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Management of Vascular Sarcoma

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      References

        • Stacchiotti S.
        • Frezza A.M.
        • Blay J.Y.
        • et al.
        Ultra-rare sarcomas: a consensus paper from the Connective Tissue Oncology Society community of experts on the incidence threshold and the list of entities.
        Cancer. 2021; 127: 2934-2942
        • de Pinieux G.
        • Karanian M.
        • Le Loarer F.
        • et al.
        Nationwide incidence of sarcomas and connective tissue tumors of intermediate malignancy over four years using an expert pathology review network.
        PLoS One. 2021; 16: e0246958
        • Lau K.
        • Massad M.
        • Pollak C.
        • et al.
        Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement: insights from an internet registry in the study of a rare cancer.
        Chest. 2011; 140: 1312-1318
        • Rosenbaum E.
        • Jadeja B.
        • Xu B.
        • et al.
        Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets.
        Mod Pathol. 2020; 33: 591-602
        • Antonescu C.
        Malignant vascular tumors--an update.
        Mod Pathol. 2014; 27: S30-S38
        • Stacchiotti S.
        • Miah A.B.
        • Frezza A.M.
        • et al.
        Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts.
        ESMO Open. 2021; 6: 100170
        • Shiba S.
        • Imaoka H.
        • Shioji K.
        • et al.
        Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study.
        BMC Cancer. 2018; 18: 993
        • Frezza A.M.
        • Napolitano A.
        • Miceli R.
        • et al.
        Clinical prognostic factors in advanced epithelioid haemangioendothelioma: a retrospective case series analysis within the Italian Rare Cancers Network.
        ESMO Open. 2021; 6: 100083
        • Rossi S.
        • Orvieto E.
        • Furlanetto A.
        • et al.
        Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody.
        Mod Pathol. 2004; 17: 547-552
        • Righi A.
        • Sbaraglia M.
        • Gambarotti M.
        • et al.
        Primary vascular tumors of bone: a monoinstitutional morphologic and molecular analysis of 427 cases with emphasis on epithelioid variants.
        Am J Surg Pathol. 2020; 44: 1192-1203
        • Errani C.
        • Zhang L.
        • Sung Y.S.
        • et al.
        A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites.
        Genes Chromosomes Cancer. 2011; 50: 644-653
        • Suurmeijer A.J.H.
        • Dickson B.C.
        • Swanson D.
        • et al.
        Variant WWTR1 gene fusions in epithelioid hemangioendothelioma-a genetic subset associated with cardiac involvement.
        Genes Chromosomes Cancer. 2020; 59: 389-395
        • Kitaichi M.
        • Nagai S.
        • Nishimura K.
        • et al.
        Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression.
        Eur Respir J. 1998; 12: 89-96
        • Otrock Z.K.
        • Al-Kutoubi A.
        • Kattar M.M.
        • et al.
        Spontaneous complete regression of hepatic epithelioid haemangioendothelioma.
        Lancet Oncol. 2006; 7: 439-441
        • Shibayama T.
        • Makise N.
        • Motoi T.
        • et al.
        Clinicopathologic characterization of epithelioid hemangioendothelioma in a series of 62 cases: a proposal of risk stratification and identification of a synaptophysin-positive aggressive subset.
        Am J Surg Pathol. 2021; 45: 616-626
        • Bagan P.
        • Hassan M.
        • Le Pimpec Barthes F.
        • et al.
        Prognostic factors and surgical indications of pulmonary epithelioid hemangioendothelioma: a review of the literature.
        Ann Thorac Surg. 2006; 82: 2010-2013
        • Epelboym Y.
        • Engelkemier D.R.
        • Thomas-Chausse F.
        • et al.
        Imaging findings in epithelioid hemangioendothelioma.
        Clin Imaging. 2019; 58: 59-65
        • Sardaro A.
        • Bardoscia L.
        • Petruzzelli M.F.
        • et al.
        Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor.
        Oncol Rev. 2014; 8: 259
        • Tong D.
        • Constantinidou A.
        • Engelmann B.
        • et al.
        The role of local therapy in multi-focal epithelioid haemangioendothelioma.
        Anticancer Res. 2019; 39: 4891-4896
        • Yousaf N.
        • Maruzzo M.
        • Judson I.
        • et al.
        Systemic treatment options for epithelioid haemangioendothelioma: the Royal Marsden Hospital experience.
        Anticancer Res. 2015; 35: 473-480
        • Frezza A.M.
        • Ravi V.
        • Lo Vullo S.
        • et al.
        Systemic therapies in advanced epithelioid haemangioendothelioma: a retrospective international case series from the World Sarcoma Network and a review of literature.
        Cancer Med. 2021; 10: 2645-2659
        • Kelly H.
