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Angiosarcoma of Soft Tissue
Stephanie A. Bernard, MD; B. J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Synonyms

          • Older terms (no longer recommended): hemangiosarcoma, malignant hemangioendothelioma, lymphangiosarcoma, hemangioblastoma, malignant angioendothelioma
        • Definitions

          • Malignant vascular neoplasm with exceptionally poor prognosis
        • Risk Factors

          • Chronic lymphedema related: Stewart-Treves syndrome
          • Radiation induced: > 5 years after XRT
          • Toxin exposure: vinyl chloride and thorium dioxide (Thorotrast) exposure → liver angiosarcoma
          • Syndrome associated: Maffucci syndrome; neurofibromatosis, Klippel-Trenaunay syndrome
          • May occur next to implants (synthetic grafts) or sites of prior trauma
          • 85% have no identifiable risk factor

        IMAGING

        • General Features

          • CT Findings

            • MR Findings

              • Nuclear Medicine Findings

                DIFFERENTIAL DIAGNOSIS

                  PATHOLOGY

                  • General Features

                    • Gross Pathologic & Surgical Features

                      • Microscopic Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                Selected References

                                1. Weidema ME et al: Targeting angiosarcomas of the soft tissues: a challenging effort in a heterogeneous and rare disease. Crit Rev Oncol Hematol. 138:120-31, 2019
                                2. Gaballah AH et al: Angiosarcoma: clinical and imaging features from head to toe. Br J Radiol. 90(1075):20170039, 2017
                                3. Chen YR et al: Distant metastases in a young woman with Stewart-Treves syndrome demonstrated by an FDG-PET/CT scan. Clin Nucl Med. 39(11):975-6, 2014
                                4. Lee R et al: Lymphedema-related angiogenic tumors and other malignancies. Clin Dermatol. 32(5):616-20, 2014
                                5. Tenjarla S et al: Cutaneous angiosarcoma of the foot: a case report and review of the literature. Case Rep Oncol Med. 2014:657876, 2014
                                6. Young RJ et al: Angiogenic growth factor expression in benign and malignant vascular tumours. Exp Mol Pathol. 97(1):148-53, 2014
                                7. Cox CA et al: Angiosarcoma presenting with minor erythema and swelling. Case Rep Ophthalmol. 4(1):59-63, 2013
                                8. Fisher C: Unusual myoid, perivascular, and postradiation lesions, with emphasis on atypical vascular lesion, postradiation cutaneous angiosarcoma, myoepithelial tumors, myopericytoma, and perivascular epithelioid cell tumor. Semin Diagn Pathol. 30(1):73-84, 2013
                                9. Sharma P et al: Detection of recurrent cutaneous angiosarcoma of lower extremity with (18)f-fluorodeoxyglucose positron emission tomography-computed tomography: report of three cases. Indian J Dermatol. 58(3):242, 2013
                                10. Mentzel T et al: Postradiation cutaneous angiosarcoma after treatment of breast carcinoma is characterized by MYC amplification in contrast to atypical vascular lesions after radiotherapy and control cases: clinicopathological, immunohistochemical and molecular analysis of 66 cases. Mod Pathol. 25(1):75-85, 2012
                                11. Chen Y et al: Epithelioid angiosarcoma of bone and soft tissue: a report of seven cases with emphasis on morphologic diversity, immunohistochemical features and clinical outcome. Tumori. 97(5):585-9, 2011
                                12. Hart J et al: Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis. Arch Pathol Lab Med. 135(2):268-72, 2011
                                13. Mentzel T: Sarcomas of the skin in the elderly. Clin Dermatol. 29(1):80-90, 2011
                                14. Sakemi M et al: A case of postirradiation angiosarcoma of the greater omentum with hemorrhage. Clin J Gastroenterol. 4(5):302-6, 2011
                                15. Suchak R et al: Primary cutaneous epithelioid angiosarcoma: a clinicopathologic study of 13 cases of a rare neoplasm occurring outside the setting of conventional angiosarcomas and with predilection for the limbs. Am J Surg Pathol. 35(1):60-9, 2011
                                16. Tokmak E et al: F18-FDG PET/CT scanning in angiosarcoma: report of two cases. Mol Imaging Radionucl Ther. 20(2):63-6, 2011
                                17. Moukaddam H et al: MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol. 38(6):535-47, 2009
                                18. Weiss SW et al: Malignant vascular tumors. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 703-19, 2008
                                19. Kransdorf MJ et al: Vascular and lymphatic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 177-88, 2006
                                20. Weiss SW et al: Angiosarcoma of soft tissue. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 175-7, 2002
                                21. Murphey MD et al: From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics. 15(4):893-917, 1995
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    • Clinical Issues

                                      TERMINOLOGY

                                      • Synonyms

                                        • Older terms (no longer recommended): hemangiosarcoma, malignant hemangioendothelioma, lymphangiosarcoma, hemangioblastoma, malignant angioendothelioma
                                      • Definitions

