胰腺神经内分泌肿瘤(pnet):手术与观察等待的两难选择。

Harefuah Pub Date : 2022-11-01
May Shafir, Reut Harel, Veronica Sandler, Riad Haddad, Wisam Khoury, Arie Bitterman
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摘要

神经内分泌肿瘤(Neuroendocrine tumor, NEN)起源于多种器官和组织中产生激素的细胞。NEN是一种独特的肿瘤,其临床表现多样,生长方式独特,位置独特,预后较好。NEN可以是分泌性或非分泌性肿瘤。临床表现和症状根据肿瘤产生的特定激素而定。非分泌性肿瘤最终会引起与肿块效应或转移性疾病相关的症状。有各种家族性和遗传性综合征与NEN有关。最常见的神经内分泌遗传综合征是1型多发性内分泌瘤变综合征(MEN 1)。NEN的临床方法和治疗不同于任何其他癌症。金标准管理是手术,但与其他癌症疾病不同,手术干预也适用于转移性疾病。有几种手术方法,它们都取决于肿瘤的大小,位置,分级,分期,淋巴结受累,远处转移和患者的年龄和合并症。除手术外,一些病例还接受全身治疗,如生长抑素类似物、化疗、免疫治疗、靶向治疗,偶尔也使用放射治疗。在过去的十年中,由于先进的成像技术和诊断工具,诊断为小非分泌性胰腺肿瘤(PNET)的患者数量显着增加。这种偶然的增加导致了一种困境的出现,即是实施还是仅仅实施一种观望的等待政策。小的非分泌性肿瘤通常不被认为是恶性的,因此问题是手术是否总是正确的方法。必须仔细考虑手术的好处,以防止在这些复杂和激进的手术中可能发生的潜在损害。此外,新的和渐进的系统药物治疗现在可以有效地抑制肿瘤激素的分泌。最近的研究质疑手术是唯一的治疗选择,在某些情况下建议保守治疗和随访。本文献综述的目的是描述PNET的诊断工具和评估,并检查PNET治疗的不同方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[PANCREATIC NEUROENDOCRINE TUMORS (PNET): THE DILEMMA OF SURGERY VS. WATCHFUL WAITING APPROACH].

Introduction: Neuroendocrine tumors (NEN) originate from hormone producing cells located in various organs and tissues. NEN are unique tumors in terms of their diverse and particular clinical presentations, growth pattern, location and relatively good prognosis. NEN can be either secreting or non-secreting tumors. The clinical presentation and symptoms are according to the specific hormone produced by the tumor. A non-secreting tumor will eventually cause symptoms that relate to a mass-effect or a metastatic disease. There are various familial and genetic syndromes that are related to NEN. The most common neuroendocrine genetic syndrome is Multiple Endocrine Neoplasia syndrome type 1 (MEN 1). The clinical approach and treatment of NEN are unlike any other cancer. The gold standard management is surgery but unlike other cancerous diseases, surgical intervention is also indicated in cases of metastatic disease. There are several surgical approaches, and they all depend on tumor size, location, grade, stage, lymph node involvement, remote metastases and patients' age and comorbidities. Besides surgery, some cases are also treated with systemic therapies such as Somatostatin analogues, chemotherapy, immunotherapies, targeted therapies and occasionally radiation therapy is used. In the last decade there is a significant increase in the number of patients diagnosed with small non-secreting pancreatic tumors (PNET) due to advanced imaging techniques and diagnostic tools. This incidental increase is the reason for the emerging dilemma of whether to operate or merely conduct a watchful waiting policy. Small non-secreting tumors are commonly not considered malignant and thus the question is if surgery is always the right approach. The benefits of surgery must be carefully considered against the potential damage that may occur during these complex and radical procedures. Moreover, new and progressive systemic pharmacological therapies are now available to efficiently suppress tumor hormonal secretion. Recent studies have challenged surgery as the only treatment of choice, and in some cases suggest conservative treatment and follow up. The aim of this present literature review is to describe PNET diagnostic tools and evaluation, and to examine the different approaches of PNET treatment.

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