为了在长期随访后针对胰腺实性乳头状肿瘤（SPN）的临床病理、分子、生存特征和生命质量（QOL）提供全面信息，该研究回顾性地检查了2001年至2021年在北京协和医院接受手术治疗的所有SPN住院患者。 SPN是罕见的肿瘤，其恶性潜能不明确，长期预后和QOL的实质信息仍然有限。该研究共纳入了454例SPN患者，其中18.5%为男性，81.5%为女性。平均年龄为31±12岁。其中61.3％的患者没有症状。肿瘤大小为5.38±3.70厘米，83.4％为实性囊性肿瘤，40.1％有钙化。局部切除、脾切除术加或不加远端胰腺切除、十二指肠胰腺切除加或不加幽门保留的比例分别为29.7％、28.9％或22.9％和11％或6.8％。多年来，开放式手术向微创手术的转变已经显著。在所有手术中，保留幽门的十二指肠胰腺切除术的2-4级并发症发病率最高（高达32.3％），而远端胰腺切除术仅为6.7％（P <0.001）。关于组织病理学，组织浸润、神经周围瘤侵袭、癌性小血管栓塞、淋巴结转移和远处转移分别出现在16.5％、2.2％、0.7％、2.0％和3.1％的患者中。 60名患者失访。在接受切除的390名患者中，16名（4.1％）在手术后经历了局部复发或远处转移。总共有361名患者回答了电话调查。近80％的患者声称手术后其QOL没有显着影响； 但是，其余20％的患者在术后3-6年期间抱怨QOL下降。在切除后，没有临床病理学因素可以可靠地预测临床复发或转移。共检测到28个驱动基因的至少有两个肿瘤样本具有突变，并且前三个频繁突变的基因是CTNNB1，ATRNL1和MUC16。该研究提供了单个中心由最大SPN患者队列手术后的QOL报告。即使在有转移的患者中，SPN与极其有利的长期生存相关，大多数患者在手术后有良好的QOL。版权所有©2023 Wolters Kluwer Health，Inc.。保留所有权利。

To present comprehensive information on the clinicopathological, molecular, survival characteristics, and quality of life (QOL) after surgery for solid pseudopapillary neoplasm (SPN) of the pancreas in a large cohort after long-term follow-up.SPN is a rare tumor with an uncertain malignant potential, and solid information on long-term prognosis and QOL remains limited.All hospitalized patients with SPNs who underwent surgery between 2001 and 2021 at the Peking Union Medical College Hospital were retrospectively reviewed. The clinicopathological characteristics of the patients were retrieved. A cross-sectional telephone questionnaire was administered to inquire about the QOL. Molecular analyses were performed using whole-exome sequencing.Exactly 454 patients with SPN were enrolled, of whom 18.5% were male, and 81.5% were female. The mean patient age was 31±12 years. In total, 61.3% of the patients had no symptoms. The size of the tumors was 5.38±3.70 cm; 83.4% were solid cystic tumors, and 40.1% had calcifications. The proportions of local resection, distal pancreatectomy with or without splenectomy, and pancreaticoduodenectomy with or without pylorus-preservation were 29.7%, 28.9% or 22.9%, and 11% or 6.8%, respectively. Over the years, there has been a significant shift from open to minimally invasive surgery. Among all surgical procedures, pylorus-preserving pancreaticoduodenectomy had the highest incidence of grade 2 to 4 complications (up to 32.3%), compared to 6.7% in distal pancreatectomy (P<0.001). Regarding histopathology, tissue invasion, perineural invasion, cancerous microvascular emboli, lymph node metastasis, and distant metastasis were present in 16.5%, 2.2%, 0.7%, 2.0%, and 3.1% of patients, respectively. Sixty patients were lost to follow-up. Sixteen of the 390 patients who underwent resection (4.1%) experienced local recurrence or distant metastasis after surgery. In total, 361 patients responded to the telephone survey. Nearly 80% of patients claimed their QOL was not significantly affected after surgery; however, the remaining 20% complained of lower QOL during 3-6 years of follow-up after surgery. No clinicopathological factor could reliably predict clinical recurrence or metastasis after resection. A total of 28 driver genes were detected with mutations in at least two tumor samples and the top three frequently mutated genes were CTNNB1, ATRNL1, and MUC16.This study presented the largest cohort of patients with SPN after surgery from a single center and reported the QOL of these patients. SPN is associated with an extremely favorable long-term survival, even in patients with metastasis, and most patients have a good QOL after surgery.Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.