Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment

Ari Rosenberg, Mark Agulnik*

*Corresponding author for this work

Research output: Contribution to journalComment/debate

26 Scopus citations

Abstract

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.

Original languageEnglish (US)
Article number19
JournalCurrent treatment options in oncology
Volume19
Issue number4
DOIs
StatePublished - Apr 1 2018

Keywords

  • CAMTA1
  • Epithelioid hemangioendothelioma
  • Sarcoma
  • WWTR1

ASJC Scopus subject areas

  • Oncology
  • Pharmacology (medical)

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