        • O'Neil B.H.
        Response of epithelioid haemangioendothelioma to liposomal doxorubicin.
        Lancet Oncol. 2005; 6: 813-815
        • Grenader T.
        • Vernea F.
        • Reinus C.
        • et al.
        Malignant epithelioid hemangioendothelioma of the liver successfully treated with pegylated liposomal doxorubicin.
        J Clin Oncol. 2011; 29: e722-e724
        • Agulnik M.
        • Yarber J.L.
        • Okuno S.H.
        • et al.
        An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas.
        Ann Oncol. 2013; 24: 257-263
        • Chevreau C.
        • Le Cesne A.
        • Ray-Coquard I.
        • et al.
        Sorafenib in patients with progressive epithelioid hemangioendothelioma: a phase 2 study by the French Sarcoma Group (GSF/GETO).
        Cancer. 2013; 119: 2639-2644
        • Semenisty V.
        • Naroditsky I.
        • Keidar Z.
        • et al.
        Pazopanib for metastatic pulmonary epithelioid hemangioendothelioma-a suitable treatment option: case report and review of anti-angiogenic treatment options.
        BMC Cancer. 2015; 15: 402
        • Kollar A.
        • Jones R.L.
        • Stacchiotti S.
        • et al.
        Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis.
        Acta Oncol. 2017; 56: 88-92
        • Stacchiotti S.
        • Provenzano S.
        • Dagrada G.
        • et al.
        Sirolimus in advanced epithelioid hemangioendothelioma: a retrospective case-series analysis from the Italian Rare Cancer Network database.
        Ann Surg Oncol. 2016; 23: 2735-2744
        • Schuetze S.
        • Ballman K.V.
        • Ganjoo K.N.
        • et al.
        P10015/SARC033: a phase 2 trial of trametinib in patients with advanced epithelioid hemangioendothelioma (EHE).
        J Clin Oncol. 2021; 39: 11503
        • Gronchi A.
        • Miah A.B.
        • Dei Tos A.P.
        • et al.
        Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS clinical practice guidelines for diagnosis, treatment and follow-up∗.
        Ann Oncol. 2021; 32: 1348-1365
        • Zimmermann C.
        • Ryan S.
        • Hannon B.
        • et al.
        Team-based outpatient early palliative care: a complex cancer intervention.
        BMJ Support Palliat Care. 2019; https://doi.org/10.1136/bmjspcare-2019-001903
        • Croteau S.E.
        • Liang M.G.
        • Kozakewich H.P.
        • et al.
        Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals.
        J Pediatr. 2013; 162: 142-147
        • Ji Y.
        • Yang K.
        • Peng S.
        • et al.
        Kaposiform haemangioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon.
        Br J Dermatol. 2018; 179: 457-463
        • Kasabach H.H.
        • Merritt K.K.
        Capillary hemangioma with extensive purpura: report of a case.
        Am J Dis Child. 1940; 59: 1063-1070
        • Lyons L.L.
        • North P.E.
        • Mac-Moune Lai F.
        • et al.
        Kaposiform hemangioendothelioma: a study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma.
        Am J Surg Pathol. 2004; 28: 559-568
        • Drolet B.A.
        • Trenor 3rd, C.C.
        • Brandao L.R.
        • et al.
        Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma.
        J Pediatr. 2013; 163: 285-291
        • Ji Y.
        • Chen S.
        • Xiang B.
        • et al.
        Sirolimus for the treatment of progressive kaposiform hemangioendothelioma: a multicenter retrospective study.
        Int J Cancer. 2017; 141: 848-855
        • Hornick J.L.
        • Fletcher C.D.
        Pseudomyogenic hemangioendothelioma: a distinctive, often multicentric tumor with indolent behavior.
        Am J Surg Pathol. 2011; 35: 190-201
        • Trombetta D.
        • Magnusson L.
        • von Steyern F.V.
        • et al.
        Translocation t(7;19)(q22;q13)-a recurrent chromosome aberration in pseudomyogenic hemangioendothelioma?.
        Cancer Genet. 2011; 204: 211-215
        • Agaram N.P.
        • Zhang L.
        • Cotzia P.
        • et al.
        Expanding the spectrum of genetic alterations in pseudomyogenic hemangioendothelioma with recurrent novel ACTB-FOSB gene fusions.
        Am J Surg Pathol. 2018; 42: 1653-1661
        • Bridge J.A.
        • Sumegi J.
        • Royce T.
        • et al.
        A novel CLTC-FOSB gene fusion in pseudomyogenic hemangioendothelioma of bone.
        Genes Chromosomes Cancer. 2021; 60: 38-42
        • Hakar M.H.
        • White K.
        • Hansford B.G.
        • et al.
