Skip to main content

Advertisement

SpringerLink
Log in

Clinical and Pathologic Features of Primary Angiosarcoma of the Kidney

  • Kidney Diseases (G Ciancio, Section Editor)
  • Published:
Current Urology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Primary angiosarcoma of the kidney is extremely rare; hence, relatively little is known regarding its clinicopathologic features and prognosis. Herein, we review the literature on primary renal angiosarcoma with emphasis on the clinical and pathologic features.

Recent Findings

Approximately 64 cases have been reported in the literature, and most cases occur in the 6th–7th decade with a strong male predominance. The aetiology is unknown. Patients present with flank pain, haematuria, abdominal mass and weight loss. A considerable number of patients develop metastatic disease at diagnosis or shortly afterwards. Grossly, the tumour comprises ill-defined haemorrhagic spongy masses often with necrosis. Microscopically, the tumour is composed of anastomosing capillary-sized vessels which are lined by malignant endothelial cells. The mainstay of treatment is surgery followed by radiation therapy with or without chemotherapy.

Summary

Renal angiosarcomas are highly aggressive tumours with dismal outcome, and they must be distinguished from morphologically similar lesions of the kidney.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: •• Of major importance

  1. •• Omiyale AO. Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases. Transl Androl Urol. 2015;4:464–73. Comprehensive review of the clinicopathologic features of primary angiosarcoma of the kidney.

    PubMed  PubMed Central  Google Scholar 

  2. Chaabouni A, Rebai N, Chabchoub K, Foratid M, Bouacida M, Slimen MH, et al. Primary renal angiosarcoma: case report and literature review. Can Urol Assoc J. 2013;7(5-6):E430–2. https://doi.org/10.5489/cuaj.1396.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Singh C, Xie L, Schmechel SC, Manivel JC, Pambuccian SE. Epithelioid angiosarcoma of the kidney: a diagnostic dilemma in fine-needle aspiration cytology. Diagn Cytopathol. 2012;40(Suppl 2):E131–9. https://doi.org/10.1002/dc.21762.

    Article  PubMed  Google Scholar 

  4. Brown JG, Folpe AL, Rao P, Lazar AJ, Paner GP, Gupta R, et al. Primary vascular tumours and tumor-like lesions of the kidney: a clinicopathologic analysis of 25 cases. Am J Surg Pathol. 2010;34(7):942–9. https://doi.org/10.1097/PAS.0b013e3181e4f32a.

    Article  PubMed  Google Scholar 

  5. Prince CL. Primary angio-endothelioma of the kidney: report of a case and brief review. J Urol. 1942;47(6):787–9. https://doi.org/10.1016/S0022-5347(17)70875-0.

    Article  Google Scholar 

  6. Juan CJ, Yu CH, Hsu HH, Chian CP, Huang GS, Fan HC, et al. Visceral and non-visceral angiosarcoma: imaging features and clinical correlation. Chin J Radiol. 2000;25:183–9.

    Google Scholar 

  7. Testa G, Talamona G, Tufano A, Marino-Marsilia G. Primary renal angiosarcoma: a case report. Acta Urologica Italica. 1998;12:225–7.

    Google Scholar 

  8. Xuan Y. Primary renal angiosarcoma: one case report and literature review. Chin J Clin Oncol. 2008;5(3):229–30. https://doi.org/10.1007/s11805-008-0229-6.

    Article  Google Scholar 

  9. Sesar P, Ulamec M, Šoša D, Trnski D, Tomas D. Primary renal angiosarcoma. Acta Clin Croat. 2012;51:182.

    Google Scholar 

  10. Zenico T, Saccomanni M, Salomone U, Bercovich E. Primary renal angiosarcoma: case report and review of world literature. Tumori. 2011;97:e6–9.

    Article  PubMed  Google Scholar 

  11. Aksoy Y, Gürsan N, Ozbey I. Spontaneous rupture of a renal angiosarcoma. Urol Int. 2002;68(1):60–2. https://doi.org/10.1159/000048419.

