Skip to main content
SpringerLink
Log in

Epithelioid Hemangioendothelioma

  • Chapter
  • First Online:
Rare Sarcomas

  • 355 Accesses

Abstract

This chapter summarizes the best of knowledge about pathological features, clinical and radiological presentation and outcome of both children and adult epithelioid hemangioendothelioma (EHE). Most of EHE display an indolent course in the initial part of their natural history. However, there is no consensus on treatment in case of progressive and symptomatic EHE. International efforts are urgently needed to improve the management of this very rare disease.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 139.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Dail DH, Liebow AA. Intravascular bronchioloalveolar tumor. Am J Pathol. 1975;78:6a–7a.

    Google Scholar 

  2. Weiss SW, Ishak KG, Enzinger FM, et al. Epithelioid hemangioendothelioma and related lesions. Semin Diagn Pathol. 1986;3:259–87.

    Google Scholar 

  3. Corrin B, Manners B, Weaver L, et al. Histogenesis of the so-called ‘intravascular bronchioloalveolar tumor’. J Pathol. 1979;123:163–7.

    Google Scholar 

  4. Weldon-Linne CM, Victor TA, Fry WA, et al. Angiogenic nature of the intravascular bronchioloalveolar tumor of the lung: an electron microscopic study. Arch Pathol. 1981;105:174–9.

    Google Scholar 

  5. Errani C, Zhang L, Antonescu CR, et al. A novel WWTR1-CAMTA1 gene fusion is a consistent abnormality in epithelioid hemangioendothelioma of different anatomic sites. Genes Chromosomes Cancer. 2011;50:644–53.

    Google Scholar 

  6. Fletcher CDM, Bridge JA, Hogendoorn P, Mertens F. WHO classification of tumours of soft tissue and bone, vol. 5. 4th ed. Geneva: WHO; 2013.

    Google Scholar 

  7. Flucke U, Vogels RJ, de Saint Aubain Somerhausen N, Creytens DH, Riedl RG, van Gorp JM, Milne AN, Huysentruyt CJ, Verdijk MA, van Asseldonk MM, Suurmeijer AJ, Bras J, Palmedo G, et al. Epithelioid Hemangioendothelioma: clinicopathologic, immunohistochemical, and molecular genetic analysis of 39 cases. Diagn Pathol. 2014;9:131.

    Google Scholar 

  8. Mentzel T, Beham A, Calonje E, Katenkamp D, Fletcher CD. Epithelioid hemangioendothelioma of skin and soft tissues: clinicopathologic and immunohistochemical study of 30 cases. Am J Surg Pathol. 1997;21(4):363–74.

    Google Scholar 

  9. Shibuya R, Matsuyama A, Shiba E, Harada H, Yabuki K, Hisaoka M. CAMTA1 is a useful immunohistochemical marker for diagnosing epithelioid haemangioendothelioma. Histopathology. 2015;67(6):827–35.

    Google Scholar 

  10. Doyle LA, Fletcher CDM, Hornick JL. Nuclear expression of CAMTA1 distinguishes epithelioid hemangioendothelioma from histologic mimics. Am J Surg Pathol. 2016;40(1):94–102.

    Google Scholar 

  11. Fukayama M, Nihei Z, Takizawa T, Kawaguchi K, Harada H, Koike M. Malignant epithelioid hemangioendothelioma of the liver, spreading through the hepatic veins. Virchows Arch A Pathol Anat Histopathol. 1984;404(3):275–87.

    Google Scholar 

  12. Penel N, Coindre J, Giraud A, et al. Abstract 1478PD - Adult Translocation-related soft tissue sarcomas (TRS): presentation, management and outcome of 2,143 cases confirmed by expert pathologists. ESMO conference 2017.

    Google Scholar 

  13. Errani C, Sung YS, Zhang L, et al. Monoclonality of multifocal epithelioid hemangioendothelioma of the liver by analysis of WWRT1-CAMTA1 breakpoints. Cancer Genet. 2012;470:12–7.

