Research Article| Volume 49, ISSUE 1, P263-270, January 2023

Evaluation of prognostic factors and treatment options for renal angiosarcoma: A retrospective analysis of 113 reported cases

Published:September 07, 2022DOI:



      Primary renal angiosarcoma (PRA) is an extremely rare and aggressive neoplasm. Indeed, due to its rarity, established clinical guidelines for PRA have not yet been established. In this study, we attempted to investigate its prognostic factors and treatment options.


      We systematically searched for articles describing PRA that had been published up until December 2021. The final cohort included 113 patients in 103 articles. The starting point of this study was the time of diagnosis and the end point was the time of recurrence and disease-specific mortality.


      Metastasis at diagnosis was associated with poorer disease-specific survival (DSS) (p = 0.001). Tumors of more than 5 cm had poorer DSS than tumors of 5 cm or less (p < 0.001). Multivariate analysis demonstrated that primary metastatic status, and tumor size were independent prognostic factors. In cases of localized PRA, tumor sizes exceeding 5 cm had also prognostic significance for recurrence-free survival (RFS) and DSS. Surgical margins, postoperative radiation, and postoperative systemic therapy were not associated with prognoses. However, in a subgroup analysis of tumors exceeding 5 cm, postoperative radiation therapy improved RFS and DSS (p = 0.022 and p = 0.031, respectively). In cases of metastatic PRA, systemic therapy improved DSS (p < 0.001).


