Overview on new progress of hereditary diffuse gastric cancer withCDH1variants
| Autor: | Shu-Hui Hu, Shu-Min Xiong, Mu-Ni Hu, Huan Deng, Xing-Wei Zhang |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Cancer Research medicine.medical_specialty biology business.industry Signet ring cell Cadherin General Medicine medicine.disease medicine.disease_cause Germline CDH1 03 medical and health sciences 030104 developmental biology 0302 clinical medicine Oncology 030220 oncology & carcinogenesis medicine biology.protein Cancer research Immunohistochemistry Histopathology Hereditary diffuse gastric cancer business Carcinogenesis |
| Zdroj: | Tumori Journal. 106:346-355 |
| ISSN: | 2038-2529 0300-8916 |
| DOI: | 10.1177/0300891620949668 |
| Popis: | Hereditary diffuse gastric cancer (HDGC), comprising 1%–3% of gastric malignances, has been associated with CDH1 variants. Accumulating evidence has demonstrated more than 100 germline CDH1 variant types. E-cadherin encoded by the CDH1 gene serves as a tumor suppressor protein. CDH1 promoter hypermethylation and other molecular mechanisms resulting in E-cadherin dysfunction are involved in the tumorigenesis of HDGC. Histopathology exhibits characteristic signet ring cells, and immunohistochemical staining may show negativity for E-cadherin and other signaling proteins. Early HDGC is difficult to detect by endoscopy due to the development of lesions beneath the mucosa. Prophylactic gastrectomy is the most recommended treatment for pathogenic CDH1 variant carriers. Recent studies have promoted the progression of promising molecular-targeted therapies and management strategies. This review summarizes recent advances in CDH1 variant types, tumorigenesis mechanisms, diagnosis, and therapy, as well as clinical implications for future gene therapies. |
| Databáze: | OpenAIRE |
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