INFLAMMATORY MYOFIBROBLASTIC TUMOR OF THE MEDIASTINUM: A RARE CASE REPORT

نویسندگان

چکیده

TOPIC: Disorders of the Mediastinum TYPE: Medical Student/Resident Case Reports INTRODUCTION: Inflammatory fibroblastic tumors (IMT) are rare. It is most common in children and adolescent. The sites lung, liver, spleen, head neck very rarely mediastinum. We present a patient who was diagnosed with rare IMT mediastinum (IMTM). CASE PRESENTATION: A white female her late twenties history substance abuse presented progressive dysphagia, odynophagia weight loss since last 1 month exertional shortness breath. Physical examination showed cachectic tenderness to palpation intravenous track marks on both upper extremities normal saturation room air. Computed tomography (CT) revealed 3 x 3.5 cm right paratracheal mass therefore she underwent CT chest which 10 9.4 para-hilar area invading resulting obstruction esophagus main bronchus along compression inferior aspect trachea. There also an encasement pulmonary artery side atrium. Bronchoscopy biopsy performed, remained non-diagnostic. Patient endoscopic bronchial ultrasound but again be Our considered as high risk for thoracoscopy procedure under general anesthesia tumor resection due airway vascular compromise aspiration esophageal obstruction. Therefore, guided mediastinal inflammatory cells atypical appearing spindle confirming diagnosis IMTM TFG-ROS1 gene fusion. She discharged home gastrostomy tube nutrition started crizotinib by oncologist. After five months, size shrink 7.5 7.2 cm. currently tolerating soft diet continued neo-adjunctively plan surgical future. DISCUSSION: discovered imaging when presents symptoms including dyspnea, cough, thoracic discomfort dysphagia. Establishing needle difficult small tissue sample. Only 6.3% cases analysis specimen alone. Definitive requires histopathologic immunohistochemical study from resected tumor. commonly have ALK rearrangements. Recently, kinase fusions ROS1 PDGFRβ identified. Surgical definitive treatment, but, crizotinib, new chemotherapeutic agent being used recently, improvement burden not amenable resection. CONCLUSIONS: In conclusion, we reporting occurring dysphagia need included differential young patients presenting obstructive REFERENCE #1: Gorolay V, Jones B. myofibroblastic invasion: case report literature review. Clin Imaging. 2017 May-Jun;43:32-35. doi: 10.1016/j.clinimag.2016.09.012. Epub 2016 Sep 28. PMID: 28178581 #2: Khalil, Sumaira, Ghafoor, Tariq, Raja, Amna Kaneez Fatima. Myofibroblastic Tumor: Rare Presentation Effective Treatment Crizotinib. (2020) Oncological Medicine #3: Mai S, Xiong G, Diao D, Wang W, Zhou Y, Cai R. report: Crizotinib effective ROS1-rearranged Lung Cancer. 2019 Feb;128:101-104. 10.1016/j.lungcan.2018.12.016. 2018 Dec 17. 30642440 DISCLOSURES: No relevant relationships Raguraj Chandradevan, source=Web Response Arish Maknojia, Taniya Mathew, Neeta Sukthanker,

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ژورنال

عنوان ژورنال: Chest

سال: 2021

ISSN: ['0012-3692', '1931-3543']

DOI: https://doi.org/10.1016/j.chest.2021.07.1175