دورية أكاديمية
Aggressive versus indolent insulinomas: new clinicopathological insights
العنوان: | Aggressive versus indolent insulinomas: new clinicopathological insights |
---|---|
المؤلفون: | Hackeng, Wenzel M, Brosens, Lodewijk A A, Dreijerink, Koen M A |
المصدر: | Endocrine-Related Cancer ; volume 30, issue 5 ; ISSN 1351-0088 1479-6821 |
بيانات النشر: | Bioscientifica |
سنة النشر: | 2023 |
الوصف: | Insulinomas are rare functional pancreatic neuroendocrine tumors. While most insulinomas are indolent and cured after surgery, 10–15% of cases show aggressive or malignant tumor behavior and metastasize locally or to distant organs. Patients with metastatic insulinoma survive significantly shorter. Recognizing aggressive insulinomas can help to predict prognosis, guide therapy and determine follow-up intensity after surgery. This review offers a summary of the literature on the significant clinical, pathological, genetic and epigenetic differences between indolent and aggressive insulinomas. Aggressive insulinomas are characterized by rapid onset of symptoms, larger size, expression of ARX and alpha-1-antitrypsin and decreased or absent immunohistochemical expression of insulin, PDX1 and GLP-1R. Moreover, aggressive insulinomas often harbor ATRX or DAXX mutations, the alternative lengthening of telomeres phenotype and chromosomal instability. Tumor grade and MEN1 and YY1 mutations are less useful for predicting behavior. Aggressive insulinomas have similarities to normal alpha-cells and non-functional pancreatic neuroendocrine tumors, while indolent insulinomas remain closely related to normal beta-cells. In conclusion, indolent and aggressive insulinoma are different entities, and distinguishing these will have future clinical value in determining prognosis and treatment. |
نوع الوثيقة: | article in journal/newspaper |
اللغة: | unknown |
DOI: | 10.1530/erc-22-0321 |
الإتاحة: | https://doi.org/10.1530/erc-22-0321Test https://erc.bioscientifica.com/view/journals/erc/30/5/ERC-22-0321.xmlTest https://erc.bioscientifica.com/downloadpdf/journals/erc/30/5/ERC-22-0321.xmlTest |
رقم الانضمام: | edsbas.D8F58245 |
قاعدة البيانات: | BASE |
DOI: | 10.1530/erc-22-0321 |
---|