    Google Scholar 

  14. Tanas MR, Sboner A, Oliveira AM, Erickson-Johnson MR, Hespelt J, Hanwright PJ, Flanagan J, Luo Y, Fenwick K, Natrajan R, Mitsopolous C, Zvelebil M, Hoch BL, Weiss SW, Debiec-Rychter M, Sciot R, West RB, Lazar AJ, Ashworth A, Reis-Filho JS, Lord CJ, Gerstein MB, Rubin MA, Rubin BP. Identification of disease-defining gene fusion in epithelioid hemangioendothelioma. Sci Transl Med. 2011;3:98ra82.

    Google Scholar 

  15. Tanas MR, Ma S, Jadaan FO, et al. Mechanism of action of a WWTR1(TAZ)-CAMTA1 fusion oncoprotein. Oncogene. 2016;35(7):929–38.

    Google Scholar 

  16. Antonescu CR, Le Loarer F, Mosquera JM, Sboner A, Zhang L, Chen CL, Chen HW, Pathan N, Krausz T, Dickson BC, Weinreb I, Rubin MA, Hameed M, Lee SJ, Yang WI, Chung WS, Kim SK. Epithelioid hemangioendotheliomas with TFE3 gene translocaion are compossible wih CAMTA1 rearrangements. Oncotarget. 2016;7:7480–8.

    Google Scholar 

  17. Patel NR, Salim AA, Sayeed H, et al. Molecular characterization of epithelioid haemangioendotheliomas identifies novel WWTR1–CAMTA1 fusion variants. Histopathology. 2015;67:699–708.

    Google Scholar 

  18. Lee SJ, Yang WI, Chung WS, Kim SK. Epithelioid hemangioendotheliomas with TFE3 gene translocations are compossible with CAMTA1 gene rearrangements. Oncotarget. 2016;16:7(7):7480–8. https://doi.org/10.18632/oncotarget.7060.

  19. Breitschwerdt EB, Maggi RG, Varanat M, et al. Isolation of Bartonella vinsonii susp berkhoffii genotype II from a boy with epithelio hemangioendothelioma and a dog with hemangiopericytoma. J Clin Microbiol. 2009;47:1957–60.

    Google Scholar 

  20. Mascarelli PE, Iredell JR, Maggi RG, et al. Bertonella species bacteremia in two patients with epithelioid hemangioendothelioma. J Clin Microbiol. 2011;49:4006–12.

    Google Scholar 

  21. Thomas RM, Aloia TA, Truty MJ, et al. Treatment sequencing strategy for hepatic epithelioid hemangioendothelioma. HPB. 2014;16:677–85.

    Google Scholar 

  22. Ishak KG, Sesterhenn IA, Goodman ZD, et al. Epithelioid hemangioendothelioma of the liver: a clinicopathologic and follow-up study of 32 cases. Hum Pathol. 1984;15:839–52.

    Google Scholar 

  23. Mehrabi A, Kashfi A, Fonouni H, Schemmer P, Schmied BM, Hallscheidt P, et al. Primary malignant hepatic epitheloid hemangioendotelioma: a comprehensive review of the literature with emphasis on the surgical therapy. Cancer. 2006;107:2108–21.

    Google Scholar 

  24. Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer. 1999;85:562–82.

    Google Scholar 

  25. Frider B, Bruno A, Selser J, Vanesa R, Pascual P, Bistoletti R. Kasabach-Merrit syndrome and adult hepatic epithelioid hemangioendothelioma an unusual association. J Hepatol. 2005;42:282–3.

    Google Scholar 

  26. Hayashi Y, Inagaki K, Hirota S, Yoshikawa T, Ikawa H. Epithelioid hemangioendothelioma with marked liver deformity and secondary Budd-Chiari syndrome: pathological and radiological correlation. Pathol Int. 1999;49:547–52.

    Google Scholar 

  27. Earnest F 4th, Johnson CD. Case 96: hepatic epithelioid hemangioendothelioma. Radiology. 2006;240(1):295–8.

    Google Scholar 

  28. Miller WJ, Dodd GD 3rd, Federle MP, et al. Epithelioid hemangioendothelioma of the liver: imaging findings with pathologic correlation. AJR Am J Roentgenol. 1992;159(1):53–7.

