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1例炎性肌纤维母细胞瘤致左肺动脉狭窄并成功接受局部放疗的病例报告,该患者患有核周型抗中性粒细胞胞浆抗体血管炎

A Rare Case of Inflammatory Myofibroblastic Tumor Causing Left Pulmonary Artery Stenosis and Successfully Treated with Localized Radiotherapy in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibody Vasculitis.

作者信息

Browne Claire P, Zeman-Pocrnich Cady, Dar A Rashid, Wyllie Blair, Joseph Mariamma

机构信息

Internal Medicine, Schulich School of Medicine & Dentistry, Western University, London, CAN.

Pathology and Laboratory Medicine, London Health Sciences Centre, London, CAN.

出版信息

Cureus. 2020 Jan 20;12(1):e6709. doi: 10.7759/cureus.6709.

Abstract

Inflammatory myofibroblastic tumor (IMT) of the lung is a rare neoplasm that commonly behaves in an indolent fashion and is generally treated with complete surgical excision. The management of unresectable IMT presents a significant challenge, especially in cases with multiple comorbidities, and a consensus has yet to be reached on the most appropriate first-line modality. We present a case of unresectable IMT causing severe stenosis of the left pulmonary artery in a patient on immunosuppressive therapy for perinuclear antineutrophil cytoplasmic antibody vasculitis. The patient was successfully treated with localized radiotherapy to a total dose of 45 Gy in five weeks, and has been followed for more than seven years since treatment. In this case report, we review the pertinent literature and illustrate the difficulties in diagnosing and treating rare neoplasms in a patient with significant medical comorbidities.

摘要

肺炎性肌纤维母细胞瘤(IMT)是一种罕见的肿瘤,通常生长缓慢,一般采用完整手术切除进行治疗。不可切除的IMT的治疗是一项重大挑战,尤其是在伴有多种合并症的情况下,对于最合适的一线治疗方式尚未达成共识。我们报告一例不可切除的IMT病例,该病例导致一名因核周抗中性粒细胞胞浆抗体血管炎接受免疫抑制治疗的患者左肺动脉严重狭窄。该患者成功接受了局部放疗,五周内总剂量达45 Gy,自治疗后已随访七年多。在本病例报告中,我们回顾了相关文献,并阐述了在患有严重内科合并症的患者中诊断和治疗罕见肿瘤的困难。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1d56/7032609/f0748582b2cc/cureus-0012-00000006709-i01.jpg

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