Pulmonary Inflammatory Myofibroblastic Tumor: A Case Report.

IF 0.6 Q4 SURGERY
European Journal of Pediatric Surgery Reports Pub Date : 2024-11-04 eCollection Date: 2024-01-01 DOI:10.1055/a-2430-0053
Lotte Bruyninckx, Paul De Leyn, Dirk Van Raemdonck, Yanina Jansen, Katrien Coppens, Francois Vermeulen, Birgit Weynand, Christopher Gieraerts, Herbert Decaluwé
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引用次数: 0

Abstract

An inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that occurs predominantly in children and young adults. Etiology remains unclear. But based on the frequent detection of chromosomic alterations, especially near the anaplastic lymphoma kinase (ALK) gene, IMT is now considered to be a true neoplasm. In addition, the possible aggressive behavior, and the ability to metastasize suggest at least an intermediate malignant potential. Surgery remains the treatment of choice, but the use of chemotherapy, nonsteroidal anti-inflammatory drugs, immunotherapy, and targeted therapy are reported. We describe a case of a pulmonary IMT in a 6-year-old boy with an incidental finding of a lesion in the right upper lobe. A video-assisted thoracoscopic right upper lobectomy with lymph node resection was performed. Microscopic examination confirmed the diagnosis of IMT with the nodule showing spindle cells in a background of plasma cells. ALK immunohistochemical expression was negative.

肺部炎性肌纤维母细胞瘤:病例报告
炎性肌纤维母细胞瘤(IMT)是一种罕见的间充质肿瘤,主要发生在儿童和年轻人身上。其病因尚不清楚。但根据染色体组学改变的频繁发现,尤其是在无性淋巴瘤激酶(ALK)基因附近,IMT 现在被认为是一种真正的肿瘤。此外,其可能的侵袭行为和转移能力也表明它至少具有中度恶性潜能。手术仍是首选治疗方法,但也有报道称使用了化疗、非甾体抗炎药、免疫疗法和靶向疗法。我们描述了一例6岁男孩的肺IMT病例,偶然发现病变位于右上叶。患者接受了视频辅助胸腔镜右上肺叶切除术和淋巴结切除术。显微镜检查证实了 IMT 的诊断,结节显示浆细胞背景下的纺锤形细胞。ALK免疫组化表达呈阴性。
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来源期刊
自引率
33.30%
发文量
39
审稿时长
12 weeks
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