    Google Scholar 

  29. Lyburn ID, Torreggiani WC, Harris AC, et al. Hepatic epithelioid hemangioendothelioma: sonographic, CT, and MR imaging appearances. AJR Am J Roentgenol. 2003;180(5):1359–64.

    Google Scholar 

  30. Giardino A, Miller FH, Kalb B, Ramalho M, Martin DR, Rodacki K, Woosley JT, Semelka RC. Hepatic epithelioid hemangioendothelioma: a report from three university centers. Radiol Bras. 2016;49(5):288–94.

    Google Scholar 

  31. Paolantonio P, Laghi A, Vanzulli A, et al. MRI of hepatic epithelioid hemangioendothelioma (HEH). J Magn Reson Imaging. 2014;40(3):552–8.

    Google Scholar 

  32. Kim EH, Rha SE, Lee YJ, et al. CT and MR imaging findings of hepatic epithelioid hemangioendotheliomas: emphasis on single nodular type. Abdom Imaging. 2015;40(3):500–9.

    Google Scholar 

  33. Bagan P, Hassan M, Riquet M, et al. Prognostic factors and surgical indications of pulmonary epithelioid hemangioendothelioma: a review of the literature. Ann Thorac Surg. 2006;82:2010–3.

    Google Scholar 

  34. Woo JH, Kim TJ, Lee KS, et al. Epithelioid hemangioendothelioma in the thorax: Clinicopathologic, CT, PET, and prognostic features. Medicine (Baltimore). 2016;95(30):e4348.

    Google Scholar 

  35. Kim EY, Kim TS, Han J, et al. Thoracic epithelioid hemangioendothelioma: imaging and pathologic features. Acta Radiol. 2011;52(2):161–6.

    Google Scholar 

  36. Cronin P, Arenberg D. Pulmonary epithelioid hemangioendothelioma: an unusual case and a review of the literature. Chest. 2004;125:789–93.

    Google Scholar 

  37. Amin RMS, Hiroshima K, Kokubo T, Nishikawa M, Narita M, Kuroki M, et al. Risk factors and independent predictors of survival in patients with pulmonary epithelioid haemangioendothelioma. Review of the literature and a case report. Respirology. 2006;11(6):818–25.

    Google Scholar 

  38. Sardaro A, Bardoscia L, Petruzzelli MF, Portaluri M. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor. Oncol Rev. 2014;8(2):259.

    Google Scholar 

  39. Kransdorf MJ, et al. Vascular and lymphatic tumors. Imaging of soft-tissue tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2006. p. 177–88.

    Google Scholar 

  40. Angelini A, Mavrogenis AF, Gambarotti M, et al. Surgival treatment and results of 62 patients with epithelioid hemangioendothelioma of bone. J Surg Oncol. 2014;109:791–7.

    Google Scholar 

  41. Kleer CG, Unni KK, McLeod RA. Epithelioid hemangioendothelioma of bone. Am J Surg Pathol. 1996;20(11):1301–11.

    Google Scholar 

  42. Deyrup AT, Tighiouart M, Montag AG, et al. Epithelioid hemangioendothelioma of soft tissue: a proposal for risk stratification based on 49 cases. Am J Surg Pathol. 2008;32:924–7.

    Google Scholar 

  43. Lau K, Massad M, Weinberg G, et al. Clinical patterns and outcome in epithelioid hemangioendothelioma with or without pulmonary involvement. Chest. 2011;140:1312–8.

    Google Scholar 

  44. Gomez-Arellano LI, Ferrari-Caballo T, Dominguez-Malagon HR. Multicentric epithelioid hemangioendothelioma of bone. Report of a case with radiologic-pathologic correlation. Ann Diagn Pathol. 2012;16:43–7.

    Google Scholar 

  45. Fagen K, Silverman ED, Cole RL. Detection of a pulmonary epithelioid hemangioendothelioma by FDG PET scan. Clin Nucl Med. 2004;29:758–9.

    Google Scholar 

  46. Furui S, Itai Y, Ohtomo K, et al. Hepatic epithelioid hemangioendothelioma: report of five cases. Radiology. 1989;171:63–8.

