Pathologic Features And Analysis Of Primary Pulmonary Vascular Tumor

Background and purposePrimary vascular tumours of lung(PVTL)are a kind of rare tumors occurring in the lung.Vascular tumours are classified into benign,intermediate and malignant tumors by WHO Soft Tissue Classification(2020).According to the review and summary of the current relevant literatures,pulmonary angiogenic tumors include:capillary hemangioma,capillary hemangiomatosis,epithelioid hemangioma,papillary lymphatic hemangiendothelioma,epithelioid hemangiendothelioma,and angiosarcoma,most of which are single case reports.The clinical symptoms,imaging features,histological findings,immunohistochemistry and molecular detection results of 38 cases of PVTL were analyzed and summarized in this paper,in order to improve the understanding of PVTL and provide help and reference for diagnosis.FSMethodA total of 38 cases of PVTL confirmed by pathology in the First Affiliated Hospital of Zhengzhou University from August 2010 to December 2020 were collected,including 24 cases of pulmonary epithelioid hemangioendothelioma(PEHE),11 cases of pulmonary angiosarcoma(PA),and 2 cases of pulmonary capillary hemangioma(PCH).Lung papillary lymphatic hemangioendothelioma(pulmonary papillary intralymphatic angioendothelioma,PPILA)in 1 case,respectively line HE staining,SP method of immunohistochemical staining detection CD31,CD34,ERG,the FLI agreed-1,F Ⅷ,TFE-3,AE1/AE3,Ki-67,such as immunology marker expression,for line FISH in situ hybridization detection WWTR1 PEHE cases-and YAP1 CAMTA1 gene-TFE3 gene,The clinical,imaging,immunohistochemical features and molecular phenotypes of pulmonary angiogenic tumors were observed.The main points of pathological diagnosis,differential diagnosis and prognosis were analyzed,and the relevant literature was reviewed..Result1.Among the 24 cases of pulmonary epithelioid hemangioendothelioma(PEHE),the ratio of male to female was 1.4:1,the age was 31-68 years old,the median age was 52.0 years old.The main clinical symptoms were cough(12/24)and sputum(8/24),and chest pain(5/24).The main CT findings were multiple nodules(20/24,83.0%)in both lungs or in one lung,or single nodules(4/24,17.0%).The nodule has a maximum diameter of 3-70mm and a median diameter of 18mm.Microscopically,it is characterized by the distribution of epithelioid endothelial cells in the opaque transparent matrix of mucus.WWTR1-CAMTA1 gene fusion was present in most cases(22/24,91.7%).A small number of tumors showed distinctive morphology with well-formed blood vessels lined by epithelioid endothelial cells,abundant eosinophilic cytoplasm,and molecular characteristics of YAP 1-TFE3 gene fusion(2/24).The expression rates of CD31,ERG and FLI-1 were 100.0%,CD34 was expressed in 86.9%of the patients,and TFE-3 was positive in 3 patients.There were 24 patients with PEHE,the follow-up rate was 100.0%,the follow-up time was 36 months,16 cases survived,8 cases died.The 1-year mortality was 12.5%,the 3-year mortality was 33.3%,and the median survival time was 50 months.2.There were 11 patients with pulmonary angiosarcoma(PA),with a male-tofemale ratio of 1.2:1,aged 31-81 years and a median age of 54 years.The main symptoms were chest tightness(5/11),cough(5/11)and sputum(6/11).Radiographic manifestations ranged from unilateral lobar mass(8/11)to multiple small nodules scattered in both lungs(3/11).The maximum diameter of the nodules ranged from 1268mm with a median diameter of 17mm.Histologically,the neoplastic cells appear as sheets or lobules,usually with infiltrating margins.The most common is a solid sheet arrangement,but you can see nests or alveolar structures that mimic cancer.Tumor cells often form irregular vascular lumen and infiltrate around the lesion.The neoplastic cells are plump,round to polygonal,with moderate amounts of eosinophilic or amphoteric cytoplasm,large vesicular nuclei,coarse chromatin,and prominent nucleoli.Both CD31 and Fli-1 were positive in 11 PA patients,and the expression rates of ERG and CD34 were 87.5%and 70.0%respectively.Among 11 PA patients,2 were lost to follow-up,3 survived and 6 died,and the 3-year survival rate was only 33.3%.Median survival was 13.5 months.3.Two patients with pulmonary capillary hemangioma(PCH)were admitted,including a 1-year-old boy and a 28-year-old woman,with no obvious clinical symptoms or acute hemoptysis.Radiographic findings showed multiple nodules in both lungs with a diameter of 8-10mm.Histologically,the alveolar septum is widened,with glomerulomatous hemangioma structures forming,capillary dilatation and congestion,and deposition of hemosiderin.The 1-year-old boy received conservative treatment,but no obvious clinical symptoms have emerged so far.Conservative observation and close follow-up were performed.A 28-year-old woman who underwent lobectomy had a good prognosis and good quality of life.4.A case of pulmonary papillary intralymphatic angioendothelioma(PPILA)was reported in a 60-year-old woman with no obvious symptoms.CT findings showed a high-density nodule in the lower lobe of the right lung.Microscopically,the vessels were composed of dilated vessels with lymphocytes infiltrating around the vessels.The vessels were covered with spigot-like vascular endothelial cells with obvious nuclei.The papillary proliferation of endothelial cells could be seen locally,and the hyaline stromal axis was in the center.PPILA patients survived after local surgical resection.The follow-up time has been 29 months,and the prognosis is good.Conclusion1.PEHE has the highest incidence in pulmonary vascular tumors,followed by PA,and other subtypes are rare.2.Loss of expression of CD34 and non-specific expression of CD31 can easily lead to misdiagnosis of vasogenic tumors.3.PEHE by tumor with epithelioid morphological characteristics,more easily and with the original mixed tumor and joint AE1/AE3,CD31,CD34,ERG,the FLI agreed-1 and F Ⅷ immunohistochemical index can improve the blood vessels,including PEHE,source of the diagnosis of tumors.4.Molecular typing of PEHE can be determined by YAP1-TFE3 molecular detection.