上皮样血管内皮瘤的诊断和治疗

doi:10.3881/j.issn.1000-503X.10302

Acta Academiae Medicinae Sinica

Acta Academiae Medicinae Sinica ›› 2018, Vol. 40 ›› Issue (5): 667-672.doi: 10.3881/j.issn.1000-503X.10302

• Original Articles • Previous Articles Next Articles

Diagnosis and Treatment of Epithelioid Hemangioendothelioma

WU Xin, LI Binglu(), ZHENG Chaoji, HE Xiaodong

Department of General Surgery,PUMC Hospital,CAMS and PUMC,Beijing 100730,China

Received:2018-01-24 Online:2018-10-30 Published:2018-11-07
Contact: Binglu LI E-mail:libinglu@pumch.cn

Abstract

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.

Key words: hemangioendothelioma, epithelioid, diagnosis, treatment

CLC Number:

R732.2

WU Xin,LI Binglu,ZHENG Chaoji,HE Xiaodong. Diagnosis and Treatment of Epithelioid Hemangioendothelioma[J].Acta Academiae Medicinae Sinica, 2018, 40(5): 667-672.

Figures/Tables 4

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Table 2

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References 25

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病变部位Site	头皮Scalp	脊椎Spine	肝脏Liver	鼻腔Nasal cavity	脾脏Spleen	胸壁Chest wall
手术Surgery	2	1	1	2	2	1
活检Biopsy	2	3	2	0	0	1
病变部位Site	肺脏Lung	舌Tongue	心房Atrium	纵隔Mediastinum	颈部Neck	腋窝Axilla
手术Surgery	1	1	1	1	1	1
活检Biopsy	1	0	0	0	0	0
病变部位Site	臀部Hip	宫颈Cervix uteri	小肠Small intestine	骶前Presacral area	颅骨Skull	食管Esophagus
手术Surgery	1	1	1	1	0	0
活检Biopsy	0	0	0	0	1	1

项目Items	CD34	CD31	SMA	AE1/AE3
检测例数Number of detected cases(n)	30	30	22	19
阳性例数Number of positive cases(n)	30	29	16	7
阳性比例 Positive rate(%)	100	96.7	72.7	36.8
项目Items	S-100	F8-R	Vimentin	CD68
检测例数Number of detected cases(n)	17	16	15	5
阳性例数Number of positive cases(n)	5	13	13	4
阳性比例 Positive rate(%)	29.4	81.3	86.7	80.0

分组Group	年龄(岁) Age(years)	性别 Gender(M/F)	冠心病 Coronary heart disease(n)	高血压 Hypertension(n)	糖尿病 Diabetes(n)
手术Surgery group(n=14)	47.9±16.6	3/11	1	4	1
活检Biopsy group(n=9)	50.7±14.6	3/6	0	2	1
t	-0.403	-	-	-	-
P	0.691	0.643	1.000	1.000	1.000
分组Group	住院天数 Hospital stay(d)	原发肿瘤大小 Primary tumor size(cm)	肿瘤标志物异常 Abnormal tumor markers(n)	远处转移 Distant metastasis (n)	死亡人数 Death toll(n)
手术Surgery group(n=14)	17.6±13.4	5.6±2.9	1	2	1
活检Biopsy group(n=9)	19.2±18.5	6.0±2.6	4	7	5
t	-0.249	-0.290	-	-	-
P	0.806	0.775	0.056	0.007	0.018

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