        Novel EGFL7-FOSB fusion in pseudomyogenic haemangioendothelioma with widely metastatic disease.
        Histopathology. 2021; 79: 888-891
        • Murshed K.A.
        • Torres-Mora J.
        • ElSayed A.M.
        • et al.
        Pseudomyogenic hemangioendothelioma of bone with rare WWTR1-FOSB fusion gene: case report and literature review.
        Clin Case Rep. 2021; 9: 1494-1499
        • Sugita S.
        • Hirano H.
        • Kikuchi N.
        • et al.
        Diagnostic utility of FOSB immunohistochemistry in pseudomyogenic hemangioendothelioma and its histological mimics.
        Diagn Pathol. 2016; 11: 75
        • Hung Y.P.
        • Fletcher C.D.
        • Hornick J.L.
        FOSB is a useful diagnostic marker for pseudomyogenic hemangioendothelioma.
        Am J Surg Pathol. 2017; 41: 596-606
        • Joseph J.
        • Wang W.L.
        • Patnana M.
        • et al.
        Cytotoxic and targeted therapy for treatment of pseudomyogenic hemangioendothelioma.
        Clin Sarcoma Res. 2015; 5: 22
        • Ozeki M.
        • Nozawa A.
        • Kanda K.
        • et al.
        Everolimus for treatment of pseudomyogenic hemangioendothelioma.
        J Pediatr Hematol Oncol. 2017; 39: e328-e331
        • van I.D.G.P.
        • Sleijfer S.
        • Gelderblom H.
        • et al.
        Telatinib is an effective targeted therapy for pseudomyogenic hemangioendothelioma.
        Clin Cancer Res. 2018; 24: 2678-2687
        • Calonje E.
        • Fletcher C.D.
        • Wilson-Jones E.
        • et al.
        Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases.
        Am J Surg Pathol. 1994; 18: 115-125
        • Colmenero I.
        • Hoeger P.H.
        Vascular tumours in infants. Part II: vascular tumours of intermediate malignancy [corrected] and malignant tumours.
        Br J Dermatol. 2014; 171: 474-484
        • Hirsh A.Z.
        • Yan W.
        • Wei L.
        • et al.
        Unresectable retiform hemangioendothelioma treated with external beam radiation therapy and chemotherapy: a case report and review of the literature.
        Sarcoma. 2010; 2010https://doi.org/10.1155/2010/75624690
        • Nayler S.J.
        • Rubin B.P.
        • Calonje E.
        • et al.
        Composite hemangioendothelioma: a complex, low-grade vascular lesion mimicking angiosarcoma.
        Am J Surg Pathol. 2000; 24: 352-361
        • Shang Leen S.L.
        • Fisher C.
        • Thway K.
        Composite hemangioendothelioma: clinical and histologic features of an enigmatic entity.
        Adv Anat Pathol. 2015; 22: 254-259
        • Perry K.D.
        • Al-Lbraheemi A.
        • Rubin B.P.
        • et al.
        Composite hemangioendothelioma with neuroendocrine marker expression: an aggressive variant.
        Mod Pathol. 2017; 30: 1512
        • Antonescu C.R.
        • Dickson B.C.
        • Sung Y.S.
        • et al.
        Recurrent YAP1 and MAML2 gene rearrangements in retiform and composite hemangioendothelioma.
        Am J Surg Pathol. 2020; 44: 1677-1684
        • Khan J.A.
        • Maki R.G.
        • Ravi V.
        Pathologic angiogenesis of malignant vascular sarcomas: implications for treatment.
        J Clin Oncol. 2018; 36: 194-201
        • Stenback F.
        Cellular injury and cell proliferation in skin carcinogenesis by UV light.
        Oncology. 1975; 31: 61-75
        • Painter C.A.
        • Jain E.
        • Tomson B.N.
        • et al.
        The Angiosarcoma Project: enabling genomic and clinical discoveries in a rare cancer through patient-partnered research.
        Nat Med. 2020; 26: 181-187
        • Huang J.
        • Mackillop W.J.
        Increased risk of soft tissue sarcoma after radiotherapy in women with breast carcinoma.
        Cancer. 2001; 92: 172-180
        • Pereira E.S.
        • Moraes E.T.
        • Siqueira D.M.
        • et al.
        Stewart Treves syndrome.
        An Bras Dermatol. 2015; 90: 229-231
        • Makk L.
        • Creech J.L.
        • Whelan Jr., J.G.
        • et al.
        Liver damage and angiosarcoma in vinyl chloride workers. A systematic detection program.
        JAMA. 1974; 230: 64-68
        • Centeno J.A.
        • Mullick F.G.
        • Martinez L.
        • et al.
        Pathology related to chronic arsenic exposure.
