Epithelioid Hemangioendothelioma: Treatment Landscape and Innovations for an Ultra-Rare Sarcoma

Paulson KG, et al. Incidence, demographics, and survival of malignant hemangioendothelioma in the United States. Cancer Med. 2023;12:15101–6.

Article  PubMed  PubMed Central  Google Scholar 

Stacchiotti S, et al. Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts. ESMO Open. 2021;6:100170.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Shiba S, et al. Clinical characteristics of Japanese patients with epithelioid hemangioendothelioma: a multicenter retrospective study. BMC Cancer. 2018;18:993.

Article  CAS  PubMed  PubMed Central  Google Scholar 

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

Article  CAS  Google Scholar 

Tanas MR, et al. Identification of a disease-defining gene fusion in epithelioid hemangioendothelioma. Sci Transl Med. 2011;3:98ra82-98ra82.

Article  PubMed  Google Scholar 

Antonescu CR, et al. Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma. Genes Chromosome Cancer. 2013;52:775–84.

Article  CAS  Google Scholar 

Shibuya R, et al. CAMTA1 is a useful immunohistochemical marker for diagnosing epithelioid haemangioendothelioma. Histopathology. 2015;67:827–35.

Article  PubMed  Google Scholar 

Rosenbaum E, et al. Prognostic stratification of clinical and molecular epithelioid hemangioendothelioma subsets. Mod Pathol Off J U S Can Acad Pathol Inc. 2020;33:591–602.

CAS  Google Scholar 

Zhang H, et al. TEAD transcription factors mediate the function of TAZ in cell growth and epithelial-mesenchymal transition. J Biol Chem. 2009;284:13355–62.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Liu C-Y, et al. The hippo tumor pathway promotes TAZ degradation by phosphorylating a phosphodegron and recruiting the SCFβ-TrCP E3 ligase. J Biol Chem. 2010;285:37159–69.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Hansen CG, Ng YLD, Lam W-LM, Plouffe SW, Guan K-L. The Hippo pathway effectors YAP and TAZ promote cell growth by modulating amino acid signaling to mTORC1. Cell Res. 2015;25:1299–313.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Xu H, et al. MEK nuclear localization promotes YAP stability via sequestering β-TrCP in KRAS mutant cancer cells. Cell Death Differ. 2019;26:2400–15.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Pulkkinen HH, et al. BMP6/TAZ-Hippo signaling modulates angiogenesis and endothelial cell response to VEGF. Angiogenesis. 2021;24:129–44.

Article  CAS  PubMed  Google Scholar 

Stacchiotti S, et al. Activity of sirolimus in patients with progressive epithelioid hemangioendothelioma: a case-series analysis within the Italian Rare Cancer Network. Cancer. 2021;127:569–76.

Article  CAS  PubMed  Google Scholar 

Driskill JH, et al. WWTR1(TAZ)-CAMTA1 reprograms endothelial cells to drive epithelioid hemangioendothelioma. Genes Dev. 2021;35:495–511.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Seavey CN, et al. WWTR1(TAZ)-CAMTA1 gene fusion is sufficient to dysregulate YAP/TAZ signaling and drive epithelioid hemangioendothelioma tumorigenesis. Genes Dev. 2021;35:512–27.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Hu EY, et al. Image-guided percutaneous ablation of hepatic epithelioid hemangioendothelioma. Abdom Radiol N Y. 2024;49:1241–7.

Article  Google Scholar 

Vidal-Jove J, et al. First-in-man histotripsy of hepatic tumors: the THERESA trial, a feasibility study. Int J Hyperth Off J Eur Soc Hyperthermic Oncol North Am Hyperth Group. 2022;39:1115–23.

Article  Google Scholar 

Mendiratta-Lala M, et al. The #HOPE4LIVER single-arm pivotal trial for histotripsy of primary and metastatic liver tumors. Radiology. 2024;312:e233051.

Article  PubMed  Google Scholar 

Stacchiotti S, Tap W, Leonard H, Zaffaroni N, Baldi GG. New molecular insights, and the role of systemic therapies and collaboration for treatment of epithelioid hemangioendothelioma (EHE). Curr Treat Options Oncol. 2023;24:667–79.

Article  PubMed  Google Scholar 

Frezza AM, et al. Systemic therapies in advanced epithelioid haemangioendothelioma: a retrospective international case series from the World Sarcoma Network and a review of literature. Cancer Med. 2021;10:2645–59.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Cioffi A, et al. Metastatic epitheloid hemangioendothelioma (EHE): Role of systemic therapy and survival. J Clin Oncol. 2011;29:10079–10079.

Article  Google Scholar 

Yousaf N, et al. Systemic treatment options for epithelioid haemangioendothelioma: the Royal Marsden Hospital experience. Anticancer Res. 2015;35:473–80.

PubMed  Google Scholar 

Park MS, Ravi V, Araujo DM. Inhibiting the VEGF-VEGFR pathway in angiosarcoma, epithelioid hemangioendothelioma, and hemangiopericytoma/solitary fibrous tumor. Curr Opin Oncol. 2010;22:351–5.

Article  CAS  PubMed  Google Scholar 

Sampat KR, O’Neil B. Antiangiogenic therapies for advanced hepatocellular carcinoma. Oncologist. 2013;18:430–8.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Agulnik M, et al. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Ann Oncol Off J Eur Soc Med Oncol. 2013;24:257–63.

Article  CAS  Google Scholar 

Dhillon S. Bevacizumab combination therapy: a review of its use in patients with epithelial ovarian, fallopian tube, or primary peritoneal cancer. BioDrugs Clin Immunother Biopharm Gene Ther. 2013;27:375–92.

CAS  Google Scholar 

Belmont L, Zemoura L, Couderc LJ. Pulmonary epithelioid haemangioendothelioma and bevacizumab. J Thorac Oncol Off Publ Int Assoc Study Lung Cancer. 2008;3:557–8.

Google Scholar 

Telli TA, et al. VEGF-VEGFR pathway seems to be the best target in hepatic epithelioid hemangioendothelioma: a case series with review of the literature. Curr Probl Cancer. 2020;44:100568.

Article  PubMed  Google Scholar 

Tolkach Y, Petrov S, Lerut E, Van Poppel H. Epithelioid hemangioendothelioma of the kidney treated with sunitinib. Onkologie. 2012;35:376–8.

Article  CAS  PubMed  Google Scholar 

Saada E, Saint Paul M-C, Gugenheim J, Follana P, François E. Metastatic hepatic epithelioid hemangio-endothelioma: long-term response to sunitinib malate. Oncol Res Treat. 2014;37:124–6.

Article  PubMed  Google Scholar 

Chevreau C, et al. Sorafenib in patients with progressive epithelioid hemangioendothelioma: a phase 2 study by the French Sarcoma Group (GSF/GETO). Cancer. 2013;119:2639–44.

Article  CAS  PubMed  Google Scholar 

Kumar R, et al. Pharmacokinetic-pharmacodynamic correlation from mouse to human with pazopanib, a multikinase angiogenesis inhibitor with potent antitumor and antiangiogenic activity. Mol Cancer Ther. 2007;6:2012–21.

Article  CAS  PubMed  Google Scholar 

van der Graaf WT, et al. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. The Lancet. 2012;379:1879–86.

Article  Google Scholar 

Bally O, et al. Eight years tumor control with pazopanib for a metastatic resistant epithelioid hemangioendothelioma. Clin Sarcoma Res. 2015;5:12.

Comments (0)

No login
gif