    Article  PubMed  Google Scholar 

  12. Detorakis EE, Chryssou E, Raissaki M, Androulidakis E, Heretis I, Haniotis V, et al. Primary renal angiosarcoma: radiologic-pathologic correlation and literature review. Tumori. 2013;99:e111–6.

    Article  PubMed  Google Scholar 

  13. Liu H, Huang X, Chen H, Wang X, Chen L. Epithelioid angiosarcoma of the kidney: a case report and literature review. Oncol Lett. 2014;8(3):1155–8. https://doi.org/10.3892/ol.2014.2292.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Fukunaga M. Angiosarcoma of the kidney with minute clear cell carcinomas: a case report. Pathol Res Pract. 2009;205(5):347–51. https://doi.org/10.1016/j.prp.2008.11.005.

    Article  PubMed  Google Scholar 

  15. Kern SB, Gott L, Faulkner J 2nd. Occurrence of primary renal angiosarcoma in brothers. Arch Pathol Lab Med. 1995;119(1):75–8.

    CAS  PubMed  Google Scholar 

  16. Akkad T, Tsankov A, Pelzer A, Peschel R, Bartsch G, Steiner H. Early diagnosis and straightforward surgery of an asymptomatic primary angiosarcoma of the kidney led to long-term survival. Int J Urol. 2006;13(8):1112–4. https://doi.org/10.1111/j.1442-2042.2006.01490.x.

    Article  PubMed  Google Scholar 

  17. Yamamoto Y, Izaki H, Harada A, Taue R, Kishimoto T, Tanimoto S, et al. A case of renal capsular hemangiosarcoma. Hinyokika Kiyo. 2006;52(3):215–7.

    PubMed  Google Scholar 

  18. •• Lacovelli R, Orlando V, Palazzo A, Cortesi E. Clinical and pathological features of primary renal angiosarcoma. Can Urol Assoc J. 2014;8(3–4):E223–6. Recent review of primary renal angiosarcoma.

    Article  Google Scholar 

  19. Eble JN, Sauter G, Epstein JI, Sesterhenn IA. World Health Organization classification of tumours. In: Pathology and genetics of tumours of the urinary system and male genital organs. Lyon: IARC Press; 2004.

    Google Scholar 

  20. Penel N, Marréaud S, Robin YM, Hohenberger P. Angiosarcoma: state of the art and perspectives. Crit Rev Oncol Hematol. 2011;80(2):257–63. https://doi.org/10.1016/j.critrevonc.2010.10.007.

    Article  PubMed  Google Scholar 

  21. Yau T, Leong CH, Chan WK, Chan JK, Liang RHS, Epstein RJ. A case of mixed adult Wilms’ tumour and angiosarcoma responsive to carboplatin, etoposide and vincristine (CEO). Cancer Chemother Pharmacol. 2008;61(4):717–20. https://doi.org/10.1007/s00280-007-0529-1.

    Article  PubMed  Google Scholar 

  22. Sabharwal S, John NT, Kumar RM, Kekre NS. Primary renal angiosarcoma. Indian J Urol. 2013;29(2):145–7. https://doi.org/10.4103/0970-1591.114040.

    Article  PubMed  PubMed Central  Google Scholar 

  23. Fletcher CDM, Bridge JA, Hogendoorn PCW, Mertens F. World Health Organization classification of tumours of soft tissue and bone. 4th ed. Lyon: IARC; 2013.

    Google Scholar 

  24. Antonescu CR, Yoshida A, Guo T, Chang NE, Zhang L, Agaram NP, et al. KDR activating mutations in human angiosarcomas are sensitive to specific kinase inhibitors. Cancer Res. 2009;69(18):7175–9. https://doi.org/10.1158/0008-5472.CAN-09-2068.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Rathmell WK, Acs G, Simon MC, Vaughn DJ. HIF transcription factor expression and induction of hypoxic response genes in a retroperitoneal angiosarcoma. Anticancer Res. 2004;24(1):167–9.