        Environ Health Perspect. 2002; 110: 883-886
        • Daneshmend T.K.
        • Bradfield J.W.
        Hepatic angiosarcoma associated with androgenic-anabolic steroids.
        Lancet. 1979; 2: 1249
        • Ploegmakers M.J.
        • Pruszczynski M.
        • De Rooy J.
        • et al.
        Angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with Klippel-Trenaunay-Weber syndrome.
        Sarcoma. 2005; 9: 137-140
        • Calvete O.
        • Martinez P.
        • Garcia-Pavia P.
        • et al.
        A mutation in the POT1 gene is responsible for cardiac angiosarcoma in TP53-negative Li-Fraumeni-like families.
        Nat Commun. 2015; 6: 8383
        • Shustef E.
        • Kazlouskaya V.
        • Prieto V.G.
        • et al.
        Cutaneous angiosarcoma: a current update.
        J Clin Pathol. 2017; 70: 917-925
        • Ravi V.
        • Patel S.
        Vascular sarcomas.
        Curr Oncol Rep. 2013; 15: 347-355
        • Young R.J.
        • Brown N.J.
        • Reed M.W.
        • et al.
        Angiosarcoma.
        Lancet Oncol. 2010; 11: 983-991
        • Shon W.
        • Billings S.D.
        Cutaneous malignant vascular neoplasms.
        Clin Lab Med. 2017; 37: 633-646
        • Mery C.M.
        • George S.
        • Bertagnolli M.M.
        • et al.
        Secondary sarcomas after radiotherapy for breast cancer: sustained risk and poor survival.
        Cancer. 2009; 115: 4055-4063
        • Chaudhary P.
        • Bhadana U.
        • Singh R.A.
        • et al.
        Primary hepatic angiosarcoma.
        Eur J Surg Oncol. 2015; 41: 1137-1143
        • Look Hong N.J.
        • Pandalai P.K.
        • Hornick J.L.
        • et al.
        Cardiac angiosarcoma management and outcomes: 20-year single-institution experience.
        Ann Surg Oncol. 2012; 19: 2707-2715
        • Li R.
        • Li M.
        • Zhang L.F.
        • et al.
        Clinical characteristics and prognostic factors of primary splenic angiosarcoma: a retrospective clinical analysis from China.
        Cell Physiol Biochem. 2018; 49: 1959-1969
        • Meis-Kindblom J.M.
        • Kindblom L.G.
        Angiosarcoma of soft tissue: a study of 80 cases.
        Am J Surg Pathol. 1998; 22: 683-697
        • Verbeke S.L.
        • Bertoni F.
        • Bacchini P.
        • et al.
        Distinct histological features characterize primary angiosarcoma of bone.
        Histopathology. 2011; 58: 254-264
        • Miettinen M.
        • Lindenmayer A.E.
        • Chaubal A.
        Endothelial cell markers CD31, CD34, and BNH9 antibody to H- and Y-antigens--evaluation of their specificity and sensitivity in the diagnosis of vascular tumors and comparison with von Willebrand factor.
        Mod Pathol. 1994; 7: 82-90
        • Sullivan H.C.
        • Edgar M.A.
        • Cohen C.
        • et al.
        The utility of ERG, CD31 and CD34 in the cytological diagnosis of angiosarcoma: an analysis of 25 cases.
        J Clin Pathol. 2015; 68: 44-50
        • Behjati S.
        • Tarpey P.S.
        • Sheldon H.
        • et al.
        Recurrent PTPRB and PLCG1 mutations in angiosarcoma.
        Nat Genet. 2014; 46: 376-379
        • Italiano A.
        • Chen C.L.
        • Thomas R.
        • et al.
        Alterations of the p53 and PIK3CA/AKT/mTOR pathways in angiosarcomas: a pattern distinct from other sarcomas with complex genomics.
        Cancer. 2012; 118: 5878-5887
        • Naka N.
        • Tomita Y.
        • Nakanishi H.
        • et al.
        Mutations of p53 tumor-suppressor gene in angiosarcoma.
        Int J Cancer. 1997; 71: 952-955
        • Murali R.
        • Chandramohan R.
        • Moller I.
        • et al.
        Targeted massively parallel sequencing of angiosarcomas reveals frequent activation of the mitogen activated protein kinase pathway.
        Oncotarget. 2015; 6: 36041-36052
        • Huang S.C.
        • Zhang L.
        • Sung Y.S.
        • et al.
        Recurrent CIC gene abnormalities in angiosarcomas: a molecular study of 120 cases with concurrent investigation of PLCG1, KDR, MYC, and FLT4 gene alterations.
        Am J Surg Pathol. 2016; 40: 645-655
        • Fernandez A.P.
        • Sun Y.
