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Macau Periodical Index (澳門期刊論文索引)
- Author
- Lin, Yi；Hlaing, Thazin；Lam, Chi Leong；Wong, Sio In
- Title
- A case report of pulmonary epithelioid hemangioendothelioma
- Journal Name
- 澳門醫學雜誌
- Pub. Info
- 2024年1月31日, 第11卷第1期, pp. 82-85
- Link
- https://www.am.gov.mo/cn/journal/file/v11_1.pdf
- Keyword
- Epithelioid hemangioendothelioma；Immune checkpoint inhibitor；Anti-angiogenesis TKI(tyrosine kinase inhibitor)
- Abstract
- Summary: Pulmonary epithelioid hemangioendothelioma (EHE) is a rare neoplasm with variable clinical behaviour. It can manifest as multiple pulmonary nodules, pulmonary reticulonodular opacities, or diffuse infiltrative pleural thickening. Currently, there are no approved systemic agents specifically for EHE,and treatment selection remains uncertain. We present a case of a female patient with EHE who initially presented with lung lesions and pleural effusion, later found to have intrahepatic lesions. Molecular assay confirmed the presence of the WWTR1-CAMTA1 fusion gene, highly specific for EHE. The patient was treated with a regimen consisting of the immune checkpoint inhibitor Pembrolizumab and the antiangiogenesis tyrosine kinase inhibitor Axitinib. Her dyspnea improved during the course of treatment. The response to this regimen will be assessed using follow-up imaging. It is important to identify patients with EHE harbouring specific fusion genes for treatment response analysis. Further studies are needed due to the rarity of EHE, and uncontrolled trials may be inevitable. Paragraph Headings: 1. Introduction 2. Case presentation 3. Discussion 4. Conclusion Figures： 1. Histopathologic findings of hemangioendothelioma: (a) Higher magnification of the tumor reveals vacuolation of some of the tumor cells, representing primitive angiogenesis. (b) Immunostaining for Vimentin revealed positivity of the neoplastic cells (brown colour), confirming the mesenchymal origin of tumor cells (c) Immunostaining for CD34 revealed positivity of the neoplastic cells (brown colour),confirming the endothelial lineage of the tumor. (d) Immunostaining for ERG revealed positivity of the neoplastic cells (brown colour), further confirming the endothelial lineage of the tumor.