通讯作者: 王湘, wangxiang5123@126.com; 邵亚娟, ShaoYJ@pumch.cn

DOI：10.12201/bmr.202408.00018

74例成人炎性肌成纤维细胞瘤的临床病理分析与治疗

• 摘要：炎性肌纤维母细胞瘤(IMT)是一种罕见的间充质肿瘤，多发于软组织和内脏器官，成人IMT的临床和病理特点尚不清楚。 这项研究强调了诊断IMT时综合使用组织病理学、免疫组织化学和基因检测的重要性。此外，ALK阳性的患者应考虑使用ALK抑制剂，尤其是在有较高ALK阳性率的肺IMT患者中。IMT存在长期复发风险，定期随访是必要的。 方法: 收集2010-2023年在北京协和医院诊断为IMT的74例患者信息，分析临床病理资料、治疗情况及随访结果。 结果: 74例患者中女性34例，男性40例，平均年龄为50岁，多数患者无临床症状。最常见的部位是头颈部(24.1%)，其次是肺部(21.6%)。31%的患者有ALK阳性，其中肺IMT的阳性率最高(45.5%)。大多数患者接受手术切除，一些患者接受了包括放疗、化疗或ALK抑制剂的术后治疗。整体1年、3年、5年、10年OS率分别为78.4%、70.9%、69.2%、69.2%。 局限性:本研究是一项单中心回顾性研究，患者特征的代表性有限。药物治疗患者的样本量相对较小且具有异质性，使得进一步的系统分析变得困难。 结论: IMT具有明显的临床异质性，其中肺IMT的ALK阳性率最高。手术切除仍然是主要的治疗方式，但IMT存在长期复发风险，更有效的治疗是必要的，ALK阳性患者应考虑ALK抑制剂如克唑替尼等。此外，对IMTs的分子特征的进一步研究有助于提高诊断准确性，并有利于改进治疗方案。

  关键词： 炎性肌纤维母细胞瘤; 临床病理学; ALK; 预后

   

  Abstract: Objective: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm with diverse behaviors occurring in soft tissues and visceral organs. The clinical and pathological features of IMT in adult patients are not well understood. This study highlights the importance of comprehensive diagnostics, including histopathology, immunohistochemistry, and genetic testing, in managing IMTs. High ALK positivity in lung IMTs suggests targeted therapies' benefit. ALK inhibitors should be considered for ALK-positive tumors. Regular follow-up is crucial due to relapse risk. Methods: We conducted a retrospective analysis of 74 patients diagnosed with IMTs at Peking Union Medical College Hospital between 2010 and 2023. Clinicopathological data, treatments, and outcomes were collected and analyzed. Results: Among the 74 patients (34 females, 40 males) at an average age of 50, the majority were asymptomatic. The most common tumor locations were the head and neck (24.1%), followed by lung (21.6%). Anaplastic lymphoma kinase (ALK) positivity was identified in 31% of cases, with the highest prevalence in lung IMTs (45.5%). Most patients underwent surgical resection, and some received postoperative treatments including radiotherapy, chemotherapy, or ALK inhibitors. The 1-year, 3-year, 5-year, and 10-year OS rates were 78.4%, 70.9%, 69.2%, and 69.2%，respectively. Limitations: This is a single-center retrospective study, and the characteristics of these patients may not be representative of all adult IMT patients. While the sample size of drug-treated patients is relatively small and heterogeneous, making further systematic analysis difficult. The mechanism of resistance to ALK inhibitors has not been determined. Conclusions: IMTs exhibit significant clinical heterogeneity, with lung IMTs demonstrated the highest rate of ALK positivity. While surgical resection remains the primary treatment modality, the observed rates of relapse and mortality underscore the necessity for more effective therapeutic strategies. ALK inhibitor such as crizotinib should be consideration for ALK positive patients. A deeper understanding of the molecular characteristics of IMTs may help to enhance diagnostic accuracy and inform the development of improved treatment options.

  Key words: Inflammatory myofibroblastic tumors; Clinicalpathological; Anaplastic lymphoma kinase; prognosis

  提交时间：2024-08-15

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• 序号	提交日期	编号	操作
  1	2024-08-09	bmr.202408.00018V1	下载

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