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Perivascular Epithelioid Cell Tumor (PEComa)
Matthew T. Heller, MD, FSAR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Perivascular epithelioid cell tumor (PEComa)
              • Synonyms

                • Subgroups of PEComa
                  • Angiomyolipoma, clear cell "sugar" tumors, clear cell myomelanocytic tumors, sarcoma of perivascular cells, lymphangioleiomyomatosis, and pigmented melanotic tumors
              • Definitions

                • Mesenchymal tumor comprised of perivascular epithelioid cells arranged radially around a vascular lumen

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Ultrasonographic Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Shi H et al: Malignant perivascular epithelioid cell tumor of the kidney with rare pulmonary and ileum metastases. Int J Clin Exp Pathol. 7(9):6357-63, 2014
                                            2. Tirumani SH et al: Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors. AJR Am J Roentgenol. 202(2):252-8, 2014
                                            3. Tan Y et al: Perivascular epithelioid cell tumour: dynamic CT, MRI and clinicopathological characteristics--analysis of 32 cases and review of the literature. Clin Radiol. 68(6):555-61, 2013
                                            4. Chang H et al: Pigmented perivascular epithelioid cell tumor (PEComa) of the kidney: a case report and review of the literature. Korean J Pathol. 46(5):499-502, 2012
                                            5. Tan Y et al: Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features--analysis of 7 cases and review of the literature. Abdom Imaging. 37(5):781-7, 2012
                                            6. Rajiah P et al: Imaging of uncommon retroperitoneal masses. Radiographics. 31(4):949-76, 2011
                                            7. Folpe AL et al: Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 41(1):1-15, 2010
                                            8. Folpe AL et al: Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 29(12):1558-75, 2005
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Clinical Issues

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Perivascular epithelioid cell tumor (PEComa)
                                                        • Synonyms

                                                          • Subgroups of PEComa
                                                            • Angiomyolipoma, clear cell "sugar" tumors, clear cell myomelanocytic tumors, sarcoma of perivascular cells, lymphangioleiomyomatosis, and pigmented melanotic tumors
                                                        • Definitions

                                                          • Mesenchymal tumor comprised of perivascular epithelioid cells arranged radially around a vascular lumen

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Microscopic Features

                                                                            CLINICAL ISSUES

                                                                            • Presentation

                                                                              • Demographics

                                                                                • Natural History & Prognosis

                                                                                  • Treatment

                                                                                    DIAGNOSTIC CHECKLIST

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Shi H et al: Malignant perivascular epithelioid cell tumor of the kidney with rare pulmonary and ileum metastases. Int J Clin Exp Pathol. 7(9):6357-63, 2014
                                                                                      2. Tirumani SH et al: Imaging features of primary and metastatic malignant perivascular epithelioid cell tumors. AJR Am J Roentgenol. 202(2):252-8, 2014
                                                                                      3. Tan Y et al: Perivascular epithelioid cell tumour: dynamic CT, MRI and clinicopathological characteristics--analysis of 32 cases and review of the literature. Clin Radiol. 68(6):555-61, 2013
                                                                                      4. Chang H et al: Pigmented perivascular epithelioid cell tumor (PEComa) of the kidney: a case report and review of the literature. Korean J Pathol. 46(5):499-502, 2012
                                                                                      5. Tan Y et al: Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features--analysis of 7 cases and review of the literature. Abdom Imaging. 37(5):781-7, 2012
                                                                                      6. Rajiah P et al: Imaging of uncommon retroperitoneal masses. Radiographics. 31(4):949-76, 2011
                                                                                      7. Folpe AL et al: Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol. 41(1):1-15, 2010
                                                                                      8. Folpe AL et al: Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 29(12):1558-75, 2005