    Google Scholar 

  47. Watanabe S, Yano F, Kita T, et al. 18F-FDG-PET/CT as an indicator for resection of pulmonary epithelioid hemangioendothelioma. Ann Nucl Med. 2008;22:521–4.

    Google Scholar 

  48. Han JH, Kim TJ, Lee KS, et al. Epithelioid hemangioendothelioma in the thorax: clinicopathologic, CT, PET and prognostic features. Medicine. 2016;95:30.

    Google Scholar 

  49. Dong A, Dong H, Wang Y, Gong J, Lu J, Zuo C. MRI and FDG PET/CT findings of hepatic epithelioid hemangioendothelioma. Clin Nucl Med. 2013;38(2):e66–73.

    Google Scholar 

  50. Mehrabi A, Hoffman K, Weiss KH, et al. Long term follow up after resection emphasizes the role of surgery in primary hepatic epithelioid hemangioendothelioma. Ann Med Surg. 2016;11:1–4.

    Google Scholar 

  51. Kleck CJ, Seidel MJ. Epithelioid hemangioendothelioma of the distal humerus with pathologic fracture. Orthopedics. 2012;35:e116–9.

    Google Scholar 

  52. Suga K, Kawakami Y, Hiyama A, Hori K. F-18 FDG PET/CT monitoring of radiation therapeutic effect in hepatic epithelioid hemangioendothelioma. Clin Nucl Med. 2009;34(3):199–202.

    Google Scholar 

  53. Rojas-Vigott R, Castro CM, Mendez SR. Hemangioendothelioma: a case report of spontaneous regression. J Thorac Oncol. 2016;11(10S):S231–2.

    Google Scholar 

  54. Nair LK, Das A, Arun Kumar AS, Ramachandran V. Self regressing epitheloid haemangioendothelioma of tibia in an infant- a rare case report and review of literature. J Orthop Case Rep. 2015;5(1):37–40.

    Google Scholar 

  55. Otrock ZK, Al-Kutoubi A, Kattar MM, Zaatari G, Soweid A. Spontaneous complete regression of hepatic epithelioid haemangioendothelioma. Lancet Oncol. 2006;7(5):439–41.

    Google Scholar 

  56. Yousaf N, Maruzzo M, Judson I, et al. Systemic treatment options for epithelioid hemangioendothelioma: the Royal Marsden experience. Anticancer Res. 2015;35:473–80.

    Google Scholar 

  57. Kelly H, O’Neil BH. Response of epithelioid haemangioendothelioma to liposomal doxorubicin. Lancet Oncol. 2005;6:813–5.

    Google Scholar 

  58. Grenader T, Vernea F, Reinus C, Gabizon A. Malignant epithelioid hemangioendothelioma of the liver successfully treated with pegylated liposomal doxorubicin. J Clin Oncol. 2011;29:e722–4.

    Google Scholar 

  59. Pintoffl J, Meisinger I, Mayer F, et al. Long-term disease stabilization during seocn-line gemcitabine in a refractory metastatic haemangioendothelioma. Anti-Cancer Drugs. 2009;20:73–4.

    Google Scholar 

  60. Kanemura S, Kuribayashi K, Moriya Y, Shimizu S, Tsujimura T, Nakano T. Pemetrexed for epithelioid haemangioendothelioma of the pleura. Respirol Case Rep. 2016;4(6):e00191.

    Google Scholar 

  61. Stacher E, Gruber-Mösenbacher U, Halbwedl I, Dei Tos AP, Cavazza A, Papotti M, et al. The VEGF-system in primary pulmonary angiosarcomas and haemangioendotheliomas: new potential therapeutic targets? Lung Cancer. 2009;65(1):49–55.

    Google Scholar 

  62. Raphael C, Hudson E, Williams L, Lester JF, Savage PM. Successful treatment of metastatic hepatic epithelioid hemangioendothelioma with thalidomide: a case report. J Med Case Rep. 2010;4:413.

    Google Scholar 

  63. Kassam A, Mandel K. Metastatic hepatic epithelioid hemangioendothelioma in a teenage girl. J Pediatr Hematol Oncol. 2008;30:550–2.