        • Tubbs R.R.
        • et al.
        FISH for MYC amplification and anti-MYC immunohistochemistry: useful diagnostic tools in the assessment of secondary angiosarcoma and atypical vascular proliferations.
        J Cutan Pathol. 2012; 39: 234-242
        • Manner J.
        • Radlwimmer B.
        • Hohenberger P.
        • et al.
        MYC high level gene amplification is a distinctive feature of angiosarcomas after irradiation or chronic lymphedema.
        Am J Pathol. 2010; 176: 34-39
        • Mentzel T.
        • Schildhaus H.U.
        • Palmedo G.
        • 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. 2012; 25: 75-85
        • Shon W.
        • Sukov W.R.
        • Jenkins S.M.
        • et al.
        MYC amplification and overexpression in primary cutaneous angiosarcoma: a fluorescence in-situ hybridization and immunohistochemical study.
        Mod Pathol. 2014; 27: 509-515
        • Requena C.
        • Rubio L.
        • Lavernia J.
        • et al.
        Immunohistochemical and fluorescence in situ hybridization analysis of MYC in a series of 17 cutaneous angiosarcomas: a single-center study.
        Am J Dermatopathol. 2018; 40: 349-354
        • Bishop A.J.
        • Zheng J.
        • Subramaniam A.
        • et al.
        High terminal hemorrhage risk from cardiac angiosarcoma brain metastases warrants frequent brain imaging and early intervention.
        Int J Radiat Oncol Biol Phys. 2021; 111: e314
        • Mark R.J.
        • Poen J.C.
        • Tran L.M.
        • et al.
        Angiosarcoma. A report of 67 patients and a review of the literature.
        Cancer. 1996; 77: 2400-2406
        • Chen T.W.
        • Burns J.
        • Jones R.L.
        • et al.
        Optimal clinical management and the molecular biology of angiosarcomas.
        Cancers (Basel). 2020; 12https://doi.org/10.3390/cancers1211332191
        • Constantinidou A.
        • Sauve N.
        • Stacchiotti S.
        • et al.
        Evaluation of the use and efficacy of (neo)adjuvant chemotherapy in angiosarcoma: a multicentre study.
        ESMO Open. 2020; 5https://doi.org/10.1136/esmoopen-2020-000787105
        • Ravi V.
        • Wagner M.
        • Chen T.W.-W.
        • et al.
        A phase II study of oraxol in the treatment of unresectable cutaneous angiosarcoma.
        J Clin Oncol. 2020; 38: 11517
        • Guadagnolo B.A.
        • Zagars G.K.
        • Araujo D.
        • et al.
        Outcomes after definitive treatment for cutaneous angiosarcoma of the face and scalp.
        Head Neck. 2011; 33: 661-667
        • Pawlik T.M.
        • Paulino A.F.
        • McGinn C.J.
        • et al.
        Cutaneous angiosarcoma of the scalp: a multidisciplinary approach.
        Cancer. 2003; 98: 1716-1726
        • Young R.J.
        • Natukunda A.
        • Litiere S.
        • et al.
        First-line anthracycline-based chemotherapy for angiosarcoma and other soft tissue sarcoma subtypes: pooled analysis of eleven European Organisation for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group trials.
        Eur J Cancer. 2014; 50: 3178-3186
        • Italiano A.
        • Cioffi A.
        • Penel N.
        • et al.
        Comparison of doxorubicin and weekly paclitaxel efficacy in metastatic angiosarcomas.
        Cancer. 2012; 118: 3330-3336
        • Fury M.G.
        • Antonescu C.R.
        • Van Zee K.J.
        • et al.
        A 14-year retrospective review of angiosarcoma: clinical characteristics, prognostic factors, and treatment outcomes with surgery and chemotherapy.
        Cancer J. 2005; 11: 241-247
        • Penel N.
        • Bui B.N.
        • Bay J.O.
        • et al.
        Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study.
        J Clin Oncol. 2008; 26: 5269-5274
        • Ray-Coquard I.L.
        • Domont J.
        • Tresch-Bruneel E.
        • et al.
        Paclitaxel given once per week with or without bevacizumab in patients with advanced angiosarcoma: a randomized phase II trial.
        J Clin Oncol. 2015; 33: 2797-2802
        • Stacchiotti S.
        • Palassini E.
        • Sanfilippo R.
        • et al.
        Gemcitabine in advanced angiosarcoma: a retrospective case series analysis from the Italian Rare Cancer Network.
        Ann Oncol. 2012; 23: 501-508
        • Ray-Coquard I.
        • Italiano A.
        • Bompas E.
        • et al.
        Sorafenib for patients with advanced angiosarcoma: a phase II trial from the French Sarcoma Group (GSF/GETO).
