Objective      To summarize the experiences in the diagnosis and treatment of multiple epithelioid hemangioendothelioma (EHE)

which is similar to lung cancer metastasis.      Methods      The clinical and pathological characteristics of 6 cases of pulmonary epithelioid hemangioendothelioma (PEH) were retrospectively analyzed

and the related articles were reviewed.       Results      Because of lacking of specific clinical manifestations

6 cases of PEH were initially misdiagnosed as lung cancer

in which 1 case received surgery

2 cases received cisplatin-based chemotherapy

1 case was treated by oral administration of etoposide

and 2 cases gave up. The follow-up suggested that the patients had good prognosis overall.       Conclusion      PEH is easily misdiagnosed as lung cancer

and correct diagnosis and differential diagnosis about PEH require combination of imaging studies

histological observation

immunohistochemical analysis of specimens

and other methods. Combined therapy of surgery and chemotherapy is the main treatment manner

but the efficacy needs to be confirmed with more evidence-based clinical studies.