    Google Scholar 

  64. Bölke E, Gripp S, Peiper M, Budach W, Schwarz A, Orth K, et al. Multifocal epithelioid hemangioendothelioma: case report of a clinical chamaeleon. Eur J Med Res. 2006;11:462–6.

    Google Scholar 

  65. Mascarenhas RC, Sanghvi AN, Friedlander L, Geyer SJ, Beasley HS, Van Thiel DH. Thalidomide inhibits the growth and progression of hepatic epithelioid hemangioendothelioma. Oncology. 2004;67:471–5.

    Google Scholar 

  66. Salech F, Valderrama S, Nervi B, Rodriguez JC, Oksenberg D, Koch A, Smok G, Duarte I, Pérez-Ayuso RM, Jarufe N, Martínez J, Soza A, Arrese M, Riquelme. Thalidomide for the treatment of metastatic hepatic epithelioid hemangioendothelioma: a case report with a long term follow-up. Ann Hepatol. 2011;10(1):99–102.

    Google Scholar 

  67. Kollár A, Jones RL, 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;56(1):88–92.

    Google Scholar 

  68. Bally O, Tassy L, Richioud B, Decouvelaere A-V, Blay J-Y, Derbel O. Eight years tumor control with pazopanib for a metastatic resistant epithelioid hemangioendothelioma. Clin Sarcoma Res. 2015;5:12.

    Google Scholar 

  69. Chevreau C, Le Cesne A, Ray-Coquard I, et al. Sorafenib in patients with progressive epithelioid hemangioendothelioma. Cancer. 2013;119:2639–44.

    Google Scholar 

  70. Sangro B, Iñarrairaegui M, Fernández-Ros N. Malignant epithelioid hemangioendothelioma of the liver successfully treated with Sorafenib. Rare Tumors. 2012;4(2):e34.

    Google Scholar 

  71. Kobayashi N, Shimamura T, Tokuhisa M, Goto A, Ichikawa Y. Sorafenib monotherapy in a patient with unresectable hepatic epithelioid hemangioendothelioma. Case Rep Oncol. 2016;9(1):134–7.

    Google Scholar 

  72. Agulnik M, yarber JL, Okuno SH, et al. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Ann Oncol. 2013;24:257–63.

    Google Scholar 

  73. Stacchiotti S, Provenzano S, Dagrada G, Negri T, Brich S, Basso U, et al. Sirolimus in advanced epithelioid hemangioendothelioma: a retrospective case-series analysis from the italian rare cancer network database. Ann Surg Oncol. 2016;23(9):2735–44.

    Google Scholar 

  74. Hettmer S, Andrieux G, Hochrein J, Kurz P, Rössler J, Lassmann S, et al. Epithelioid hemangioendotheliomas of the liver and lung in children and adolescents. Pediatr Blood Cancer. 2017;64(12):e26675.

    Google Scholar 

  75. Davies KD, Doebele RC. Molecular pathways: ROS1 fusion proteins in cancer. Clin Cancer Res. 2013;19(15):4040–5.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Institut Bergonié, Bordeaux, France

    Sophie Cousin, François Le Loarer & Amandine Crombé

  2. Centre Léon Berard, Lyon, France

    Marie Karanian

  3. Institut Gustave Roussy, Villejuif, France

    Véronique Minard

  4. Centre Oscar Lambret, Lille, France

    Nicolas Penel

Authors
  1. Sophie Cousin
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. François Le Loarer
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Amandine Crombé
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Marie Karanian
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Véronique Minard
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Nicolas Penel
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sophie Cousin .

Editor information

Editors and Affiliations

  1. IUCT Oncopole, Institut Claudius Regaud, Toulouse, France

    Christine Chevreau

  2. Institut Bergonié, Bordeaux, France

    Antoine Italiano

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Cousin, S., Le Loarer, F., Crombé, A., Karanian, M., Minard, V., Penel, N. (2020). Epithelioid Hemangioendothelioma. In: Chevreau, C., Italiano, A. (eds) Rare Sarcomas. Springer, Cham. https://doi.org/10.1007/978-3-030-24697-6_7

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-24697-6_7

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-24696-9

  • Online ISBN: 978-3-030-24697-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation