Akirov Amit, Larouche Vincent, Alshehri Sameerah, Asa Sylvia L, Ezzat Shereen
Institute of Endocrinology, Beilinson Hospital, Petach Tikva 49100, Israel.
Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel.
Cancers (Basel). 2019 Jun 14;11(6):828. doi: 10.3390/cancers11060828.
The management of pancreatic neuroendocrine tumors (PanNETs) involves classification into non-functional or functional PanNET, and as localized or metastatic PanNET. In addition, while most PanNETs are sporadic, these endocrine neoplasms can also be manifestations of genetic syndromes. All these factors may assist in forming a risk stratification system permitting a tailored management approach. Most PanNETs are classified as non-functional because they are not associated with clinical sequelae of hormone excess. They are characterized by non-specific symptoms, such as abdominal pain or weight loss, resulting from mass effect related to the pancreatic tumor or secondary to distant metastases. Accurate staging of the disease is essential for determining the appropriate approach to therapy. As cure is only potentially possible with surgical resection of the tumor, it is recommended to remove all localized and limited metastatic disease. However, many patients present with metastatic and/or advanced local disease. In such instances, the goal of therapy is to control tumor growth and/or decrease tumor burden, lengthen survival, and palliate local symptoms and those of hormone excess. This typically requires a multimodal approach, including surgery, liver-directed treatment, and systemic medical therapy.
胰腺神经内分泌肿瘤（PanNETs）的管理包括将其分类为无功能或有功能的PanNET，以及局限性或转移性PanNET。此外，虽然大多数PanNETs是散发性的，但这些内分泌肿瘤也可能是遗传综合征的表现。所有这些因素可能有助于形成一个风险分层系统，从而允许采用量身定制的管理方法。大多数PanNETs被归类为无功能，因为它们与激素过多的临床后遗症无关。它们的特征是出现非特异性症状，如腹痛或体重减轻，这些症状是由胰腺肿瘤的占位效应或远处转移继发引起的。准确的疾病分期对于确定合适的治疗方法至关重要。由于只有通过手术切除肿瘤才有可能治愈，因此建议切除所有局限性和有限的转移性疾病。然而，许多患者表现为转移性和/或局部晚期疾病。在这种情况下，治疗的目标是控制肿瘤生长和/或减轻肿瘤负担，延长生存期，并缓解局部症状和激素过多的症状。这通常需要多模式方法，包括手术、肝脏定向治疗和全身药物治疗。
