Dralle Henning, Krohn Sabine L, Karges Wolfram, Boehm Bernhard O, Brauckhoff Michael, Gimm Oliver
Department of General, Visceral, and Vascular Surgery, Medical Faculty, University of Halle-Wittenberg, Halle/Saale, Ernst-Grube-Strasse 40, D-06097 Halle, Germany.
World J Surg. 2004 Dec;28(12):1248-60. doi: 10.1007/s00268-004-7609-8. Epub 2004 Nov 11.
Nonfunctioning neuroendocrine pancreatic tumors (NFNEPTs) comprise about one-third of pancreatic endocrine tumors. Based on immunohistochemistry, nonfunctioning tumors are difficult to distinguish from functioning ones; therefore the final diagnosis is basically the result of a synopsis of pathology and clinical data. Owing to their incapacity to produce hormone-dependent symptoms, NFNEPTs are detected incidentally or because of uncharacteristic symptoms resulting from local or distant growth. About two-thirds of NFNEPTs are located in the pancreatic head, so jaundice may be a late symptom of this tumor. Modern diagnostic procedures are best applied by a stepwise approach: first endoscopic ultrasonography and computed tomography/magnetic resonance imaging followed by somatostatin receptor scintigraphy or positron emission tomography (or both). Due to significant false-positive and false-negative findings, for decision-making the latter should be confirmed by a second imaging modality. Regarding indications for surgery and the surgical approach to the pancreas, three pancreatic manifestations of NFNEPTs can be distinguished: (1) solitary benign non-multiple endocrine neoplasia type 1 (non-MEN-1); (2) multiple benign MEN-1; and (3) malignant NFNEPTs. Reviewing the literature and including our experience with 18 NFNEPTs (8 benign, 10 malignant) reported here, the following conclusions can be drawn: (1) Solitary benign non-MEN-1 NFNEPTs can be removed by enucleation or by pancreas-, spleen-, and duodenum-preserving techniques in most cases. The choice of surgical technique depends on the location and site of the tumor and its anatomic relation to the pancreatic duct. (2) With multiple benign MEN-1 NFNEPTs, because of the characteristics of the underlying disease a preferred, more conservative concept (removal of only macrolesions) competes with a more radical procedure (left pancreatic resection with enucleation of head macrolesions). Further studies are necessary to clarify the best way to balance quality of life (by preserving organ function) with growth control of potentially malignant tumors in the pancreas. (3) Malignant NFNEPTs comprise more than half of all NFNEPTs. Few studies have analyzed treatment strategies for localized or metastatic tumors. Whereas radical (including multivisceral) resection of tumors without distant metastases is widely accepted, the indication for radical surgery on metastasizing tumors has been questioned, as radical removal of the primary tumor may fail to increase survival. Adjuvant regimens in these tumor stages are mandatory and should be further optimized.
无功能性神经内分泌胰腺肿瘤（NFNEPTs）约占胰腺内分泌肿瘤的三分之一。基于免疫组织化学，无功能性肿瘤很难与功能性肿瘤区分开来；因此，最终诊断基本上是病理和临床数据综合分析的结果。由于它们无法产生激素依赖性症状，NFNEPTs是偶然发现的，或者是由于局部或远处生长导致的非特异性症状而被发现。约三分之二的NFNEPTs位于胰头，因此黄疸可能是该肿瘤的晚期症状。现代诊断程序最好采用逐步推进的方法：首先是内镜超声检查和计算机断层扫描/磁共振成像，然后是生长抑素受体闪烁扫描或正电子发射断层扫描（或两者）。由于存在显著的假阳性和假阴性结果，为了做出决策，后者应通过第二种成像方式进行确认。关于手术指征和胰腺的手术方式，NFNEPTs的三种胰腺表现可以区分：（1）孤立性良性非多发性内分泌肿瘤1型（非MEN-1）；（2）多发性良性MEN-1；（3）恶性NFNEPTs。回顾文献并结合我们在此报告的18例NFNEPTs（8例良性，10例恶性）的经验，可以得出以下结论：（1）大多数情况下，孤立性良性非MEN-1 NFNEPTs可以通过剜除术或保留胰腺、脾脏和十二指肠的技术切除。手术技术的选择取决于肿瘤的位置和部位及其与胰管的解剖关系。（2）对于多发性良性MEN-1 NFNEPTs，由于基础疾病的特点，一种更倾向于保守的观念（仅切除大的病变）与一种更激进的手术方式（左胰腺切除并剜除胰头大的病变）相互竞争。需要进一步研究以阐明在保留器官功能（从而提高生活质量）与控制胰腺潜在恶性肿瘤生长之间取得平衡的最佳方法。（3）恶性NFNEPTs占所有NFNEPTs的一半以上。很少有研究分析局部或转移性肿瘤的治疗策略。虽然对于无远处转移的肿瘤进行根治性（包括多脏器）切除已被广泛接受，但对于转移性肿瘤进行根治性手术的指征受到质疑，因为切除原发肿瘤可能无法提高生存率。在这些肿瘤阶段，辅助治疗方案是必需的，并且应该进一步优化。
