inflammatory myofibroblastic tumor

炎性肌纤维母细胞瘤
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  • 文章类型: Case Reports
    炎性肌纤维母细胞瘤(IMT)是由分化的肌纤维母细胞梭形细胞组成的中间肿瘤,具有炎性细胞浸润。它可以发生在身体的所有部位,肺部是最常见的,而肺外的组织,包括乙状结肠,是罕见的。在这里,我们介绍了一例10岁的乙状结肠IMT女孩,她因腹痛来到我们医院。腹部计算机断层扫描(CT)显示,她的下腹部有轻微的低密度肿块,与乙状结肠没有明确分界。肿块在对比增强CT上显示出显着的不均匀增强,在正电子发射断层扫描(PET)上氟18氟脱氧葡萄糖(18F-FDG)的摄取增加。此外,我们对已发表的乙状结肠IMT相关文献进行了系统评价,并总结了乙状结肠IMT的临床和影像学特征,以提高对这一罕见疾病的认识.
    Inflammatory myofibroblastic tumor (IMT) is an intermediate tumor composed of differentiated myofibroblastic spindle cells with inflammatory cell infiltration. It can occur in all parts of the body, with the lungs being the most common, while the tissues outside the lungs, including the sigmoid colon, are rare. Herein, we present a case of a 10-year-old girl with sigmoid IMT who presented to our hospital with abdominal pain. An abdominal computed tomography (CT) revealed a well-defined, slightly low-density mass in her lower abdomen that was not clearly demarcated from the sigmoid colon. The mass showed significant uneven enhancement on contrast-enhanced CT and increased fluorine-18 fluorodeoxyglucose (18F-FDG) uptake on positron emission tomography (PET). Moreover, a systematic review of the published literature on sigmoid IMT was conducted and its clinical and radiographic features were summarized to increase the understanding of this rare disease.
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  • 文章类型: Case Reports
    炎性肌纤维母细胞瘤是几乎所有器官中都有描述的罕见肿瘤。尽管它们在食道中极为罕见,文献中已经描述了几个案例。手术切除是大多数情况下使用的治疗方式。在这份报告中,我们描述了一例炎性肌纤维母细胞瘤,首次通过内镜黏膜下剥离术成功治疗.
    Inflammatory myofibroblastic tumors are rare tumors that have been described in virtually all organs. Even though they are extremely rare in the esophagus, several cases have been described in the literature. Surgical resection has been the therapeutic modality used in most of those cases. In this report, we describe a case of inflammatory myofibroblastic tumor that was successfully managed endoscopically for the first time with the endoscopic submucosal dissection technique.
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  • 文章类型: Journal Article
    炎性肌纤维母细胞瘤(IMT)是一种罕见的软组织肿瘤,主要发生在年轻患者的腹盆腔区域。它的特征是纺锤形的肌成纤维细胞,或者被炎症浸润包围的成纤维细胞。在这里,我们报告了一例24岁男性患者,其诊断为IMT,14个月后复发.通过使用NextSeq测序仪的下一代测序,该肿瘤显示了一种新型THBS1::ALK融合物,其中包含血小板反应蛋白1(THBS1)基因的外显子1-7与间变性淋巴瘤激酶(ALK)基因的外显子19融合。THBS1与ALK的融合可能导致ALK激酶结构域的表达增加和组成型激活。这些发现不仅拓宽了与肿瘤发生有关的已知ALK融合伴侣的范围,而且通过将此融合事件视为因果因素,为研究复发性IMT的病因提供了新的途径。据我们所知,这是文献中报道的第二例具有这种新突变的喉IMT病例,也是第一例详细描述了这种特异性融合和临床复发的病例.
    Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue tumor primarily occurring in the abdominopelvic region of young patients, and it is characterized by spindle-shaped myofibroblasts, or fibroblasts surrounded by inflammatory infiltrate. Herein, we report a case of a 24-year-old male with a firm submucosal mass in the anterior right vocal fold diagnosed as an IMT that recurred 14 months later. The tumor demonstrated a novel THBS1::ALK fusion containing Exons 1-7 of the thrombospondin 1 (THBS1) gene fused to Exon 19 of the anaplastic lymphoma kinase (ALK) gene via next-generation sequencing with the NextSeq sequencer. The fusion of THBS1 to ALK potentially results in increased expression and constitutive activation of the ALK kinase domain. These findings not only broaden the repertoire of known ALK fusion partners implicated in tumorigenesis but also provide a novel avenue for investigating the etiology of recurrent IMT by considering this fusion event as a causal factor. To our knowledge, this is the second case of IMT of the larynx with this novel mutation reported in the literature and the first such case with a detailed description of this specific fusion and clinical recurrence.
