Department of General Surgery, Key Laboratory of Research in Pancreatic Tumor, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Dongcheng, Beijing, China.
Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Dongcheng, Beijing, China.
Ann Surg. 2023 Dec 1;278(6):1009-1017. doi: 10.1097/SLA.0000000000005842. Epub 2023 Apr 10.
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 males and 81.5% were females. 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 (PPPD) had the highest incidence of grade 2 to 4 complications (up to 32.3%), compared with 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 to 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 2 tumor samples and the top 3 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 extremely favorable long-term survival, even in patients with metastasis, and most patients have a good QOL after surgery.
通过长期随访，在一个大型队列中报告胰腺实性假乳头状瘤（SPN）患者的临床病理、分子、生存特征和手术后生活质量（QOL）。
SPN 是一种具有不确定恶性潜能的罕见肿瘤，关于长期预后和 QOL 的可靠信息仍然有限。
回顾性分析 2001 年至 2021 年期间在北京协和医学院医院接受手术治疗的所有 SPN 住院患者。检索患者的临床病理特征。通过横断面电话问卷调查询问 QOL。使用全外显子组测序进行分子分析。
共纳入 454 例 SPN 患者，其中男性占 18.5%，女性占 81.5%。患者平均年龄为 31±12 岁。共有 61.3%的患者无症状。肿瘤大小为 5.38±3.70cm；83.4%为实性囊性肿瘤，40.1%有钙化。局部切除术、远端胰腺切除术伴或不伴脾切除术、胰十二指肠切除术伴或不伴幽门保留术的比例分别为 29.7%、28.9%或 22.9%和 11%或 6.8%。多年来，手术方式已从开放手术明显转变为微创手术。在所有手术中，保留幽门的胰十二指肠切除术（PPPD）的 2 级至 4 级并发症发生率最高（高达 32.3%），而远端胰腺切除术为 6.7%（P<0.001）。在组织病理学方面，组织侵犯、神经周围侵犯、癌微血管栓塞、淋巴结转移和远处转移分别见于 16.5%、2.2%、0.7%、2.0%和 3.1%的患者。60 例患者失访。390 例接受切除术的患者中有 16 例（4.1%）术后出现局部复发或远处转移。共有 361 例患者对电话调查做出了回应。近 80%的患者表示手术后生活质量没有明显受影响；然而，其余 20%的患者在手术后 3 至 6 年的随访中抱怨生活质量较低。没有临床病理因素可以可靠地预测手术后的局部复发或转移。共检测到 28 个突变至少在 2 个肿瘤样本中存在驱动基因，前 3 个常突变基因是 CTNNB1、ATRNL1 和 MUC16。
本研究报告了来自单一中心的最大胰腺 SPN 手术后患者队列，并报告了这些患者的 QOL。SPN 与极好的长期生存相关，即使在发生转移的患者中也是如此，大多数患者在手术后生活质量良好。
