囊性胰腺神经内分泌肿瘤生物学行为特征及诊断与治疗策略
Biological behavior characteristics and diagnosis and treatment strategies of cystic pancreatic neuroendocrine tumors
摘要胰腺神经内分泌肿瘤(pNETs)是一类异质性较高的肿瘤,多为实性,囊性较少。近年来随着CT、MRI等断层影像学检查技术的发展和广泛应用,极大地提高了无症状囊性pNETs的检出率。目前,囊性pNETs的病因仍不明确,存在多种假说,但尚缺乏研究结果验证。与实性pNETs比较,囊性pNETs多为无功能性和无症状,生物学行为更为惰性,具有较低的Ki-67增殖指数和核分裂象,较少发生淋巴结和远处器官转移,其长期预后亦优于实性pNETs,但应警惕仍有部分囊性pNETs具有侵袭性的生物学行为。囊性pNETs无特异性影像学特征,术前定性诊断仍具有挑战性。超声内镜检查联合细针穿刺可极大提高囊性pNETs诊断的灵敏度与特异度。应根据患者临床症状、肿瘤部位、肿瘤大小、囊性成分比例及危险因素分析等综合评估后个性化制订囊性pNETs治疗方案。手术是囊性pNETs的标准治疗方法,但鉴于其相对惰性的生物学行为,对于无功能、完全囊性、直径≤2 cm的囊性pNETs亦可考虑定期随诊。笔者结合团队经验及相关文献,对囊性pNETs的病因、临床特征及其诊断与治疗策略进行探讨,旨在为广大外科医师临床治疗提供参考。
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abstractsPancreatic neuroendocrine tumors (pNETs) are a highly heterogeneous group of neoplasms, which are predominantly solid and rarely present as cystic lesions. Due to the development and widespread use of cross-sectional imaging, such as CT and MRI examination, the detection rate of asymptomatic cystic pNETs has dramatically risen in the past decade. The etiology of cystic pNETs remains unclear. Despite several proposed hypothesis, the current studies still show contradictory results. Compared with solid pNETs, cystic pNETs are more likely to be non-functional and asymptomatic, and present with indolent biological behavior, such as lower Ki-67 proliferation index and mitotic count, less lymph node involvement and distant organ metastasis. The long-term prognosis of patients with cystic pNETs is also better compared with those with solid pNETs. However, a small part of cystic pNETs have invasive biological behavior. Preoperative diagnosis of this disease is challenging owing to the lack of specific imaging features. Endoscopic ultrasonography combined with fine needle aspiration can significantly improve the sensitivity and specificity in diagnosis of cystic pNETs. For its treatment strategy, all following factors, including clinical symptoms, tumor location, tumor size, degree of cystic component should be considered compre-hensively to individually make decision. Surgical resection is the standard procedure of cystic pNETs. Owing to its indolent behaviors, observation and active surveillance should be considered as an alternative to surgery for patients with non-functional, purely cystic and diameter less than 2 cm cystic pNETs. Combined with their own experience and relative literature, the authors make an investigation on the etiology, clinical characteristics, diagnosis and treatment strategies of cystic pNETs to provide reference for clinical treatment of surgeons.
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