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Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment

  • Sarcoma (SH Okuno, Section Editor)
  • Published:
Current Treatment Options in Oncology Aims and scope Submit manuscript

Opinion statement

Epithelioid hemangioendothelioma (EHE) is an extremely rare sarcoma, as such it can pose a clinical dilemma based solely on its rarity. Also, the spectrum of disease varies greatly between an indolent disease and aggressive disease with widespread metastases. In our clinical practice, the primary focus has been to get a handle on the aggressive nature of the disease, which will then dictate how urgently one needs to treat the patient. Pathological review with immunohistochemistry and molecular characterization is paramount. Our treatment strategy is watch-and-wait versus active therapy on clinical trial or based on results of prior clinical trials. There is evidence to support the use of chemotherapeutics and targeted therapies specifically focusing on anti-angiogenesis. The current landscape of oncology with the emergence and excitement of immunotherapy could also translate in a role for immunotherapy in this disease. While rare, there is certainly no reason that research and trials for patients with EHE should not remain on utmost importance for those of us who specialize in the treatment of sarcomas.

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Authors and Affiliations

  1. Division of Hematology/Oncology, Northwestern University, Feinberg School of Medicine, 676 North Saint Clair, Suite 850, Chicago, IL, 60611, USA

    Ari Rosenberg MD & Mark Agulnik MD

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  1. Ari Rosenberg MD
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Correspondence to Mark Agulnik MD.

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Ari Rosenberg and Mark Agulnik declare they have no conflict of interest.

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Rosenberg, A., Agulnik, M. Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment. Curr. Treat. Options in Oncol. 19, 19 (2018). https://doi.org/10.1007/s11864-018-0536-y

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