Clinical Characteristics, Diagnosis, Treatment And Prognosisof Pulmonary Epithelioid Hemangioendothelioma

Objective: Pulmonary epithelioid hemangioendothelioma(PEH)is an intravascular bronchiolecular alveoloma.It is a rare angiogenic lesion in the lungs and is a borderline tumor.The literature published in China is mostly reported in small cases.At present,the research on the clinical progress of PEH in China is in the exploration stage.This article is mainly to summarize the gender,age,clinical manifestations,auxiliary examination,diagnosis,treatment and prognosis of PEH.It is hoped to increase the clinician's understanding and understanding of the disease and reduce the missed diagnosis and misdiagnosis during the diagnosis and treatment.To prolong the surviva l time of patients with PEH.Method: The article retrospectively analyzed 2 cases of PEH patients diagnosed by pathological immunohistochemistry in the Department of Respiratory and Critical Care of the First Affiliated Hospital of Wannan Medical College from 2016 to 2018,and the literature search for all current domestic reports of PEH cases.For example,26 cases of Wanfang database,10 cases of China Knowledge Network,5 cases of Weipu database,complete clinical relevant data,a total of 43 cases,the following indicators:epidemiological characteristics,clinical manifestations,auxiliary examination,diagnosis,treatment plan,Prognosis,all data were statistically analyzed using SPSS19.0.When the P value is less than 0.05,it means statistically significant.Results: 1.Epidemiological performance: The ratio of male to female incidence of the disease is 1.39:1,and there is no obvious gender difference.The prevalent population is middle-aged,with an average age of onset of 45.53±13.83 years(age span 19-70years).2.Causes of the disease include smoking(20 cases),previous chronic basic diseases(8cases),long-term dust exposure history(3 cases),long-term oral contraceptives(1case),etc.;among them,smoking and/or chronic foundation Disease is one of the independent risk factors for the prognosis of PEH.3.The clinical symptoms are "diversified",no significant features.90.70% of patients were diagnosed with respiratory symptoms.The most common symptoms were cough and expectoration(30.23%),hemoptysis or hemoptysis(16.28%),chest pain(13.95%),chest tightness,shortness of breath(9.30%),and fever.6.98%),weight loss(4.65%)dyspnea(2.33%),other systemic symptoms such as shoulder and back pain(4.65%);clubbing(2.33%);in addition to 9.30% The patient had no symptoms and the physical examination revealed abnormal chest imaging findings.The positive signs mainly included low lung breath sounds(20 cases),superficial lymph node enlargement(2cases),and clubbing fingers(1 case).60.47% of patients showed diffuse or multiple lung lobes,and 39.53% were unilateral lung lobe lesions.No matter what kind of lesions,there was no significant difference in lung left and right lobe involvement(left lung: right lung = 8:9).More than 50% of patients were diagnosed with lung alone(31/43 cases).With the development of clinical course,the lesion can invade the pleura(46.51%),liver(18.60%),lymph nodes(13.95%),bones(4.65).%),heart(2.33%)and other organs,a small number of patients with mediastinal,diaphragmatic muscles can also be affected.4.The gold standard for diagnosis is lung histopathology immunohistochemical examination: at the same time,it is necessary to combine laboratory examination and chest imaging findings.In this study,only 23.26% of patients had laboratoryabnormalities,including 6 with elevated blood levels,14 with abnormal erythrocyte sedimentation rate,3 with abnormal blood biochemistry(2 with impaired liver function),and markers of lung tumors.Abnormalities in 14 cases,mainly CA125,CA19-9,CEA,neuron-specific olefinase abnormalities,etc.;2 of them were cancer cells in pleural effusion;7 cases of tuberculosis-related positive indicators,mostly PPD test(+);2 cases with positive G test;2 cases with abnormal lung function.The imaging of PEH is often characterized by multiple or(and)single nodules in the lower lobe of both lungs.72.09% of the 43 patients presented with "star-like" signs: uneven distribution of patchy or nodular segments in the lungs,27.91% in isolated nodules,and high-density in the nodules accounted for 65.79%,low density 34.21% of the small nodules were measured by high-resolution chest CT.The results showed that the size of 3-5MM was the most common(76.67%),and the diameter of nodules in a small number of patients could be greater than 1cm(10%).Pleural thickening and/or pleural effusion(46.52%)were seen when the lesion was transferred to the pleura.The final diagnosis of PEH relies on the pathological immunohistochemistry of lung tissue:the microscopic epithelioid-like factors CD31(93.02%),CD34(88.37%),FVIII-RAg(81.82%),Vimentin(69.78%)are strongly positive or Positive expression,while TTF-1(100%),S-100(100%),CgA(100%),SMA(93.75%),Syn(93.33%)were mostly negative expression.Vascular endothelial growth factor receptor 2(VEGFR-2)may be the future research direction.5.Treatment: There is no unified plan,and more surgical surgery is preferred.In this study,the treatment of the disease included simple wedge resection of the lung(30.23%),surgical resection combined with radiotherapy and chemotherapy(4.65%),and clear chemotherapy(27.91%)after diagnosis;no treatment observation(18.60%),Oral interferon treatment(2.33%),other(including anti-angiogenic drugs,Chinese medicine treatment,etc.)(16.28%).6.Prognosis: Age,smoking,or(and)previous history of chronic disease,pleural effusion,and treatment are independent risk factors for prognosis. Univariate Log-rank test for factors that may affect prognosis,suggesting age(>50years/<50 years),whether smoking or previous chronic disease,pleural effusion,treatment(surgical/non-surgical)There was statistically significant difference(P<0.05),and gender,asymptomatic signs,abnormal lung tumor markers,and lesion involvement were not statistically significant(P>0.05),which would have clinically significant factors.Multivariate analysis was performed in the Cox proportional hazard regression model,which ultimately indicated that age,smoking or previous history of pulmonary disease,pleural effusion and treatment were independent risk factors for prognosis,and pleural effusion was the effect.The most significant factor in the survival of prognosis.Conclusion: 1.The clinical manifestations of PEH are “diversified”.There is no significant feature in laboratory examination.Chest CT often shows “star-like”,that is,diffuse distribution of nodules in both lungs,which is of great significance for the early diagnosis of the disease.Pathological examination of lung tissue is the gold standard for the diagnosis of this disease.Immunohistochemistry is characteristic:CD31,CD34,vimentin and FVIII factor-related antigens are strongly positive or positive.2.At the current stage of treatment,a unified plan has not been determined.For patients with no obvious surgical contraindications,it is recommended to use surgical resection of the lesion as the first choice.For patients with distant organ involvement,chemotherapy combined with radiotherapy is the best option.Interferon and targeted therapy against VEGF are expected to become the future research direction of PEH.3.PEH is a rare junctional and malignant tumor,prone to multiple metastases,the prognosis of the disease is uncertain.The overall annual survival rate is about 51.8%.For patients with a disease age of more than 50 years,long-term smoking or(and)patients with chronic diseases,PEH patients with pleural effusion often have a poor prognosis and a higher risk of death.It can reduce the risk of death and prolong the prognosis survival time.