目的:VIPoma属于神经内分泌肿瘤。这些肿瘤主要位于胰腺中并产生高水平的血管活性肠肽(VIP)。在大多数情况下,在初步诊断时已经达到转移状态,高水平的VIP导致广泛的症状。这些症状包括强烈的腹泻和随后的低钾血症,以及心脏并发症,危及生命的后果。治疗选择包括对症治疗,全身化疗和靶向治疗,还有放射和手术.由于VIPoma的发病率低,目前尚无前瞻性研究或循证治疗标准.
方法:为了评估不同治疗策略的可能影响,我们使用PubMed进行了文献研究。
结果:VIPoma的所有可能的治疗方式至少具有两个治疗目标之一:抗分泌作用(症状控制)和抗肿瘤作用(肿瘤负荷降低)。在紧急情况下,对症治疗是最重要的补水,平衡电解质并稳定患者。对症治疗在围手术期也非常重要。生长抑素类似物在症状控制中起主要作用,虽然他们的效率往往是有限的。化疗可能在一定时间内有效达到疾病稳定,尽管其对症状控制的影响有限且经常延迟。在靶向治疗选择中,舒尼替尼的使用似乎在症状控制和显示抗肿瘤作用方面最有效。辐射方面的经验仍然有限;然而,局部消融程序似乎是有希望的选择。使用放射性标记的生长抑素类似物(SSAs,177Lu-DOTATATE)提供了一种有针对性的方法,尤其是高生长抑素受体密度的患者。手术是非转移性VIPoma的一线治疗。此外,如果可以切除所有可见的肿瘤病变,在高度症状的患者中,手术方法似乎优于其他策略.手术在仅可能进行肿瘤减积的非常晚期阶段的作用仍有争议。然而,通过肿瘤缩小,然后进行生长抑素治疗,可以实现较高的立即症状控制率,虽然对生存率的影响尚不清楚.
结论:手术是非转移性VIPoma的唯一治疗选择。此外,对于症状严重的患者,手术应该是一线治疗选择,特别是如果所有肿瘤病变(原发肿瘤和转移)的切除是可以实现的。在虚弱的病人中,可以使用其他方式。
OBJECTIVE: VIPoma belongs to the group of neuroendocrine neoplasms. These tumours are located mostly in the pancreas and produce high levels of vasoactive intestinal peptide (
VIP). In most cases, a metastatic state has already been reached at the initial diagnosis, with high levels of
VIP leading to a wide spectrum of presenting symptoms. These symptoms include intense diarrhoea and subsequent hypopotassaemia but also cardiac complications, with life-threatening consequences. Treatment options include symptomatic therapy, systemic chemotherapy and targeted therapy, as well as radiation and surgery. Due to the low incidence of VIPoma, there are no prospective studies or evidence-based therapeutic standards to date.
METHODS: To evaluate the possible impact of different therapy strategies, we performed literature research using PubMed.
RESULTS: All possible treatment modalities for VIPoma have at least one of two therapy goals: antisecretory effects (symptom control) and antitumoural effects (tumour burden reduction). Symptomatic therapy is the most important in the emergency setting to rehydrate, balance electrolytes and stabilise the patient. Symptomatic therapy is also of great importance perioperatively. Somatostatin analogues play a major role in symptom control, although their efficiency is often limited. Chemotherapy may be effective in reaching stable disease for a certain time period, although its impact on symptom control is limited and often delayed. Among targeted therapy options, the usage of sunitinib appears to be the most effective in terms of symptom control and showing antitumoural effects at the same time. Experience with radiation is still limited; however, local ablative procedures seem to be promising options. Peptide receptor radiotherapy (PRRT) with radiolabelled somatostatin analogues (SSAs, 177Lu-DOTATATE) offers a targeted approach, especially in patients with high somatostatin receptor density. Surgery is the first-line therapy for nonmetastatic VIPoma. Additionally, if the resection of all visible tumour lesions is possible, the surgical approach seems preferable to other strategies in highly symptomatic patients. The role of surgery in very advanced stages where only tumour debulking is possible remains debatable. However, a high rate of immediate symptom control can be achieved by tumour debulking followed by somatostatin therapy, although the impact on survival remains unclear.
CONCLUSIONS: Surgery is the only curative option for nonmetastatic VIPoma. Additionally, surgery should be a first-line therapy option for highly symptomatic patients, especially if the resection of all tumour lesions (primary tumour and metastasis) is achievable. In frail patients, other modalities can be used.