Pulmonary Epithelioid Hemangioendothelioma:A Case Report And Literature Review

Objective:To improve the identification of pulmonary epithelioid hemangioendothelioma,especially for its treatment,by reporting one case,reviewing literature reported in English and analyzing the clinical characteristics,radiological,pathological features,treatment and prognosis of characteristics.Methods:Report 1 case of pulmonary epithelioid hemangioendothelioma confirmed by lobectomy of lung biopsy in respiratory medicine of the Sir Run Run Shaw Hospital Affiliated to Zhejiang University Medicine of School.Sixty-three pulmonary epithelioid hemangioendothelioma cases reported in English so far were retroactively analyzed and literature reviewed.Results:We analyzed 63 cases diagnosed with pulmonary epithelioid hemangioendothelioma confirmed by lung biopsies.There were 22 males and 41 females,who were ranging from 13 years old to 76 years old,with a mean age of 43.6.Clinical manifestations were nonspecific.19 cases had no obvious clinical symptoms.Majority of the symptomatic patients were presented cough,chest pain and shortness of breath,minority of them were presented hemoptysis,fever,weight loss and other symptoms.The imaging findings were mostly bilateral pulmonary nodules.The diagnosis method was histopathological examination.Referring to immunohistochemical staining,CD31,CD34 and FⅧ were mostly positive.The patients without obvious clinical symptoms or extrapulmonary metastases could be observed or simple treated with surgical excision.Their prognosis was generally good.The patients who could not tolerate surgery or had multiple organ metastasis were given to chemotherapy.Cisplatin,paclitaxel or anthracycline-based drugs were mostly used.Anti-angiogenesis targeted drugs,such as bevacizumab,were also used in few patients.There was a difference between the prognosis of patients.Conclusion:Pulmonary epithelioid hemangioendothelioma is relatively rare.Its clinical manifestation,laboratory examination and imaging findings have no obvious specificity.A standard treatment and consensual management for P-EHE are not presently available.antiangiogenic therapy may be a promising treatment for advanced P-EHE due to the expression of VEGFR-2 in this malignancy.Apatinib as a targeted therapy may have a more promising role in treating selected advanced.P-EHE patients than multitargeted agents acting on various tyrosine kinases.However,further studies using large sample sizes are required to determine the safety and efficacy of apatinib for treating P-EHE.