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一例表现为张口受限、左侧颏部及眶下区感觉减退、左侧眼睑间歇性下垂及集合性斜视的炎性肌纤维母细胞瘤

An Inflammatory Myofibroblastic Tumour Presenting with Limited Mouth-Opening, Hypoesthaesia of The Left Chin and Infraorbital Area, Intermittent Left Eye Ptosis and Converging Strabismus.

作者信息

Ben Hassine Imen, Romdhane Wiem, Belkacem Raouaa, Anoun Jihed, Baya Wafa, Mzabi Anis, Ben Fredj Fatma

机构信息

Faculty of Medicine, University of Sousse, Susa, Tunisia.

Internal Medicine Department, Sahloul University Hospital, Susa, Tunisia.

出版信息

Eur J Case Rep Intern Med. 2024 Aug 23;11(9):004568. doi: 10.12890/2024_004568. eCollection 2024.

DOI:10.12890/2024_004568
PMID:39247249
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11379115/
Abstract

UNLABELLED

An inflammatory myofibroblastic tumour (IMT) is a rare neoplasm of mesenchymal origin, defined by myofibroblastic spindle cells accompanied by inflammatory cells, lymphocytes and eosinophils. Its symptomatology depends on the involved site and tends to mimic a malignant tumour clinically and radiologically. The head and neck region accounts for 5% of all IMTs. Here, we report a case of a 35-year-old woman, with no medical history, who presented with a mouth-opening limitation of 8 mm evolving for three years and occurring six months after of a wisdom tooth extraction. She also experienced a recent occurrence of left eye ptosis and a converging strabismus. On examination, the patient had a body temperature at 37°C, with hypoesthaesia of the left chin and infraorbital area, without any other abnormality. Laboratory examinations did not reveal a biological inflammatory syndrome or rhabdomyolysis. The infectious investigations were all negative, as well as the immunological tests, in particular negative for anti-AChR and anti-MuSK antibodies. On the facial computed tomography (CT) scan, we noted an active reshuffle in the left mandible ascending branch with a thickening of the ipsilateral pterygoid muscles and the left temporal meningeal tissue. After corticosteroid therapy 0.7 mg/kg/j, we obtained an improvement in the patient's mouth-opening, thus a biopsy of the lesion was performed under local anaesthesia, revealing IMT. The patient continued the corticosteroids therapy with a progressive tapering resulting in a marked clinical improvement of the mouth-opening limitation and her ptosis.

LEARNING POINTS

An inflammatory myofibroblastic tumour (IMT) is a challenging disease.Given the variable clinical and radiological presentation of the disease, it is of paramount importance to know it, to be swiftly recognised so diagnosis can be promptly made.The adapted treatment should be immediately started to prevent possible life-threatening outcomes.

摘要

未标注

炎性肌纤维母细胞瘤(IMT)是一种罕见的间叶源性肿瘤,由肌纤维母细胞性梭形细胞伴有炎性细胞、淋巴细胞和嗜酸性粒细胞构成。其症状取决于受累部位,在临床和放射学上往往类似恶性肿瘤。头颈部区域占所有IMT的5%。在此,我们报告一例35岁女性病例,该患者无病史,出现张口受限8毫米,病程三年,在拔除智齿六个月后出现。她近期还出现左眼上睑下垂和内斜视。检查时,患者体温37°C,左下颌和眶下区域感觉减退,无其他异常。实验室检查未发现生物学炎症综合征或横纹肌溶解。感染性检查均为阴性,免疫学检查也为阴性,尤其是抗AChR和抗MuSK抗体阴性。在面部计算机断层扫描(CT)上,我们注意到左下颌升支有活跃的重塑,同侧翼状肌和左颞脑膜组织增厚。在给予0.7毫克/千克/天的皮质类固醇治疗后,患者的张口情况有所改善,因此在局部麻醉下对病变进行活检,结果显示为IMT。患者继续接受皮质类固醇治疗,并逐渐减量,导致张口受限和上睑下垂有明显的临床改善。

学习要点

炎性肌纤维母细胞瘤(IMT)是一种具有挑战性的疾病。鉴于该疾病临床表现和放射学表现多样,了解它并迅速识别以便能及时做出诊断至关重要。应立即开始适当的治疗以防止可能危及生命的后果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/2a88b8c41543/4568_Fig6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/50cdbd0593d8/4568_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/feeec9049ff2/4568_Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/d1982ae8c0e8/4568_Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/e4cdea39e516/4568_Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/21b7bf42e4aa/4568_Fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/2a88b8c41543/4568_Fig6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/50cdbd0593d8/4568_Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/feeec9049ff2/4568_Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/d1982ae8c0e8/4568_Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/e4cdea39e516/4568_Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/21b7bf42e4aa/4568_Fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/67b0/11379115/2a88b8c41543/4568_Fig6.jpg

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本文引用的文献

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Locally Advanced Inflammatory Myofibroblastic Tumor Treated With Targeted Therapy: A Case Report and Literature Review.局部晚期炎性肌纤维母细胞瘤的靶向治疗:一例报告及文献综述
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