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实性假乳头状肿瘤:单机构罕见胰腺肿瘤病例系列

Solid Pseudopapillary Neoplasm: A Single Institutional Case Series of a Rare Pancreatic Tumor.

作者信息

Oase Kristen, Cheryl Meguid, Oba Atsushi, Al-Musawi Mohammed H, Sheridan Alison, Norris Evan, Mehrotra Sanjana, Lovell Mark A, Schulick Richard D, Ahrendt Steven A, Del Chiaro Marco

机构信息

From University of Colorado Hospital, Aurora, Colorado.

Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan.

出版信息

J Adv Pract Oncol. 2022 Jul;13(5):497-505. doi: 10.6004/jadpro.2022.13.5.3. Epub 2022 Jul 27.

DOI:10.6004/jadpro.2022.13.5.3
PMID:35910503
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9328450/
Abstract

PURPOSE

Solid pseudopapillary neoplasms (SPN) are rare pancreatic cystic neoplasms with low malignant potential that tend to occur in young women. Due to the rarity of this disease, there are few large case series in the literature, and the exact pathophysiology remains unknown. In this article, we aim to share our institutional experience.

METHODS

Retrospective clinical data collection and analysis was performed on all patients with a diagnosis of SPN at the University of Colorado Hospital and Children's Hospital of Colorado (n = 28).

RESULTS

Twenty-eight patients were diagnosed with SPN during the study period. The median age was 21.5 years, and the majority of patients were female (89.3%) and Caucasian (60.7%). Six patients were diagnosed incidentally (21.4%). The majority of tumors were in the pancreatic tail (46.4%), and most underwent distal pancreatectomy (64.3%). The mean tumor size was 5.4 cm, and R0 resection was achieved in 25 patients (89.3%). Ten patients underwent laparoscopic resection (35.7%). The median hospital length of stay was 8.5 days, and postoperative complication rate was 39.3%. Median follow-up was 41 months, with 78.6% of patients alive without evidence of disease, while 2 patients were lost to follow-up. Two patients developed recurrence/metastases, which were resected; both are alive without evidence of disease.

CONCLUSION

SPN are rare pancreatic tumors diagnosed most frequently in young women. Surgical resection is the mainstay of treatment, and outcomes are excellent if complete resection is achieved. Predictors of malignant disease are inconsistent in current literature. Considerations should be made for a minimally invasive approach in patients with SPN. Multidisciplinary clinics may be helpful in the diagnosis, management, and surveillance of pancreatic cystic lesions, with major potential for the advanced practitioner role.

摘要

目的

实性假乳头状肿瘤(SPN)是一种罕见的胰腺囊性肿瘤,恶性潜能低,多见于年轻女性。由于该疾病罕见,文献中大型病例系列较少,确切的病理生理学仍不清楚。在本文中,我们旨在分享我们机构的经验。

方法

对科罗拉多大学医院和科罗拉多儿童医院所有诊断为SPN的患者(n = 28)进行回顾性临床数据收集和分析。

结果

在研究期间,28例患者被诊断为SPN。中位年龄为21.5岁,大多数患者为女性(89.3%)和白种人(60.7%)。6例患者为偶然诊断(21.4%)。大多数肿瘤位于胰尾(46.4%),大多数患者接受了胰体尾切除术(64.3%)。肿瘤平均大小为5.4 cm,25例患者(89.3%)实现了R0切除。10例患者接受了腹腔镜切除术(35.7%)。中位住院时间为8.5天,术后并发症发生率为39.3%。中位随访时间为41个月,78.6%的患者存活且无疾病证据,2例患者失访。2例患者出现复发/转移,均接受了手术切除;两人均存活且无疾病证据。

结论

SPN是罕见的胰腺肿瘤,最常见于年轻女性。手术切除是主要治疗方法,如果实现完全切除,预后良好。目前文献中恶性疾病的预测指标并不一致。对于SPN患者,应考虑采用微创方法。多学科诊所可能有助于胰腺囊性病变的诊断、管理和监测,对高级从业者角色具有重大潜力。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a1e/9328450/c1658732c447/jadpro-13-497-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a1e/9328450/cc91880a9af4/jadpro-13-497-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a1e/9328450/010012316be4/jadpro-13-497-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a1e/9328450/9e790f3e8c5c/jadpro-13-497-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a1e/9328450/c1658732c447/jadpro-13-497-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a1e/9328450/cc91880a9af4/jadpro-13-497-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a1e/9328450/010012316be4/jadpro-13-497-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a1e/9328450/9e790f3e8c5c/jadpro-13-497-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3a1e/9328450/c1658732c447/jadpro-13-497-g004.jpg

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