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  • 文章类型: Case Reports
    炎性肌纤维母细胞瘤(IMT)是一种罕见的实体,分类在软组织肉瘤。这是一种中间恶性肿瘤,很少表现为转移性疾病。选择的治疗方法是手术,除非由于定位或存在转移性疾病而无法进行手术。大约50%的IMT会表现出ALK易位,为这些患者提供治疗靶点。
    一例患者患有转移性IMT,对阿来替尼治疗完全缓解,维持4年以上。
    该病例在接受阿来替尼治疗的IMT患者中显示出长时间的完全反应。
    UNASSIGNED: Inflammatory myofibroblastic tumor (IMT) is a rare entity, classified within soft tissue sarcomas. It is an intermediate malignancy tumor, which seldom presents as metastatic disease. The treatment of choice is surgery, except in cases where surgery is not possible due to localization or if it presents with metastatic disease. Approximately 50% of IMTs will exhibit ALK translocation, providing a therapeutic target for these patients.
    UNASSIGNED: A case is presented of a patient with metastatic IMT in complete response to treatment with alectinib, maintained for over 4 years.
    UNASSIGNED: This case showed a long time complete response in patient with IMT treated with alectinib.
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  • 文章类型: Journal Article
    炎性肌纤维母细胞瘤(IMT)是由肌纤维母细胞和成纤维细胞组成的罕见肉瘤,伴有炎性细胞浸润。许多IMT表现出间变性淋巴瘤激酶(ALK)的克隆重排。我们在此报告了一名56岁的子宫IMT妇女,该妇女在异基因造血干细胞移植(allo-HSCT)后发生血小板反应蛋白1::ALK融合。全身治疗前的实验室数据显示白细胞介素6和严重的白细胞增多。患者接受了氯拉替尼治疗;然而,应答持续时间约为2个月.需要汇编和评估类似的病例报告,以阐明氯拉替尼在ALK重排后allo-HSCTIMT中的疗效。
    Inflammatory myofibroblastic tumors (IMTs) are rare sarcomas composed of myofibroblastic and fibroblastic cells, accompanied by inflammatory cell infiltration. Many IMTs exhibit clonal rearrangement of anaplastic lymphoma kinase (ALK). We herein report a 56-year-old woman with uterine IMT harboring a thrombospondin-1::ALK fusion that developed after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Laboratory data before systemic therapy indicated increased interleukin-6 and severe leukocytosis. The patient was treated with lorlatinib; however, the response duration was approximately two months. Similar case reports need to be compiled and evaluated to elucidate the efficacy of lorlatinib in post-allo-HSCT IMT with ALK rearrangement.
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  • 文章类型: Case Reports
    炎性假瘤包括广泛的非肿瘤性和肿瘤性实体,包括炎性肌纤维母细胞瘤(IMT)。因为它是一种罕见的间叶性肿瘤,病因和病机不明,其临床症状和放射学特征不明显,颅内IMT可误诊为其他轴外肿瘤。这里,我们介绍一例颅内IMT疑为脑脓肿.
    在这种情况下,一名73岁的妇女出现头痛,恶心,和眩晕.脑计算机断层扫描(CT)和磁共振成像显示,左桥小脑角上有4×3cm大小的椭圆形边缘增强病变。考虑到患者的中耳炎病史和CT表现,我们假设该病变是慢性脑脓肿。最初的毛刺孔引流手术没有成功,因为没有脓肿,导致第二次根治性切除手术。组织病理学和免疫组织化学分析最终揭示了颅内IMT的最终诊断。
    颅内IMT是一种发病机制不明的罕见疾病。诊断主要取决于组织病理学和免疫组织化学分析。正如在我们的案例中观察到的,这种疾病可能被误认为是脑膜瘤,孤立性纤维瘤,或慢性脓肿,由于其罕见的发生。
    UNASSIGNED: Inflammatory pseudotumor encompasses a broad range of non-neoplastic and neoplastic entities, including inflammatory myofibroblastic tumors (IMTs). Because it is a rare mesenchymal tumor of unknown etiology and pathogenesis, and its clinical symptoms and radiologic features are not distinctive, intracranial IMT could be misdiagnosed as other extra-axial tumors. Here, we present a case of intracranial IMT suspected to be a brain abscess.
