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恶性炎性肌纤维母细胞瘤:1例罕见病例报告

Malignant inflammatory myofibroblastic tumor: a rare case presentation.

作者信息

Spafford Megan, Lunn Danley, Graham Peter

机构信息

Department of General Surgery, University of Saskatchewan, Saskatoon, SK, Canada.

出版信息

J Surg Case Rep. 2022 Sep 19;2022(9):rjac403. doi: 10.1093/jscr/rjac403. eCollection 2022 Sep.

Abstract

Malignant inflammatory myofibroblastic tumors (IMT) are extremely rare, aggressive tumors with variable presentation. This is a case of a 29-year-old female presented with severe anemia and a large abdominal mass presumed to be a gastrointestinal stromal tumor (GIST). Severe anemia, leukocytosis and thrombocytosis accompanied the presentation. Final pathological assessment yielded a diagnosis of malignant IMT. Given the rarity of these tumors, no established diagnostic criteria exist aside from histological analysis of the tissue, which may result in delays or inappropriate treatment. As these tumors are aggressive in nature, a high index of suspicion is critical to improve outcomes. Further reports on the presentation, diagnosis and treatment of such rare tumors are important to develop clinical diagnostic guidelines to improve diagnosis and treatment and improve outcomes.

摘要

恶性炎性肌纤维母细胞瘤(IMT)极为罕见,是一种具有多种表现形式的侵袭性肿瘤。这是一例29岁女性患者,表现为严重贫血和一个被推测为胃肠道间质瘤(GIST)的腹部巨大肿块。就诊时伴有严重贫血、白细胞增多和血小板增多。最终病理评估诊断为恶性IMT。鉴于这些肿瘤的罕见性,除了对组织进行组织学分析外,尚无既定的诊断标准,这可能导致诊断延迟或治疗不当。由于这些肿瘤本质上具有侵袭性,高度的怀疑指数对于改善治疗结果至关重要。进一步报道此类罕见肿瘤的表现、诊断和治疗对于制定临床诊断指南以改善诊断和治疗并提高治疗结果具有重要意义。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4cf0/9487198/a32add27357e/rjac403f1.jpg

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