Bruyninckx Lotte, De Leyn Paul, Van Raemdonck Dirk, Jansen Yanina, Coppens Katrien, Vermeulen Francois, Weynand Birgit, Gieraerts Christopher, Decaluwé Herbert
Department of Thoracic Surgery, University Hospitals Leuven, Leuven, Belgium.
Department of Paediatrics, Imelda Hospital, Bonheiden, Belgium.
European J Pediatr Surg Rep. 2024 Nov 4;12(1):e73-e76. doi: 10.1055/a-2430-0053. eCollection 2024 Jan.
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.
炎性肌纤维母细胞瘤（IMT）是一种罕见的间叶性肿瘤，主要发生于儿童和年轻人。病因尚不清楚。但基于染色体改变的频繁检测，尤其是在间变性淋巴瘤激酶（ALK）基因附近，IMT现在被认为是一种真正的肿瘤。此外，其可能的侵袭性行为和转移能力提示至少具有中度恶性潜能。手术仍然是首选治疗方法，但也有使用化疗、非甾体类抗炎药、免疫治疗和靶向治疗的报道。我们描述了一例6岁男孩的肺部IMT病例，该病例是偶然发现右上叶有一个病灶。进行了电视辅助胸腔镜右上叶切除术及淋巴结切除术。显微镜检查确诊为IMT，结节在浆细胞背景中可见梭形细胞。ALK免疫组化表达为阴性。