    UNASSIGNED: In this case, a 73-year-old woman presented headaches, nausea, and vertigo. Brain computed tomography (CT) and magnetic resonance imaging showed 4 × 3 cm sized oval rim-enhanced lesion on the left cerebellopontine angle. Considering the patient\'s history of otitis media and CT findings, we hypothesized that this lesion was a chronic brain abscess. The initial burr hole drain surgery was unsuccessful because there was no abscess, leading to a second radical excision surgery. Histopathological and immunohistochemical analyses eventually revealed a final diagnosis of intracranial IMT.
    UNASSIGNED: Intracranial IMT is a rare disease with unknown pathogenesis. Diagnosis primarily depends on histopathological and immunohistochemistry analyses. As observed in our case, this disease may be mistaken for meningiomas, solitary fibrous tumors, or chronic abscesses due to its rare occurrence.
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  • 文章类型: Case Reports
    钙化纤维瘤(CFT),也被称为钙化性纤维假瘤,是一种不常见的非癌性肿瘤,通常位于胃肠道。它在肺中的位置极为罕见,只有少数病例报告发表。此病例报告描述了我们在一名9岁男性偶然肺部肿块患者中的诊断方法。该肿块最初被误诊,需要多种影像学检查和干预才能获得肺部CFT的明确诊断。本文旨在通过提供计算机断层扫描和磁共振成像的详细发现,为有关肺CFT的有限信息做出贡献。
    A calcifying fibrous tumor (CFT), also known as calcifying fibrous pseudotumor, is an uncommon non-cancerous neoplasm usually located in the gastrointestinal tract. Its location in the lung is extremely rare, and only a few case reports have been published. This case report describes our diagnostic approach in a 9-year-old male patient with an incidental pulmonary mass. The mass was initially misdiagnosed, requiring multiple imaging tests and interventions to obtain the definitive diagnosis of pulmonary CFT. This paper aims to contribute to the limited information available on pulmonary CFT by presenting detailed findings from computed tomography and magnetic resonance imaging.
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  • 文章类型: Case Reports
    直肠乙状结肠交界处炎性肌纤维母细胞瘤(IMT)的发生在儿童中并不常见。这是一种罕见的间充质肿瘤,属于软组织肿瘤,并且可以在从中枢神经系统到胃肠道的任何解剖部位找到。我们的患者是一名10岁的男性受试者,抱怨排便和便秘不足。患者在转诊前约两周减少了排便和便秘的频率,尽管使用了泻药,但没有改善。腹部完全扩张,检查中没有压痛或保护。腹部X射线上显示了几种空气流体水平。在超声波中,据报道,小叶间和骨盆间隙有游离液。患者被转移到手术室。检测到直肠乙状结肠交界处的肿瘤。组织病理学研究显示IMT的证据。IMT是一种未知来源的罕见肿瘤,这可能发生在身体的不同部位。完全手术切除通常是治愈性的,但需要早期发现复发。治疗方案包括化疗,放射治疗,和免疫疗法。需要进一步的研究来提高对这种罕见肿瘤的理解和管理。
    The occurrence of rectosigmoid junction inflammatory myofibroblastic tumor (IMT) is uncommon in children. This is a rare form of mesenchymal tumor, belonging to the category of soft tissue tumors, and can be found at any anatomical site from the central nervous system to the gastrointestinal tract. Our patient was a 10-year-old male subject complaining of lack of defecation and constipation. The patient had decreased the frequency of defecation and constipation about two weeks before his referral and had not improved despite the use of laxatives. The abdomen was completely distended and there was no tenderness or guarding in the examination. Several airfluid levels are shown on the abdominal X-ray. In the ultrasound, free fluid was reported in the interlobular and pelvic spaces. The patient was transferred into the operating room. A tumor of the rectosigmoid junction was detected. Histopathologic studies showed evidence of IMT. IMT is a rare neoplasm of unknown origin, which may occur in various sites of the body. Complete surgical removal is usually curative, but early detection of recurrence is required. Treatment options include chemotherapy, radiation therapy, and immunotherapy. Further investigations are needed to improve the understanding and management of this rare tumor.
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