      We identified several prognostic factors for PRA. Among them, primary metastatic status and tumor size exceeding 5 cm were selected as independent prognostic factors. Postoperative radiation therapy for large, localized PRA and systemic therapy for recurrent and metastatic PRA might be a treatment option.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to European Journal of Surgical Oncology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Young R.J.
        • Brown N.J.
        • Reed M.W.
        • Hughes D.
        • Woll P.J.
        Angiosarcoma. Lancet Oncol. 2010 Oct; 11: 983-991
        • Iacovelli R.
        • Orlando V.
        • Palazzo A.
        • Cortesi E.
        Clinical and pathological features of primary renal angiosarcoma.
        Can Urol Assoc J. 2014 Mar; 8: E223-E226
        • Mark R.J.
        • Poen J.C.
        • Tran L.M.
        • Fu Y.S.
        • Juillard G.F.
        Angiosarcoma. A report of 67 patients and a review of the literature.
        Cancer. 1996 Jun 1; 77: 2400-2406
        • Abraham J.A.
        • Hornicek F.J.
        • Kaufman A.M.
        • Harmon D.C.
        • Springfield D.S.
        • Raskin K.A.
        • et al.
        Treatment and outcome of 82 patients with angiosarcoma.
        Ann Surg Oncol. 2007 Jun; 14: 1953-1967
        • Fayette J.
        • Martin E.
        • Piperno-Neumann S.
        • Le Cesne A.
        • Robert C.
        • Bonvalot S.
        • et al.
        Angiosarcomas, a heterogeneous group of sarcomas with specific behavior depending on primary site: a retrospective study of 161 cases.
        Ann Oncol. 2007 Dec; 18: 2030-2036
        • Omiyale A.O.
        • Carton J.
        Clinical and pathologic features of primary angiosarcoma of the kidney.
        Curr Urol Rep. 2018 Jan 31; 19: 4
        • Zhang H.M.
        • Yan Y.
        • Luo M.
        • Xu Y.F.
        • Peng B.
        • Zheng J.H.
        Primary angiosarcoma of the kidney: case analysis and literature review.
        Int J Clin Exp Pathol. 2014; 7: 3555-3562
        • Pawlik T.M.
        • Paulino A.F.
        • McGinn C.J.
        • Baker L.H.
        • Cohen D.S.
        • Morris J.S.
        • et al.
        Cutaneous angiosarcoma of the scalp: a multidisciplinary approach.
        Cancer. 2003 Oct 15; 98: 1716-1726
        • Patel S.H.
        • Hayden R.E.
        • Hinni M.L.
        • Wong W.W.
        • Foote R.L.
        • Milani S.
        • et al.
        Angiosarcoma of the scalp and face: the Mayo Clinic experience.
        JAMA Otolaryngol Head Neck Surg. 2015 Apr; 141: 335-340
        • Prince C.L.
        Primary angio-endothelioma of the kidney: report of a case and brief review.
        J Urol. 1942; 47: 787-792
        • Lahat G.
        • Dhuka A.R.
        • Hallevi H.
        • Xiao L.
        • Zou C.
        • Smith K.D.
        • et al.
        Angiosarcoma: clinical and molecular insights.
        Ann Surg. 2010 Jun; 251: 1098-1106
        • Lindet C.
        • Neuville A.
        • Penel N.
        • Lae M.
        • Michels J.J.
        • Trassard M.
        • et al.
        Localised angiosarcomas: the identification of prognostic factors and analysis of treatment impact. A retrospective analysis from the French Sarcoma Group (GSF/GETO).
        Eur J Cancer. 2013 Jan; 49: 369-376
        • Clark M.A.
        • Fisher C.
        • Judson I.
        • Thomas J.M.
        Soft-tissue sarcomas in adults.
        N Engl J Med. 2005 Aug 18; 353: 701-711
        • Zenico T.
        • Saccomanni M.
        • Salomone U.
        • Bercovich E.
        Primary renal angiosarcoma: case report and review of world literature.
        Tumori. 2011 Jul-Aug; 97: 6-9
        • Young R.J.
        • Natukunda A.
        • Litière S.
        • Woll P.J.
        • Wardelmann E.
        • van der Graaf W.T.
        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 Dec; 50: 3178-3186
        • Penel N.
        • Bui B.N.
        • Bay J.O.
        • Cupissol D.
        • Ray-Coquard I.
        • Piperno-Neumann S.
        • et al.
        Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study.
        J Clin Oncol. 2008 Nov 10; 26: 5269-5274
        • Boni A.
        • Cochetti G.
        • Sidoni A.
        • Bellezza G.
        • Lepri E.
        • Giglio A.
        • et al.
        Primary angiosarcoma of the kidney: case report and comprehensive literature review.
        Open Med. 2019; 14: 443-455
        • Stacchiotti S.
        • Palassini E.
        • Sanfilippo R.
        • Vincenzi B.
        • Arena M.G.
        • Bochicchio A.M.
        • et al.
        Gemcitabine in advanced angiosarcoma: a retrospective case series analysis from the Italian Rare Cancer Network.
        Ann Oncol. 2012 Feb; 23: 501-508
        • Zietz C.
        • Rössle M.
        • Haas C.
        • Sendelhofert A.
        • Hirschmann A.
        • Stürzl M.
        • et al.
        MDM-2 oncoprotein overexpression, p53 gene mutation, and VEGF up-regulation in angiosarcomas.
        Am J Pathol. 1998 Nov; 153: 1425-1433
        • Maki R.G.
        • D'Adamo D.R.
        • Keohan M.L.
        • Saulle M.
        • Schuetze S.M.
        • Undevia S.D.
        • et al.
        Phase II study of sorafenib in patients with metastatic or recurrent sarcomas.
        J Clin Oncol. 2009 Jul 1; 27: 3133-3140
        • Kollár A.
        • Jones R.L.
        • Stacchiotti S.
        • Gelderblom H.
        • Guida M.
        • Grignani G.
        • et al.
        Pazopanib in advanced vascular sarcomas: an EORTC soft tissue and bone sarcoma group (STBSG) retrospective analysis.
        Acta Oncol. 2017 Jan; 56: 88-92
        • Agulnik M.
        • Schulte B.
        • Robinson S.
        • Hirbe A.C.
        • Kozak K.
        • Chawla S.P.
        • et al.
        An open-label single-arm phase II study of regorafenib for the treatment of angiosarcoma.
        Eur J Cancer. 2021 Sep; 154: 201-208