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10岁女性多中心实性假乳头状肿瘤的保留中段胰腺切除术

Middle-Preserving Pancreatectomy for Multicentric Solid Pseudopapillary Neoplasm in a 10-Year-Old Female.

作者信息

Marshall Grace, Byrne Matthew, Wirth Korry, Liao Xiaoyan, Linehan David C, Wilson Nicole A

机构信息

University of Rochester School of Medicine and Dentistry, Rochester, New York.

Department of Surgery, University of Rochester Medical Center, Rochester, New York.

出版信息

European J Pediatr Surg Rep. 2024 Oct 17;12(1):e58-e62. doi: 10.1055/s-0044-1791812. eCollection 2024 Jan.

DOI:10.1055/s-0044-1791812
PMID:39421548
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11486527/
Abstract

Solid pseudopapillary neoplasm (SPN) is a rare low-grade malignant tumor of the pancreas that occurs predominantly in young females. This tumor is occasionally multicentric, posing a unique surgical conundrum for resection. We present a case of a 10-year-old female with a history of multicystic dysplastic left kidney and persistent urogenital sinus who was diagnosed with biopsy-proven multicentric SPN of the pancreatic head and tail and underwent middle-preserving pancreatectomy. The patient tolerated the surgery very well. Our case is one of the few reported cases of multicentric SPN in a pediatric patient, and the only case treated with middle-preserving pancreatectomy, which is a novel surgical option for protecting pediatric patients from total endocrine and exocrine pancreatic insufficiency. With the increase in the incidence of SPN, there is an increasing need for pancreas-preserving surgical options, particularly in pediatric patients.

摘要

实性假乳头状肿瘤(SPN)是一种罕见的胰腺低度恶性肿瘤,主要发生于年轻女性。该肿瘤偶尔为多中心性,给手术切除带来了独特的难题。我们报告一例10岁女性病例,其有左侧多囊性发育不良肾和持续性泌尿生殖窦病史,经活检证实为胰头和胰尾多中心性SPN,并接受了保留中段的胰腺切除术。患者对手术耐受性良好。我们的病例是儿科患者中少数报道的多中心性SPN病例之一,也是唯一接受保留中段胰腺切除术治疗的病例,这是一种保护儿科患者避免出现完全性胰腺内分泌和外分泌功能不全的新型手术选择。随着SPN发病率的增加,对保留胰腺的手术选择的需求日益增加,尤其是在儿科患者中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fe3c/11486527/71faf84a6cf1/10-1055-s-0044-1791812-i2023090733cr-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fe3c/11486527/71faf84a6cf1/10-1055-s-0044-1791812-i2023090733cr-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fe3c/11486527/71faf84a6cf1/10-1055-s-0044-1791812-i2023090733cr-1.jpg

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本文引用的文献

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Pancreatic solid pseudopapillary neoplasm with concomitant left unilateral renal agenesis and bicornuate uterus: a case report.胰腺实性假乳头状瘤合并左侧单侧肾缺如及双角子宫:一例报告
Ann Med Surg (Lond). 2023 Mar 25;85(4):1166-1171. doi: 10.1097/MS9.0000000000000360. eCollection 2023 Apr.
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Solid Pseudopapillary Neoplasm of the Pancreas in a Pediatric Patient with Associated Urogenital Anomalies.一名患有泌尿生殖系统异常的儿科患者的胰腺实性假乳头状肿瘤。
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LEF1, TFE3, and AR are putative diagnostic markers of solid pseudopapillary neoplasms.淋巴细胞增强因子1(LEF1)、转录因子E3(TFE3)和雄激素受体(AR)是实性假乳头状肿瘤的潜在诊断标志物。
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Recurrence of Solid Pseudopapillary Neoplasms of the Pancreas: Results of a Nationwide Study of Risk Factors and Treatment Modalities.胰腺实性假乳头状肿瘤的复发:一项关于危险因素和治疗方式的全国性研究结果
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