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炎性肌纤维母细胞瘤

Inflammatory myofibroblastic tumour.

作者信息

McDermott Michael

机构信息

Dept of Paediatric Laboratory Medicine, Our Lady׳s Children׳s Hospital, Crumlin, Dublin 12, Ireland.

出版信息

Semin Diagn Pathol. 2016 Aug 31. doi: 10.1053/j.semdp.2016.08.007.

Abstract

While initially controversial, the proposal that a subset of inflammatory pseudotumours were myofibroblastic neoplasms is now acknowledged. Inflammatory myofibroblastic tumour is a spindle cell neoplasm of intermediate biological potential that may arise in a wide range of anatomic sites but has a particular propensity for the lung and abdominal soft tissues. Depending on its location, IMT may present with a variety of clinical symptoms and it may also express a variable pathologic phenotype, leading to a broad range of clinical and pathological differentials. Recent discoveries about the molecular signatures of IMT not only provide additional tools to assist in their diagnosis, they also point to possible therapeutic interventions that may transform the management algorithms for patients with this condition.

摘要

虽然最初存在争议,但炎症性假瘤的一个子集是肌成纤维细胞肿瘤这一观点现在已得到认可。炎症性肌成纤维细胞肿瘤是一种具有中等生物学潜能的梭形细胞肿瘤,可发生于广泛的解剖部位,但特别好发于肺和腹部软组织。根据其位置,炎症性肌成纤维细胞肿瘤可能表现出多种临床症状,也可能表现出可变的病理表型,从而导致广泛的临床和病理鉴别诊断。关于炎症性肌成纤维细胞肿瘤分子特征的最新发现不仅为辅助诊断提供了更多工具,还指出了可能的治疗干预措施,这可能会改变这种疾病患者的管理算法。

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