                                        • Malignant vascular neoplasm with exceptionally poor prognosis
                                      • Risk Factors

                                        • Chronic lymphedema related: Stewart-Treves syndrome
                                        • Radiation induced: > 5 years after XRT
                                        • Toxin exposure: vinyl chloride and thorium dioxide (Thorotrast) exposure → liver angiosarcoma
                                        • Syndrome associated: Maffucci syndrome; neurofibromatosis, Klippel-Trenaunay syndrome
                                        • May occur next to implants (synthetic grafts) or sites of prior trauma
                                        • 85% have no identifiable risk factor

                                      IMAGING

                                      • General Features

                                        • CT Findings

                                          • MR Findings

                                            • Nuclear Medicine Findings

                                              DIFFERENTIAL DIAGNOSIS

                                                PATHOLOGY

                                                • General Features

                                                  • Gross Pathologic & Surgical Features

                                                    • Microscopic Features

                                                      CLINICAL ISSUES

                                                      • Presentation

                                                        • Demographics

                                                          • Natural History & Prognosis

                                                            • Treatment

                                                              Selected References

                                                              1. Weidema ME et al: Targeting angiosarcomas of the soft tissues: a challenging effort in a heterogeneous and rare disease. Crit Rev Oncol Hematol. 138:120-31, 2019
                                                              2. Gaballah AH et al: Angiosarcoma: clinical and imaging features from head to toe. Br J Radiol. 90(1075):20170039, 2017
                                                              3. Chen YR et al: Distant metastases in a young woman with Stewart-Treves syndrome demonstrated by an FDG-PET/CT scan. Clin Nucl Med. 39(11):975-6, 2014
                                                              4. Lee R et al: Lymphedema-related angiogenic tumors and other malignancies. Clin Dermatol. 32(5):616-20, 2014
                                                              5. Tenjarla S et al: Cutaneous angiosarcoma of the foot: a case report and review of the literature. Case Rep Oncol Med. 2014:657876, 2014
                                                              6. Young RJ et al: Angiogenic growth factor expression in benign and malignant vascular tumours. Exp Mol Pathol. 97(1):148-53, 2014
                                                              7. Cox CA et al: Angiosarcoma presenting with minor erythema and swelling. Case Rep Ophthalmol. 4(1):59-63, 2013
                                                              8. Fisher C: Unusual myoid, perivascular, and postradiation lesions, with emphasis on atypical vascular lesion, postradiation cutaneous angiosarcoma, myoepithelial tumors, myopericytoma, and perivascular epithelioid cell tumor. Semin Diagn Pathol. 30(1):73-84, 2013
                                                              9. Sharma P et al: Detection of recurrent cutaneous angiosarcoma of lower extremity with (18)f-fluorodeoxyglucose positron emission tomography-computed tomography: report of three cases. Indian J Dermatol. 58(3):242, 2013
                                                              10. Mentzel T et al: Postradiation cutaneous angiosarcoma after treatment of breast carcinoma is characterized by MYC amplification in contrast to atypical vascular lesions after radiotherapy and control cases: clinicopathological, immunohistochemical and molecular analysis of 66 cases. Mod Pathol. 25(1):75-85, 2012
                                                              11. Chen Y et al: Epithelioid angiosarcoma of bone and soft tissue: a report of seven cases with emphasis on morphologic diversity, immunohistochemical features and clinical outcome. Tumori. 97(5):585-9, 2011
                                                              12. Hart J et al: Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis. Arch Pathol Lab Med. 135(2):268-72, 2011
                                                              13. Mentzel T: Sarcomas of the skin in the elderly. Clin Dermatol. 29(1):80-90, 2011
                                                              14. Sakemi M et al: A case of postirradiation angiosarcoma of the greater omentum with hemorrhage. Clin J Gastroenterol. 4(5):302-6, 2011
                                                              15. Suchak R et al: Primary cutaneous epithelioid angiosarcoma: a clinicopathologic study of 13 cases of a rare neoplasm occurring outside the setting of conventional angiosarcomas and with predilection for the limbs. Am J Surg Pathol. 35(1):60-9, 2011
                                                              16. Tokmak E et al: F18-FDG PET/CT scanning in angiosarcoma: report of two cases. Mol Imaging Radionucl Ther. 20(2):63-6, 2011
                                                              17. Moukaddam H et al: MRI characteristics and classification of peripheral vascular malformations and tumors. Skeletal Radiol. 38(6):535-47, 2009
                                                              18. Weiss SW et al: Malignant vascular tumors. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 703-19, 2008
                                                              19. Kransdorf MJ et al: Vascular and lymphatic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 177-88, 2006
                                                              20. Weiss SW et al: Angiosarcoma of soft tissue. In Fletcher CDM et al: WHO Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 175-7, 2002
                                                              21. Murphey MD et al: From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics. 15(4):893-917, 1995