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青少年转移性胰腺母细胞瘤的成功治疗结果：一例病例报告及文献综述

Successful Outcome of Metastatic Pancreatoblastoma in an Adolescent: A Case Report and Review of the Literature.

作者信息

Jayaraman Dhaarani, John Arockia, Bose Jagadesh Chandra, Venkitaraman Balasubramanian, Shalini Sai, Murali Arunan

机构信息

Department of Pediatrics, Division of Pediatric Hemato-Oncology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India.

Department of Pediatrics, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India.

出版信息

J Indian Assoc Pediatr Surg. 2022 Nov-Dec;27(6):747-750. doi: 10.4103/jiaps.jiaps_4_22. Epub 2022 Nov 14.

DOI:10.4103/jiaps.jiaps_4_22
PMID:36714488
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9878534/
Abstract

Pancreatoblastoma, an orphan disease, is the most common malignant epithelial neoplasm of the pancreas in children. With vague clinical features, diagnosis is made by radiological suggestions and histopathology. The presence of metastatic disease and inoperable/incomplete excision remains the poor prognostic markers. We present a rare instance of an adolescent who has survived metastatic pancreatoblastoma after neoadjuvant chemoreduction/complete surgical excision.

摘要

胰腺母细胞瘤是一种罕见病，是儿童胰腺最常见的恶性上皮性肿瘤。其临床特征不明确，通过影像学提示和组织病理学进行诊断。存在转移性疾病以及无法手术切除/切除不完全仍然是预后不良的指标。我们报告了一例罕见病例，一名青少年在接受新辅助化疗减瘤/完整手术切除后，转移性胰腺母细胞瘤得以存活。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4562/9878534/6233824e1a39/JIAPS-27-747-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4562/9878534/8c933f058eb7/JIAPS-27-747-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4562/9878534/053e23a22c09/JIAPS-27-747-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4562/9878534/6233824e1a39/JIAPS-27-747-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4562/9878534/8c933f058eb7/JIAPS-27-747-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4562/9878534/053e23a22c09/JIAPS-27-747-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4562/9878534/6233824e1a39/JIAPS-27-747-g003.jpg

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Successful Outcome of Metastatic Pancreatoblastoma in an Adolescent: A Case Report and Review of the Literature.青少年转移性胰腺母细胞瘤的成功治疗结果：一例病例报告及文献综述
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本文引用的文献

1
Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors.定义和列出儿科年龄极罕见癌症：罕见癌症联合行动与欧洲儿科罕见肿瘤合作研究组合作的共识。
Eur J Cancer. 2019 Mar;110:120-126. doi: 10.1016/j.ejca.2018.12.031. Epub 2019 Feb 19.
2
Liver transplant for metastatic pancreatoblastoma: 7-year event-free survival after chemotherapy, pancreatectomy, complete hepatectomy, and liver transplant.转移性胰腺母细胞瘤的肝移植：化疗、胰腺切除、全肝切除及肝移植后的7年无事件生存期
Pediatr Transplant. 2018 Feb;22(1). doi: 10.1111/petr.13098. Epub 2017 Dec 13.
3
Pancreatic tumors in children and adolescents: the Italian TREP project experience.
儿童和青少年胰腺肿瘤：意大利 TREP 项目经验。
Pediatr Blood Cancer. 2010 May;54(5):675-80. doi: 10.1002/pbc.22385.
4
Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature.胰腺母细胞瘤合并不完全性贝克威思-维德曼综合征：病例报告及文献复习
J Pediatr Surg. 2005 Aug;40(8):1341-4. doi: 10.1016/j.jpedsurg.2005.05.025.
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Distinctive molecular genetic alterations in sporadic and familial adenomatous polyposis-associated pancreatoblastomas : frequent alterations in the APC/beta-catenin pathway and chromosome 11p.散发性和家族性腺瘤性息肉病相关的胰腺母细胞瘤中的独特分子遗传学改变：APC/β-连环蛋白途径和11号染色体p臂频繁改变
Am J Pathol. 2001 Nov;159(5):1619-27. doi: 10.1016/s0002-9440(10)63008-8.