[Diagnosis and treatment of pancreatic neuroendocrine neoplasmas in Von Hippel-Lindau syndrome]. Liu, W. K., Tian, X. D., & Yang, Y. M. Zhonghua Wai Ke Za Zhi [Chinese Journal of Surgery], 56(11):869–872, November, 2018. doi abstract bibtex Von Hippel-Lindau(VHL) syndrome is a rare autosomal dominant hereditary disease, and pancreas is one of the frequently involved intra-abdominal organs, including simple pancreatic cysts, pancreatic serous cystadenomas and neuroendocrine neoplasmas. Most of the VHL-related pancreatic neuroendocrine neoplasmas (VHL-pNEN)were non-functional, but they still have a tendency to be malignant. Treatment options for VHL-pNEN include regular follow-up, surgical resection, and medication therapy. When compared with sporadic pNEN, the malignant degree of VHL-pNEN is lower, with a better prognosis, so the surgical treatment should be carefully considered. The indications of surgery for VHL-pNEN include big primary lesions (≥3 cm), fast tumor doubling time (\textless500 days), VHL gene mutation on exon 3, malignant manifestations on imaging findings, and functional pNEN lesions. The function-preserving approach should be performed to keep the functional pancreatic parenchyma as much as possible. Even for patients with a late stage malignancy that cannot be radically resected, active medication therapy may still lead to a long-term survival.
@article{liu_diagnosis_2018,
title = {[{Diagnosis} and treatment of pancreatic neuroendocrine neoplasmas in {Von} {Hippel}-{Lindau} syndrome]},
volume = {56},
issn = {0529-5815},
doi = {10.3760/cma.j.issn.0529-5815.2018.11.017},
abstract = {Von Hippel-Lindau(VHL) syndrome is a rare autosomal dominant hereditary disease, and pancreas is one of the frequently involved intra-abdominal organs, including simple pancreatic cysts, pancreatic serous cystadenomas and neuroendocrine neoplasmas. Most of the VHL-related pancreatic neuroendocrine neoplasmas (VHL-pNEN)were non-functional, but they still have a tendency to be malignant. Treatment options for VHL-pNEN include regular follow-up, surgical resection, and medication therapy. When compared with sporadic pNEN, the malignant degree of VHL-pNEN is lower, with a better prognosis, so the surgical treatment should be carefully considered. The indications of surgery for VHL-pNEN include big primary lesions (≥3 cm), fast tumor doubling time ({\textless}500 days), VHL gene mutation on exon 3, malignant manifestations on imaging findings, and functional pNEN lesions. The function-preserving approach should be performed to keep the functional pancreatic parenchyma as much as possible. Even for patients with a late stage malignancy that cannot be radically resected, active medication therapy may still lead to a long-term survival.},
language = {chi},
number = {11},
journal = {Zhonghua Wai Ke Za Zhi [Chinese Journal of Surgery]},
author = {Liu, W. K. and Tian, X. D. and Yang, Y. M.},
month = nov,
year = {2018},
pmid = {30392308},
keywords = {Pancreas, Pancreatic Neuroendocrine Tumor, pNET},
pages = {869--872},
}
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