        Oncologist. 2012; 17: 260-266
        • Maki R.G.
        • D'Adamo D.R.
        • Keohan M.L.
        • et al.
        Phase II study of sorafenib in patients with metastatic or recurrent sarcomas.
        J Clin Oncol. 2009; 27: 3133-3140
        • Jones R.L.
        • Ravi V.
        • Brohl A.S.
        • et al.
        Results of the TAPPAS trial: an adaptive enrichment phase III trial of TRC105 and pazopanib (P) versus pazopanib alone in patients with advanced angiosarcoma (AS).
        Ann Oncol. 2019; 30: v683
        • Jones R.L.
        • Ratain M.J.
        • O'Dwyer P.J.
        • et al.
        Phase II randomised discontinuation trial of brivanib in patients with advanced solid tumours.
        Eur J Cancer. 2019; 120: 132-139
        • Wagner M.J.
        • Othus M.
        • Patel S.P.
        • et al.
        Multicenter phase II trial (SWOG S1609, cohort 51) of ipilimumab and nivolumab in metastatic or unresectable angiosarcoma: a substudy of dual anti-CTLA-4 and anti-PD-1 blockade in rare tumors (DART).
        J Immunother Cancer. 2021; 9https://doi.org/10.1136/jitc-2021-002990109
        • Florou V.
        • Rosenberg A.E.
        • Wieder E.
        • et al.
        Angiosarcoma patients treated with immune checkpoint inhibitors: a case series of seven patients from a single institution.
        J Immunother Cancer. 2019; 7: 213
        • Albores-Saavedra J.
        • Schwartz A.M.
        • Henson D.E.
        • et al.
        Cutaneous angiosarcoma. Analysis of 434 cases from the Surveillance, Epidemiology, and End Results Program, 1973-2007.
        Ann Diagn Pathol. 2011; 15: 93-97
        • Shin J.Y.
        • Roh S.G.
        • Lee N.H.
        • et al.
        Predisposing factors for poor prognosis of angiosarcoma of the scalp and face: systematic review and meta-analysis.
        Head Neck. 2017; 39: 380-386
        • Weidema M.E.
        • Flucke U.E.
        • van der Graaf W.T.A.
        • et al.
        Prognostic factors in a large nationwide cohort of histologically confirmed primary and secondary angiosarcomas.
        Cancers (Basel). 2019; 11https://doi.org/10.3390/cancers11111780150
        • Bui N.
        • Kamat N.
        • Ravi V.
        • et al.
        A multicenter phase II study of Q3 week or weekly paclitaxel in combination with bevacizumab for the treatment of metastatic or unresectable angiosarcoma.
        Rare Tumors. 2018; 10 (2036361318771771)
        • Fata F.
        • O'Reilly E.
        • Ilson D.
        • et al.
        Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face.
        Cancer. 1999; 86: 2034-2037
        • Apice G.
        • Pizzolorusso A.
        • Di Maio M.
        • et al.
        Confirmed activity and tolerability of weekly paclitaxel in the treatment of advanced angiosarcoma.
        Sarcoma. 2016; 2016: 6862090
        • Nagano T.
        • Yamada Y.
        • Ikeda T.
        • et al.
        Docetaxel: a therapeutic option in the treatment of cutaneous angiosarcoma: report of 9 patients.
        Cancer. 2007; 110: 648-651
        • Dickson M.A.
        • D'Adamo D.R.
        • Keohan M.L.
        • et al.
        Phase II trial of gemcitabine and docetaxel with bevacizumab in soft tissue sarcoma.
        Sarcoma. 2015; 2015: 532478
        • Kim J.H.
        • Park H.S.
        • Heo S.J.
        • et al.
        Differences in the efficacies of pazopanib and gemcitabine/docetaxel as second-line treatments for metastatic soft tissue sarcoma.
        Oncology. 2019; 96: 59-69
        • Mehren M.V.
        • Litwin S.
        • Ravi V.
        • et al.
        Multicenter phase II trial of pazopanib (P) in patients with angiosarcoma (AS).
        J Clin Oncol. 2019; 37: 11039
      1. Kaposi. Idiopathisches multiples Pigmentsarkom der Haut.
        Archiv für Dermatologie und Syphilis. 1872; 4: 265-273
        • Chang Y.
        • Cesarman E.
        • Pessin M.S.
        • et al.
        Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi's sarcoma.
        Science. 1994; 266: 1865-1869
        • Flore O.
        • Rafii S.
        • Ely S.
        • et al.
        Transformation of primary human endothelial cells by Kaposi's sarcoma-associated herpesvirus.
        Nature. 1998; 394: 588-592
        • Cesarman E.
        • Damania B.
        • Krown S.E.
        • et al.
