Diagnosis and Treatment of Epithelioid Hemangioendothelioma / 中国医学科学院学报

ABSTRACT

Objective To evaluate the clinical feature,diagnosis,and treatment of epithelioid hemangioendothelioma(EHE).Methods Data of 30 EHE patients admitted from January 2001 to December 2017 were retrospectively analyzed. The auxiliary examinations,treatment method,pathological findings,and prognoses were analyzed.Results There were 10 male patients and 20 females aged(47.9±16.1)years. The lesions sized(5.8±2.8)cm. Seventeen cases(56.7%)were asymptomatic and 17(56.7%)had no positive sign. The most common locations of EHE were scalp and spine,followed by liver. Nineteen patients underwent surgical operation(surgery group)and 11 only accepted needle biopsy(biopsy group). EHE was pathologically confirmed in all cases. CD34 and CD31 showed the highest positive rates in immunohistochemical stains. Twenty-three cases were followed up by(74.1±56.8)months. Seventeen cases survived and 6 died. The 1-,3-,and 5-year cumulative survival rates were 95.7%,86.3%,and 73.6%,respectively. The metastasis(14.3% vs. 77.8%,P=0.007)and mortality(7.1% vs. 55.6%,P=0.018)rates were significantly lower in surgery group than in biopsy group.Conclusions EHE is a rare tumor without specific symptom or sign. It can occur in any system of the body. Diagnosis depends mainly on pathology. The prognosis is acceptable. Complete surgical resection reduces distant metastases and mortality.