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Hemangioendothelioma: Soft Tissue
Catherine C. Roberts, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Epithelioid hemangioendothelioma (EH)
          • Kaposiform hemangioendothelioma (KH)
          • Retiform hemangioendothelioma (RH)
          • Composite hemangioendothelioma (CH)
          • Papillary intralymphatic angioendothelioma (PILA)
        • Synonyms

          • EH: Intravascular bronchioloalveolar tumor, angioglomoid tumor, myxoid angioblastomatosis
          • KH: Kaposi-like infantile hemangioendothelioma, hemangioma with Kaposi sarcoma-like features
          • RH: Hobnail hemangioendothelioma
          • PILA: Dabska tumor, hobnail hemangioendothelioma, malignant endothelial papillary angioendothelioma
        • Definitions

          • Group of intermediate to malignant vascular neoplasms with less aggressive behavior than angiosarcoma
            • Epithelioid hemangioendothelioma is malignant
            • Other forms have intermediate biologic behavior with local aggressiveness ± rare metastases

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    • Gross Pathologic & Surgical Features

                      • Microscopic Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                Selected References

                                1. Amary MF et al: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: characterization of five cases. Skeletal Radiol. 42(7):947-57, 2013
                                2. Azzam RI et al: AIRP best cases in radiologic-pathologic correlation: Hepatic epithelioid hemangioendothelioma. Radiographics. 32(3):789-94, 2012
                                3. Errani C et al: Vascular bone tumors: a proposal of a classification based on clinicopathological, radiographic and genetic features. Skeletal Radiol. 41(12):1495-507, 2012
                                4. Mohankumar R et al: Multi-focal epithelioid haemangioendothelioma of the bone and skin. Skeletal Radiol. 40(7):955-6, 2011
                                5. Tamai N et al: Kaposiform hemangioendothelioma arising in the deltoid muscle without the Kasabach-Merritt phenomenon. Skeletal Radiol. 39(10):1043-6, 2010
                                6. Weiss SW et al: Hemangioendothelioma: Vascular tumors of intermediate malignancy. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 681-701, 2008
                                7. Kransdorf MJ et al: Vascular and lymphatic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 177-88, 2006
                                8. Calonje E: Papillary intralymphatic angioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 167, 2002
                                9. Calonje E: Retiform hemangioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 165-66, 2002
                                10. Rubin BP: Composite hemangioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 168-9, 2002
                                11. Tsang WYW: Kaposiform hemangioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 163-4, 2002
                                12. Weiss SW et al: Epithelioid hemangioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 173-5, 2002
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Clinical Issues

                                      TERMINOLOGY

                                      • Abbreviations

                                        • Epithelioid hemangioendothelioma (EH)
                                        • Kaposiform hemangioendothelioma (KH)
                                        • Retiform hemangioendothelioma (RH)
                                        • Composite hemangioendothelioma (CH)
                                        • Papillary intralymphatic angioendothelioma (PILA)
                                      • Synonyms

                                        • EH: Intravascular bronchioloalveolar tumor, angioglomoid tumor, myxoid angioblastomatosis
                                        • KH: Kaposi-like infantile hemangioendothelioma, hemangioma with Kaposi sarcoma-like features
                                        • RH: Hobnail hemangioendothelioma
                                        • PILA: Dabska tumor, hobnail hemangioendothelioma, malignant endothelial papillary angioendothelioma
                                      • Definitions

                                        • Group of intermediate to malignant vascular neoplasms with less aggressive behavior than angiosarcoma
                                          • Epithelioid hemangioendothelioma is malignant
                                          • Other forms have intermediate biologic behavior with local aggressiveness ± rare metastases

                                      IMAGING

                                      • General Features

                                        • Radiographic Findings

                                          • CT Findings

                                            • MR Findings

                                              DIFFERENTIAL DIAGNOSIS

                                                PATHOLOGY

                                                • General Features

                                                  • Gross Pathologic & Surgical Features

                                                    • Microscopic Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              Selected References

                                                              1. Amary MF et al: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: characterization of five cases. Skeletal Radiol. 42(7):947-57, 2013
                                                              2. Azzam RI et al: AIRP best cases in radiologic-pathologic correlation: Hepatic epithelioid hemangioendothelioma. Radiographics. 32(3):789-94, 2012
                                                              3. Errani C et al: Vascular bone tumors: a proposal of a classification based on clinicopathological, radiographic and genetic features. Skeletal Radiol. 41(12):1495-507, 2012
                                                              4. Mohankumar R et al: Multi-focal epithelioid haemangioendothelioma of the bone and skin. Skeletal Radiol. 40(7):955-6, 2011
                                                              5. Tamai N et al: Kaposiform hemangioendothelioma arising in the deltoid muscle without the Kasabach-Merritt phenomenon. Skeletal Radiol. 39(10):1043-6, 2010
                                                              6. Weiss SW et al: Hemangioendothelioma: Vascular tumors of intermediate malignancy. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 681-701, 2008
                                                              7. Kransdorf MJ et al: Vascular and lymphatic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 177-88, 2006
                                                              8. Calonje E: Papillary intralymphatic angioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 167, 2002
                                                              9. Calonje E: Retiform hemangioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 165-66, 2002
                                                              10. Rubin BP: Composite hemangioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 168-9, 2002
                                                              11. Tsang WYW: Kaposiform hemangioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 163-4, 2002
                                                              12. Weiss SW et al: Epithelioid hemangioendothelioma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. Lyon: IARC Press. 173-5, 2002