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ROS1 阳性小肠炎性肌纤维母细胞瘤致梗阻:1 例报告。

ROS1-positive Inflammatory Myofibroblastic Tumor of the Small Bowel Causing Obstruction: A Case Report.

机构信息

Department of Radiology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

The Institute of Pathology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.

出版信息

J Radiol Case Rep. 2022 Jan 1;16(1):14-21. doi: 10.3941/jrcr.v16i1.3928. eCollection 2022 Jan.

Abstract

Inflammatory Myofibroblastic Tumors (IMTs) are rare fibroblastic/myofibroblastic neoplasms that affect predominately pediatric patients and young adults. Almost half of the patients with IMTs have a chromosomal abnormality in the Anaplastic Lymphoma Kinase 1 gene on chromosome 2p23. Although these tumors occur primarily in the lung, lesions have been reported in a variety of intra-abdominal organs like the liver, spleen, and mesentery. Small bowel IMTs are particularly rare. IMTs generally pursue a benign clinical course, however intra-abdominal and retroperitoneal tumors have typically shown higher local recurrence and even distant metastases. The most common presenting symptoms of an intra-abdominal IMT are abdominal pain and change in bowel habits. Laboratory results are nonspecific and can include anemia and minor elevation of inflammatory markers like C-reactive protein. We report an unusual case of IMT in the small bowel causing the obstruction.

摘要

炎性肌纤维母细胞瘤(IMTs)是一种罕见的成纤维细胞/肌纤维母细胞瘤性肿瘤,主要影响儿童和年轻成人。几乎一半的 IMT 患者在染色体 2p23 上的间变性淋巴瘤激酶 1 基因存在染色体异常。虽然这些肿瘤主要发生在肺部,但已在各种腹腔内器官(如肝、脾和肠系膜)中报告了病变。小肠 IMT 尤其罕见。IMTs 通常表现出良性的临床过程,然而腹腔内和腹膜后肿瘤通常表现出更高的局部复发率,甚至远处转移。腹腔内 IMT 的最常见表现症状为腹痛和肠道习惯改变。实验室结果是非特异性的,可包括贫血和炎症标志物(如 C 反应蛋白)的轻度升高。我们报告了一例不常见的小肠 IMT 导致梗阻的病例。

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