        Kaposi sarcoma.
        Nat Rev Dis Primers. 2019; 5: 9
        • Iscovich J.
        • Boffetta P.
        • Franceschi S.
        • et al.
        Classic Kaposi sarcoma: epidemiology and risk factors.
        Cancer. 2000; 88: 500-517
        • Cook-Mozaffari P.
        • Newton R.
        • Beral V.
        • et al.
        The geographical distribution of Kaposi's sarcoma and of lymphomas in Africa before the AIDS epidemic.
        Br J Cancer. 1998; 78: 1521-1528
        • Parkin D.M.
        • Sitas F.
        • Chirenje M.
        • et al.
        Part I: cancer in indigenous Africans--burden, distribution, and trends.
        Lancet Oncol. 2008; 9: 683-692
        • Beral V.
        • Peterman T.A.
        • Berkelman R.L.
        • et al.
        Kaposi's sarcoma among persons with AIDS: a sexually transmitted infection?.
        Lancet. 1990; 335: 123-128
        • Armstrong A.W.
        • Lam K.H.
        • Chase E.P.
        Epidemiology of classic and AIDS-related Kaposi's sarcoma in the USA: incidence, survival, and geographical distribution from 1975 to 2005.
        Epidemiol Infect. 2013; 141: 200-206
        • Mendez J.C.
        • Paya C.V.
        Kaposi's sarcoma and transplantation.
        Herpes. 2000; 7: 18-23
        • Grulich A.E.
        • Vajdic C.M.
        The epidemiology of cancers in human immunodeficiency virus infection and after organ transplantation.
        Semin Oncol. 2015; 42: 247-257
        • Denis D.
        • Seta V.
        • Regnier-Rosencher E.
        • et al.
        A fifth subtype of Kaposi's sarcoma, classic Kaposi's sarcoma in men who have sex with men: a cohort study in Paris.
        J Eur Acad Dermatol Venereol. 2018; 32: 1377-1384
        • Amerson E.
        • Woodruff C.M.
        • Forrestel A.
        • et al.
        Accuracy of clinical suspicion and pathologic diagnosis of Kaposi sarcoma in East Africa.
        J Acquir Immune Defic Syndr. 2016; 71: 295-301
        • Delyon J.
        • Rabate C.
        • Euvrard S.
        • et al.
        Management of Kaposi sarcoma after solid organ transplantation: a European retrospective study.
        J Am Acad Dermatol. 2019; 81: 448-455
        • Letang E.
        • Lewis J.J.
        • Bower M.
        • et al.
        Immune reconstitution inflammatory syndrome associated with Kaposi sarcoma: higher incidence and mortality in Africa than in the UK.
        AIDS. 2013; 27: 1603-1613
        • Bower M.
        • Dalla Pria A.
        • Coyle C.
        • et al.
        Prospective stage-stratified approach to AIDS-related Kaposi's sarcoma.
        J Clin Oncol. 2014; 32: 409-414
        • Lebbe C.
        • Garbe C.
        • Stratigos A.J.
        • et al.
        Diagnosis and treatment of Kaposi's sarcoma: European consensus-based interdisciplinary guideline (EDF/EADO/EORTC).
        Eur J Cancer. 2019; 114: 117-127
        • Northfelt D.W.
        • Dezube B.J.
        • Thommes J.A.
        • et al.
        Pegylated-liposomal doxorubicin versus doxorubicin, bleomycin, and vincristine in the treatment of AIDS-related Kaposi's sarcoma: results of a randomized phase III clinical trial.
        J Clin Oncol. 1998; 16: 2445-2451
        • Stewart S.
        • Jablonowski H.
        • Goebel F.D.
        • et al.
        Randomized comparative trial of pegylated liposomal doxorubicin versus bleomycin and vincristine in the treatment of AIDS-related Kaposi's sarcoma. International Pegylated Liposomal Doxorubicin Study Group.
        J Clin Oncol. 1998; 16: 683-691
        • Gill P.S.
        • Tulpule A.
        • Espina B.M.
        • et al.
        Paclitaxel is safe and effective in the treatment of advanced AIDS-related Kaposi's sarcoma.
        J Clin Oncol. 1999; 17: 1876-1883
        • Cianfrocca M.
        • Lee S.
        • Von Roenn J.
        • et al.
        Randomized trial of paclitaxel versus pegylated liposomal doxorubicin for advanced human immunodeficiency virus-associated Kaposi sarcoma: evidence of symptom palliation from chemotherapy.
        Cancer. 2010; 116: 3969-3977
        • Krown S.E.
        • Moser C.B.
        • MacPhail P.
        • et al.
        Treatment of advanced AIDS-associated Kaposi sarcoma in resource-limited settings: a three-arm, open-label, randomised, non-inferiority trial.
