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寻常型天疱疮患者并发肺部炎性肌纤维母细胞瘤:一例报告

Pulmonary Inflammatory Myofibroblastic Tumor in a Patient Treated for Pemphigus Vulgaris: A Case Report.

作者信息

Rhazari Meriem, Thouil Afaf, Gartini Sara, Kouismi Hatim, Lakhal Mohamed

机构信息

Department of Pulmonology, Mohammed VI University Hospital, Oujda, MAR.

Department of Respiratory Diseases, Faculty of Medicine and Pharmacy of Oujda, Mohammed VI University Hospital, Mohammed First University, Oujda, MAR.

出版信息

Cureus. 2025 May 26;17(5):e84811. doi: 10.7759/cureus.84811. eCollection 2025 May.

DOI:10.7759/cureus.84811
PMID:40568253
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12188145/
Abstract

Inflammatory myofibroblastic tumors (IMTs) are benign tumors with diverse histological presentations. Pulmonary IMTs are particularly uncommon. Diagnosis often necessitates surgical intervention for both therapeutic and diagnostic purposes. We report the case of a 70-year-old woman with a history of uncontrolled asthma and pemphigus vulgaris treated with rituximab and corticosteroids. She presented with chest pain and a dry cough, without systemic symptoms. Imaging revealed a solitary pulmonary nodule. Thoracoscopic resection confirmed the diagnosis of an IMT, characterized histologically by spindle cell proliferation and inflammatory infiltrates, with negative anaplastic lymphoma kinase (ALK) expression. Pulmonary IMTs are rare and diagnostically challenging due to their overlapping features with other pulmonary lesions. Etiologies include infections, autoimmune conditions, and chromosomal abnormalities, with a potential link to IgG4 involvement in allergic and autoimmune contexts. Radiologically, they typically present as well-circumscribed solitary nodules. Definitive diagnosis often necessitates surgical excision, given the limitations of imaging and biopsy. Pulmonary IMTs, while benign, require prompt diagnosis and management due to their potential for local recurrence and rare malignant transformation. Surgical resection remains the cornerstone of treatment, underscoring the importance of vigilant long-term follow-up.

摘要

炎性肌纤维母细胞瘤(IMTs)是具有多种组织学表现的良性肿瘤。肺IMTs尤为罕见。诊断通常需要手术干预以达到治疗和诊断目的。我们报告一例70岁女性病例,她有未控制的哮喘病史和寻常型天疱疮病史,接受过利妥昔单抗和皮质类固醇治疗。她出现胸痛和干咳,无全身症状。影像学检查发现一个孤立性肺结节。胸腔镜切除确诊为IMT,组织学特征为梭形细胞增殖和炎症浸润,间变性淋巴瘤激酶(ALK)表达阴性。肺IMTs很罕见,且因其与其他肺部病变有重叠特征而在诊断上具有挑战性。病因包括感染、自身免疫性疾病和染色体异常,在过敏和自身免疫背景下可能与IgG4参与有关。在放射学上,它们通常表现为边界清晰的孤立性结节。鉴于影像学和活检的局限性,明确诊断通常需要手术切除。肺IMTs虽然是良性的,但由于其有局部复发和罕见恶性转化的可能性,需要及时诊断和处理。手术切除仍然是治疗的基石,强调了长期密切随访的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/1bfa1c2b5806/cureus-0017-00000084811-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/10eabd318543/cureus-0017-00000084811-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/532b668539e6/cureus-0017-00000084811-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/9015e25292bb/cureus-0017-00000084811-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/073a151791e2/cureus-0017-00000084811-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/1bfa1c2b5806/cureus-0017-00000084811-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/10eabd318543/cureus-0017-00000084811-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/532b668539e6/cureus-0017-00000084811-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/9015e25292bb/cureus-0017-00000084811-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/073a151791e2/cureus-0017-00000084811-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b7db/12188145/1bfa1c2b5806/cureus-0017-00000084811-i05.jpg

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