Objective·To investigate the clinical,pathological,and molecular genetic features and prognosis of epithelioid hemangioendothelioma(EHE)patients.Methods·Clinical and follow-up data of 12 EHE patients diagnosed at Renji Hospital,Shanghai Jiao Tong University School of Medicine from September 2016 to December 2023 were collected.Tissue samples were analyzed using hematoxylin-eosin(H-E)staining,immunohistochemistry(IHC),and fluorescence in situ hybridization(FISH).Results·Among the 12 patients,there were 3 males and 9 females,with a mean age of(47.17±11.15)years.Tumors were located in the liver(6 cases),lung(4 cases),mediastinum(1 case),and supraclavicular region(1 case).Nine patients were asymptomatic,while 3 presented with mild symptoms such as chest tightness and fatigue.CT imaging revealed that EHE patients with involvement of livers and lungs exhibited multiple nodules,and 2 cases had tumors in both organs.Patients with tumors in the supraclavicular region and mediastinum presented with solitary nodules.H-E staining demonstrated that tumor tissues were composed of epithelioid,dendritic,and intermediate cells,arranged in acinar,cord-like,or clustered patterns.Epithelioid cells had round vesicular nuclei and eosinophilic cytoplasm,with some showing a signet-ring appearance and cytoplasmic vacuoles.The stroma contained a mucoid matrix.IHC staining revealed that mesenchymal endothelial markers,including vimentin,CD31,ETS transcription factor ERG,and factor Ⅷ-related antigen,were positive in the tumor tissues,while epithelial markers showed low positivity with weak staining.The Ki-67 indexes were also low.FISH analysis showed that 10 patients had a calmodulin-binding transcription activator 1(CAMTA1)gene break,while 2 patients had a transcription factor E3(TFE3)gene break.Of the 12 patients,11 were followed up for 2 to 38 months,with a mean follow-up time of 21.7 months.Three patients achieved tumor-free survival,6 were alive with tumors,1 died 4 months after surgery,and 1 died of heart disease 24 months after surgery.Conclusion·EHE has atypical clinical features,a tendency to recur,a and variable prognosis.Accurate diagnosis requires a combination of histopathology,IHC,and a molecular testing.

Objective·To investigate the clinical,pathological,and molecular genetic features and prognosis of epithelioid hemangioendothelioma(EHE)patients.Methods·Clinical and follow-up data of 12 EHE patients diagnosed at Renji Hospital,Shanghai Jiao Tong University School of Medicine from September 2016 to December 2023 were collected.Tissue samples were analyzed using hematoxylin-eosin(H-E)staining,immunohistochemistry(IHC),and fluorescence in situ hybridization(FISH).Results·Among the 12 patients,there were 3 males and 9 females,with a mean age of(47.17±11.15)years.Tumors were located in the liver(6 cases),lung(4 cases),mediastinum(1 case),and supraclavicular region(1 case).Nine patients were asymptomatic,while 3 presented with mild symptoms such as chest tightness and fatigue.CT imaging revealed that EHE patients with involvement of livers and lungs exhibited multiple nodules,and 2 cases had tumors in both organs.Patients with tumors in the supraclavicular region and mediastinum presented with solitary nodules.H-E staining demonstrated that tumor tissues were composed of epithelioid,dendritic,and intermediate cells,arranged in acinar,cord-like,or clustered patterns.Epithelioid cells had round vesicular nuclei and eosinophilic cytoplasm,with some showing a signet-ring appearance and cytoplasmic vacuoles.The stroma contained a mucoid matrix.IHC staining revealed that mesenchymal endothelial markers,including vimentin,CD31,ETS transcription factor ERG,and factor Ⅷ-related antigen,were positive in the tumor tissues,while epithelial markers showed low positivity with weak staining.The Ki-67 indexes were also low.FISH analysis showed that 10 patients had a calmodulin-binding transcription activator 1(CAMTA1)gene break,while 2 patients had a transcription factor E3(TFE3)gene break.Of the 12 patients,11 were followed up for 2 to 38 months,with a mean follow-up time of 21.7 months.Three patients achieved tumor-free survival,6 were alive with tumors,1 died 4 months after surgery,and 1 died of heart disease 24 months after surgery.Conclusion·EHE has atypical clinical features,a tendency to recur,a and variable prognosis.Accurate diagnosis requires a combination of histopathology,IHC,and a molecular testing.

Objective:To investigate the effect of botulinum toxin type A on children with odorihidrosis.Methods:From March 2017 to February 2021, 121 children with odorihidrosis, including 48 males and 73 females, aged 13 to 17 (15.9±1.2) years, were admitted to the Burn and Plastic Surgery Department of the 980 Hospital of PLA. There were 24 cases in mild group, 50 cases in moderate group and 47 cases in severe group. Botulinum toxin A was injected into 20-50 points on each side, and 1 U was injected into each point. The total amount of botulinum toxin A was 50-100 U on both sides.Results:Three groups of children were evaluated for efficacy, 24 cases of mild group was significantly effective in 23 cases, accounting for 95.8%. In the moderate group, 46 (92.0%) of 50 cases showed obvious effect. 49 cases (98.0%) were effective; In the severe group, 40 cases (85.1%) showed obvious effect and 45 cases (95.7%) were effective. Three groups of children with different efficacy had no statistical significance ( P>0.05). The significant efficiency in mild and moderate groups was higher than that in severe group, and the difference was statistically significant ( P<0.05). Conclusions:Botulinum toxin type A is effective in the treatment of children with mild and moderate bromhidrosis, and is worthy of clinical application.