        Lancet. 2020; 395: 1195-1207
        • Uldrick T.S.
        • Wyvill K.M.
        • Kumar P.
        • et al.
        Phase II study of bevacizumab in patients with HIV-associated Kaposi's sarcoma receiving antiretroviral therapy.
        J Clin Oncol. 2012; 30: 1476-1483
        • Polizzotto M.N.
        • Uldrick T.S.
        • Wyvill K.M.
        • et al.
        Pomalidomide for symptomatic Kaposi's sarcoma in people with and without hiv infection: a phase I/II study.
        J Clin Oncol. 2016; 34: 4125-4131
        • Koon H.B.
        • Krown S.E.
        • Lee J.Y.
        • et al.
        Phase II trial of imatinib in AIDS-associated Kaposi's sarcoma: AIDS Malignancy Consortium Protocol 042.
        J Clin Oncol. 2014; 32: 402-408
        • Evans S.R.
        • Krown S.E.
        • Testa M.A.
        • et al.
        Phase II evaluation of low-dose oral etoposide for the treatment of relapsed or progressive AIDS-related Kaposi's sarcoma: an AIDS Clinical Trials Group clinical study.
        J Clin Oncol. 2002; 20: 3236-3241
        • Reid E.G.
        • Suazo A.
        • Lensing S.Y.
        • et al.
        Pilot Trial AMC-063: safety and efficacy of bortezomib in AIDS-associated Kaposi sarcoma.
        Clin Cancer Res. 2020; 26: 558-565
        • Garcia A.
        • Nelson K.
        • Patel V.
        Emerging therapies for rare cutaneous cancers: a systematic review.
        Cancer Treat Rev. 2021; 100: 102266
        • Galanina N.
        • Goodman A.M.
        • Cohen P.R.
        • et al.
        Successful treatment of HIV-associated Kaposi sarcoma with immune checkpoint blockade.
        Cancer Immunol Res. 2018; 6: 1129-1135
        • Delyon J.
        • Bizot A.
        • Battistella M.
        • et al.
        PD-1 blockade with nivolumab in endemic Kaposi sarcoma.
        Ann Oncol. 2018; 29: 1067-1069
        • Uldrick T.S.
        • Goncalves P.H.
        • Abdul-Hay M.
        • et al.
        Assessment of the safety of pembrolizumab in patients with HIV and advanced cancer-a phase 1 study.
        JAMA Oncol. 2019; 5: 1332-1339
        • Delyon J.
        • Resche-Rigon M.
        • Renaud M.
        • et al.
        PD1 blockade with pembrolizumab in classic and endemic Kaposi sarcoma: a multicenter phase II study.
        Ann Oncol. 2020; 31: S732
        • Franceschi S.
        • Arniani S.
        • Balzi D.
        • et al.
        Survival of classic Kaposi's sarcoma and risk of second cancer.
        Br J Cancer. 1996; 74: 1812-1814
        • Benajiba L.
        • Lambert J.
        • La Selva R.
        • et al.
        Systemic treatment initiation in classical and endemic Kaposi's sarcoma: risk factors and global multi-state modelling in a monocentric cohort study.
        Cancers (Basel). 2021; 13https://doi.org/10.3390/cancers13112519
        • Krown S.E.
        • Testa M.A.
        • Huang J.
        AIDS-related Kaposi's sarcoma: prospective validation of the AIDS Clinical Trials Group staging classification. AIDS Clinical Trials Group Oncology Committee.
        J Clin Oncol. 1997; 15: 3085-3092
        • Nasti G.
        • Talamini R.
        • Antinori A.
        • et al.
        AIDS-related Kaposi's sarcoma: evaluation of potential new prognostic factors and assessment of the AIDS Clinical Trial Group Staging System in the Haart Era--the Italian Cooperative Group on AIDS and Tumors and the Italian Cohort of Patients Naive From Antiretrovirals.
        J Clin Oncol. 2003; 21: 2876-2882
        • Lodi S.
        • Guiguet M.
        • Costagliola D.
        • et al.
        Kaposi sarcoma incidence and survival among HIV-infected homosexual men after HIV seroconversion.
        J Natl Cancer Inst. 2010; 102: 784-792
        • Einollahi B.
        • Lessan-Pezeshki M.
        • Nourbala M.H.
        • et al.
        Kaposi's sarcoma following living donor kidney transplantation: review of 7,939 recipients.
        Int Urol Nephrol. 2009; 41: 679-685
        • Taborelli M.
        • Piselli P.
        • Ettorre G.M.
        • et al.
        Survival after the diagnosis of de novo malignancy in liver transplant recipients.
        Int J Cancer. 2019; 144: 232-239