乳腺导管原位癌合并肺炎性肌纤维母细胞瘤1例并文献复习

A Case of Ductal Carcinoma in Situ of Breast with Pneumonia Myofibroblastoma and Literature Review

目的炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT)是一种具有浸润、恶化等特点的间叶性肿瘤,好发于肺部。由于IMT缺乏特异性临床症状且影像学表现多变,易出现误诊。针对IMT的发病机制、临床表现、诊断、治疗方式以及预后等方面进行讨论,为提高对间叶性肿瘤诊断的准确率提供临床经验。方法回顾分析经手术病理证实的1例乳腺癌导管原位癌合并肺炎性肌纤维母细胞瘤的影像学表现并对IMT发病机制、临床特点、影像学表现、治疗方法及预后的相关文献进行复习和总结。结果肺炎性肌纤维母细胞瘤临床表现为呼吸困难或为发热、咳嗽、咳痰、胸闷等,实验室检查无特异性,可有血红蛋白浓度降低、血小板增多及沉降率升高。在影像上分为浸润型、肿块型、结节型,尤以肿块型多见,多为单发,呈圆形或类圆形,多发生在肺的表浅部位。病理是IMT诊断的金标准,镜下肿瘤由分化肌纤维母细胞性梭形细胞组成,呈束状或漩涡状排列,间质内伴有大量浆细胞和/或淋巴细胞浸润;免疫组化可见梭形细胞表达平滑肌肌动蛋白SMA和波形蛋白Vimentin,预后较好,手术切除是主要治疗方法。结论IMT临床表现无特异性,诊断依据有胸部X线片、胸部CT和经皮肺穿刺活检等,确诊IMT唯一方法是病理学检查,镜下表现为编织状排列的梭形细胞伴大量炎性细胞浸润,手术切除是主要治疗方法,预后取决于手术切除的质量和肿瘤的大小。

Objective The inflammatory myofibroblastic tumor(IMT)is a mesenchymal tumor with infiltrating and worsening characteristics,usually in the lungs.Because IMT lacks specific clinical symptoms and the imaging findings are changeable,it is easy to be misdiagnosed.This article discusses the pathogenesis,clinical manifestations,diagnosis,treatment,and prognosis of IMT and provides clinical experience for improving the accuracy of diagnosing mesenchymal tumors.Methods The imaging manifestations of a case of breast ductal carcinoma in situ combined with pneumonia myofibroblastoma confirmed by surgery and pathology were analyzed retrospectively.A review and review of the relevant literature on the pathogenesis,clinical features,imaging manifestations,treatment methods,and prognosis of IMT was summarized.Results Pneumonia myofibroblastoma was clinically dyspnea or fever,cough,sputum,chest tightness,etc.Laboratory tests were not specific and may have reduced hemoglobin concentration,increased thrombocytosis,and increased sedimentation rate.It was divided into infiltrating type,mass type,and nodular type on the image.The mass type was more common,mostly single,round,or quasi-circular,and mostly in the superficial part of the lung.Pathology was the gold standard for IMT diagnosis.The tumor was composed of differentiated myofibroblastic spindle cells,arranged in bundles or spirals,with a large number of plasma cells and/or lymphocytes infiltration in the interstitium.Immunohistochemistry showed the spindle shape cells expressing smooth muscle actin and vimentin with a good prognosis,and surgical resection was the main treatment.Conclusion The clinical manifestations of IMT are not specific.The diagnosis is based on a chest X-ray,chest CT,and percutaneous lung biopsy.The only way to confirm IMT is through pathological examination.The microscopic findings are woven-like spindle cells with many inflammatory cells.Surgical resection is the primary treatment method,and the prognosis depends on the quality of surgical resection and the tumor size.