    CAS  PubMed  Google Scholar 

  26. Guo T, Zhang L, Chang NE, Singer S, Maki RG, Antonescu CR. Consistent MYC and FLT4 gene amplification in radiation-induced angiosarcoma but not in other radiation-associated atypical vascular lesions. Genes Chromosom Cancer. 2011;50(1):25–33. https://doi.org/10.1002/gcc.20827.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. •• Behjati S, Tarpey PS, Sheldon H, Martincorena I, Loo PV, Gundem G, et al. Recurrent PTPRB and PLCG1 mutations in angiosarcoma. Nat Genet. 2014;46(4):376–9. https://doi.org/10.1038/ng.2921. Study identified novel recurrent mutations in angiogenesis signalling genes in angiosarcoma.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Dangoor A, Seddon B, Gerrand C, Grimer R, Whelan J, Judson I. UK guidelines for the management of soft tissue sarcomas. Clin Sarcoma Res. 2016;6(20) https://doi.org/10.1186/s13569-016-0060-4.

  29. ESMO/European Sarcoma Network Working Group. Soft tissue and visceral sarcomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2014;25(Suppl 3):iii102–12.

    Article  Google Scholar 

  30. Mark RJ, Poen JC, Tran LM, Fu YS, Julliard GF. Angiosarcoma. A report of 67 patients and a review of the literature. Cancer. 1996;77(11):2400–6. https://doi.org/10.1002/(SICI)1097-0142(19960601)77:11<2400::AID-CNCR32>3.0.CO;2-Z.

    Article  CAS  PubMed  Google Scholar 

  31. Pawlik TM, Paulino AF, McGinn CJ, Baker LH, Cohen DS, Morris JS, et al. Cutaneous angiosarcoma of the scalp: a multidisciplinary approach. Cancer. 2003;98(8):1716–26. https://doi.org/10.1002/cncr.11667.

    Article  PubMed  Google Scholar 

  32. Yoshida K, Ito F, Nakazawa H, Maeda Y, Tomoe H, Aiba M. A case of primary renal angiosarcoma. Rare Tumors. 2009;1:e28.

    Article  PubMed  PubMed Central  Google Scholar 

  33. Celebi F, Pilanci KN, Saglam S, Balci NC. Primary renal angiosarcoma with progressive clinical course despite surgical and adjuvant treatment: a case report. Oncol Lett. 2015;9(4):1937–9. https://doi.org/10.3892/ol.2015.2902.

    Article  PubMed  PubMed Central  Google Scholar 

  34. Omiyale AO. Anastomosing haemangioma of the kidney: a literature review of a rare morphological variant of haemangioma. Ann Transl Med. 2015;3:151.

    PubMed  PubMed Central  Google Scholar 

  35. Omiyale AO, Golash A, Mann A, Kyriakidis D, Kalyanasundaram K. Anastomosing haemangioma of the kidney involving a segmental branch of the renal vein. Case Rep Surg. 2015;2015:927286.

    PubMed  PubMed Central  Google Scholar 

  36. Viswanathan S, Desai SB, Prabhu SR, Amin MB. Squamous differentiation in a sarcomatoid chromophobe renal cell carcinoma: an unusual case report with review of the literature. Arch Pathol Lab Med. 2008;132(10):1672–4.

  37. Lahat G, Dhuka AR, Hallevi H, Xiao L, Zou C, Smith KD, et al. Angiosarcoma clinical and molecular insights. Ann Surg. 2010;251(6):1098–106. https://doi.org/10.1097/SLA.0b013e3181dbb75a.

    Article  PubMed  Google Scholar 

  38. Meis-Kindblom J, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol. 1998;22(6):683–97. https://doi.org/10.1097/00000478-199806000-00005.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London, UK

    Ayo O. Omiyale & James Carton

Authors
  1. Ayo O. Omiyale
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. James Carton
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Ayo O. Omiyale.

Ethics declarations

Conflict of Interest

Ayo O. Omiyale and James Carton each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

This article is part of the Topical Collection on Kidney Diseases

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Omiyale, A.O., Carton, J. Clinical and Pathologic Features of Primary Angiosarcoma of the Kidney. Curr Urol Rep 19, 4 (2018). https://doi.org/10.1007/s11934-018-0755-6

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11934-018-0755-6

Keywords

Search

Navigation