Liu Jiawei, Ling Jian, Li Lujie, Wu Yuxin, Song Chenyu, Shi Siya, Dong Zhi, Wang Jifei, Tang Mimi, Feng Shi-Ting, Luo Yanji, Xu Danyang
Department of Radiology, The First Affiliated Hospital, Sun Yat-Sen University, No.58, Second Zhongshan Road, Yuexiu District, Guangzhou, Guangdong, 510080, China.
Abdom Radiol (NY). 2024 Dec 19. doi: 10.1007/s00261-024-04764-0.
Pancreatic neuroendocrine neoplasms (pNENs) are the second most common pancreatic malignancy. While most cases are sporadic, a small proportion is associated with genetic syndromes, such as Multiple Endocrine Neoplasia (MEN), Von Hippel-Lindau Syndrome (VHL), Neurofibromatosis Type 1 (NF1), and Tuberous Sclerosis Complex (TSC). This review aims to use pNENs as a clue to reveal the full spectrum of disease, providing a comprehensive understanding of diagnosis. It aids in promptly identifying abnormalities in other organs, recognizing familial genetic mutations, and achieving personalized treatment.
胰腺神经内分泌肿瘤（pNENs）是第二常见的胰腺恶性肿瘤。虽然大多数病例是散发性的，但一小部分与遗传综合征有关，如多发性内分泌腺瘤病（MEN）、冯·希佩尔-林道综合征（VHL）、1型神经纤维瘤病（NF1）和结节性硬化症（TSC）。本综述旨在以pNENs为线索揭示疾病的全貌，提供对诊断的全面理解。它有助于及时发现其他器官的异常，识别家族性基因突变，并实现个性化治疗。
