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与疑似同步胃肠道间质瘤难以区分的同步纤维瘤病:病例报告。

Synchronous Fibromatosis Indistinguishable from Suspected Synchronous Gastrointestinal Stromal Tumor: A Case Report.

机构信息

Department of Surgery, Nepal Medical College and Teaching Hospital, Attarkhel, Kathmandu, Nepal.

Department of Pathology, Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal.

出版信息

JNMA J Nepal Med Assoc. 2021 Sep 11;59(241):919-921. doi: 10.31729/jnma.6516.

DOI:10.31729/jnma.6516
PMID:35199727
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9107889/
Abstract

Desmoid tumors most commonly occur in the anterior abdominal wall in approximately 50% of cases and are locally aggressive. We describe a case of a 38-year-old lady who was investigated as a case of gastrointestinal tumor. Post-operative immunohistochemistry staining showed the presence of a synchronous desmoid in the abdominal wall and proximal ileum. Wide local excision remains the gold-standard of treatment with pharmacotherapeutics and radiotherapy serving as adjuvant or palliative treatment options.

摘要

纤维瘤病最常发生在前腹壁,约占 50%病例,且具有局部侵袭性。我们描述了一例 38 岁女性,她被诊断为胃肠道肿瘤。术后免疫组化染色显示腹壁和回肠近端存在同步纤维瘤病。广泛局部切除仍然是治疗的金标准,药物治疗和放射治疗作为辅助或姑息治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f74/9107889/8196ed6f63af/JNMA-59-241-919-g3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f74/9107889/55b73c81354b/JNMA-59-241-919-g1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f74/9107889/d0360869739a/JNMA-59-241-919-g2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f74/9107889/8196ed6f63af/JNMA-59-241-919-g3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f74/9107889/55b73c81354b/JNMA-59-241-919-g1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f74/9107889/d0360869739a/JNMA-59-241-919-g2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0f74/9107889/8196ed6f63af/JNMA-59-241-919-g3.jpg

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本文引用的文献

1
Intra-Abdominal and Abdominal Wall Desmoid Fibromatosis.腹内及腹壁硬纤维瘤病
Oncol Ther. 2016;4(1):57-72. doi: 10.1007/s40487-016-0017-z. Epub 2016 Feb 3.
2
Intra-abdominal desmoid tumor difficult to distinguish from a gastrointestinal stromal tumor: report of two cases.难以与胃肠道间质瘤相鉴别的腹腔内硬纤维瘤:两例报告
Surg Today. 2014 Nov;44(11):2174-9. doi: 10.1007/s00595-013-0681-7. Epub 2013 Aug 20.
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Giant desmoid tumor of the anterior abdominal wall in a young female: a case report.一名年轻女性的前腹壁巨大硬纤维瘤:病例报告
Case Rep Surg. 2013;2013:780862. doi: 10.1155/2013/780862. Epub 2013 Apr 21.
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Gastrointestinal stromal tumors.胃肠道间质瘤。
Gastroenterol Clin North Am. 2013 Jun;42(2):399-415. doi: 10.1016/j.gtc.2013.01.001. Epub 2013 Mar 13.
5
CTNNB1 mutation analysis is a useful tool for the diagnosis of desmoid tumors: a study of 260 desmoid tumors and 191 potential morphologic mimics.CTNNB1 突变分析是诊断韧带样瘤的有用工具:260 例韧带样瘤和 191 例潜在形态模拟物的研究。
Mod Pathol. 2012 Dec;25(12):1551-8. doi: 10.1038/modpathol.2012.115. Epub 2012 Jul 6.
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Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells.侵袭性纤维瘤病(硬纤维瘤)来源于间充质祖细胞。
Cancer Res. 2010 Oct 1;70(19):7690-8. doi: 10.1158/0008-5472.CAN-10-1656. Epub 2010 Sep 14.
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The surgical management of soft tissue tumours arising in the abdominal wall.腹壁软组织肿瘤的外科治疗。
Eur J Surg Oncol. 2010 May;36(5):489-95. doi: 10.1016/j.ejso.2010.03.007. Epub 2010 Apr 9.
8
Clinical significance of oncogenic KIT and PDGFRA mutations in gastrointestinal stromal tumours.致癌性KIT和PDGFRA突变在胃肠道间质瘤中的临床意义
Histopathology. 2008 Sep;53(3):245-66. doi: 10.1111/j.1365-2559.2008.02977.x. Epub 2008 Feb 28.
9
Soft tissue tumors of the abdominal wall: analysis of disease patterns and treatment.腹壁软组织肿瘤:疾病模式与治疗分析
Arch Surg. 2001 Jan;136(1):70-9. doi: 10.1001/archsurg.136.1.70.
10
The enigma of desmoid tumors.韧带样瘤之谜。
Ann Surg. 1999 Jun;229(6):866-72; discussion 872-3. doi: 10.1097/00000658-199906000-00014.