Apatinib for the treatment of pulmonary epithelioid hemangioendothelioma

Abstract

Rationale:Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare tumor, with no established standard treatment. Overexpression of vascular endothelial growth factor receptor 2 (VEGFR-2) has been reported in some P-EHE patients. Apatinib, a new small molecule tyrosine kinase inhibitor that specifically targets VEGFR-2, has therapeutic benefits in some advanced tumors. However, its efficacy in P-EHE cases has not been reported.Patient concerns:Herein, we presented a 44-year-old man with recurrent hemoptysis for approximately 9 years.Diagnoses:After hospitalization, relevant examinations were conducted. The disease was subsequently diagnosed as P-EHE.Interventions:The patient underwent pulmonary lobectomy, but subsequently developed multiple metastases. Within the tumor, CD31, CK, and Vimentin were found to be positive, while CD34 was negative. Apatinib was initially administered 250 mg daily doses and after 1 month was increased to 500 mg daily.Outcomes:He showed noticeable symptomatic improvements and positive imaging changes in the first month of treatment. However, the disease progressed in the following month, despite the increased apatinib dose.Lessons:Apatinib is possibly a new treatment for P-EHE. However, further clinical trials are necessary to confirm an effective dose and the efficacy and safety of apatinib in P-EHE treatment.

Activity of sirolimus in patients with progressive epithelioid hemangioendothelioma: A case‐series analysis within the Italian Rare Cancer Network

“…The role for the drugs commonly used in sarcomas is limited, 9,12 although signs of activity were detected with antiangiogenics, thalidomide, interferon, and celecoxib. 9,[13][14][15][16][17][18] Our group previously reported on the activity of the mTOR inhibitor sirolimus in a retrospective series of 18 patients with advanced EHE who were treated at the IRCCS Foundation National Cancer Institute of Milan and within the Italian Rare Cancer Network. 19 Herein we update that series with a larger number of patients.…”

<p>Metastatic Hepatic Epithelioid Hemangioendothelioma Treated with Olaratumab: A Falling Star Rising?</p>

“…[18][19][20][21][22] Tyrosine kinase inhibitors such as pazopanib, sunitinib, apatinib and sorafenib have been studied showing PRor stabilization of the disease. [23][24][25][26][27][28] Targeting angiogenesis with bevacizumab, interferon alpha 2b, thalidomide and lenalidomide revealed contradictory results. [29][30][31][32][33] Case reports using metronomic cyclophosphamide and sirolimus have also been published.…”

Epithelioid Hemangioendothelioma as a Model of YAP/TAZ-Driven Cancer: Insights from a Rare Fusion Sarcoma

“…Use of anti-angiogenic agents is rational given that EHE is a disease of unregulated proliferation in a cell with endothelial markers [ 85 , 86 ]. Drugs used in EHE have included thalidomide, bevacizumab (vascular endothelial growth factor inhibitor), and apatinib (specific VEGFR2 inhibitor) [ 87 , 88 , 89 ]. Case reports combining the use of cytotoxic chemotherapy regimens, such as carboplatin or pemetrexed with the addition of bevacizumab, to treat pulmonary EHE also show promise [ 90 ].…”

“…One case report in a patient with metastatic EHE treated with thalidomide after failure of interferon documents radiological and clinical stable disease for 7 years [ 88 ]. Conversely, there are also case reports that document progression or partial response on thalidomide [ 87 , 89 ]. The mTOR inhibitor sirolimus has shown some promise in advanced EHE.…”