Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Fetal Pediatr Pathol. 2022 Jun;41(3):475-479. doi: 10.1080/15513815.2020.1836098. Epub 2020 Oct 23.
Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm with unknown etiology and recurrent potential. They are widely reported in children and young adults. Nearly 50% of inflammatory myofibroblastic tumor harbor rearrangement in anaplastic lymphoma kinase () gene with the majority expressing ALK protein. ALK-negative IMTs harbor alteration in gene in a subset of cases. Few reports have shown association of IMT with Epstein-Barr virus (EBV). We report a case of IMT of the spleen in an 18-month-old infant presenting with abdominal distention and failure to thrive. Workup for ALK-1, ROS1, and EBV small-encoded RNA in-situ hybridization using immunohistochemistry was negative. IMT can arise in an infant spleen.
炎性肌纤维母细胞瘤（IMT）是一种间叶性肿瘤，病因不明，有复发潜能。它们广泛见于儿童和青年。近 50%的炎性肌纤维母细胞瘤存在间变性淋巴瘤激酶（ALK）基因重排，多数表达 ALK 蛋白。ALK 阴性的 IMT 中，少数病例存在基因改变。有少数报道显示 IMT 与 EBV 相关。我们报告了一例 18 个月大婴儿的脾脏 IMT，表现为腹胀和生长不良。免疫组化检测 ALK-1、ROS1 和 EBV 小 RNA 原位杂交的 ALK 检测结果为阴性。婴儿的脾脏也可发生 IMT。
