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幼儿腹部纤维瘤病:病例研究及文献综述

Abdominal fibromatosis in a young child: a case study and review of the literature.

作者信息

Chu Hyun Hee, Hwang Pyoung Han, Jeong Yeon Jun, Chung Myoung Ja

机构信息

Department of Pathology, Chonbuk National University Medical School, Jeonju, Korea.

出版信息

Korean J Pathol. 2013 Oct;47(5):472-6. doi: 10.4132/KoreanJPathol.2013.47.5.472. Epub 2013 Oct 25.

DOI:10.4132/KoreanJPathol.2013.47.5.472
PMID:24255636
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3830995/
Abstract

Fibromatoses comprise many different entities of well-differentiated fibroblastic proliferation with variable collagen production and form a firm nodular mass. Abdominal fibromatosis is distinguishable from other forms of fibromatosis because of its location and its tendency to occur in women of childbearing age during or following pregnancy. Abdominal fibromatosis in children is an extremely rare condition. A 15-month-old boy presented with an abdominal wall mass that had recently increased in size. Mass excision was perfomed. The tumor was 4.3×4.1 cm and partly circumscribed. Histologically, the tumor was composed of parallel long fascicles of spindle-cells with a uniform appearance. The edges of the resected mass were infiltrative, and the surgical margins were positive. Mitotic figures were <1/10 high power fields. No cellular atypia or necrosis was present. The tumor cells were positive for vimentin and nuclear β-catenin staining.

摘要

纤维瘤病由许多不同的、分化良好的成纤维细胞增殖实体组成,胶原产生情况各异,并形成坚实的结节状肿块。腹壁纤维瘤病因其位置以及在育龄期女性孕期或产后发病的倾向,有别于其他形式的纤维瘤病。儿童腹壁纤维瘤病极为罕见。一名15个月大的男童出现腹壁肿块,且肿块近期增大。进行了肿块切除术。肿瘤大小为4.3×4.1厘米,部分边界清晰。组织学上,肿瘤由外观一致的平行长梭形细胞束组成。切除肿块的边缘呈浸润性,手术切缘阳性。有丝分裂象<1/10高倍视野。未发现细胞异型性或坏死。肿瘤细胞波形蛋白和核β-连环蛋白染色呈阳性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2094/3830995/d34f4fef3d80/kjpathol-47-472-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2094/3830995/1aacaec77816/kjpathol-47-472-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2094/3830995/d34f4fef3d80/kjpathol-47-472-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2094/3830995/1aacaec77816/kjpathol-47-472-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2094/3830995/d34f4fef3d80/kjpathol-47-472-g002.jpg

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本文引用的文献

1
Desmoid tumors: clinical features and treatment options for advanced disease.韧带样瘤:晚期疾病的临床特征和治疗选择。
Oncologist. 2011;16(5):682-93. doi: 10.1634/theoncologist.2010-0281. Epub 2011 Apr 8.
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Aggressive fibromatosis in children and adolescents: the Italian experience.儿童和青少年侵袭性纤维瘤病:意大利经验。
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Nuclear beta-catenin expression distinguishes deep fibromatosis from other benign and malignant fibroblastic and myofibroblastic lesions.细胞核β-连环蛋白表达可将深部纤维瘤病与其他良性及恶性成纤维细胞和肌成纤维细胞病变区分开来。
Am J Surg Pathol. 2005 May;29(5):653-9. doi: 10.1097/01.pas.0000157938.95785.da.
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Aggressive fibromatosis from infancy to adolescence.从婴儿期到青春期的侵袭性纤维瘤病。
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Low-dose chemotherapy with vinblastine and methotrexate in childhood desmoid tumors.长春花碱和甲氨蝶呤低剂量化疗用于儿童硬纤维瘤
J Clin Oncol. 1999 Mar;17(3):1086. doi: 10.1200/JCO.1999.17.3.1086.
10
Combination chemotherapy using vinblastine and methotrexate for the treatment of progressive desmoid tumor in children.使用长春碱和甲氨蝶呤联合化疗治疗儿童进行性硬纤维瘤病。
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