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• W4387265816 abstract "SESSION TITLE: Lung Pathology Global Case Report Posters 1 SESSION TYPE: Global Case Reports PRESENTED ON: 10/10/2023 12:00 pm - 12:45 pm INTRODUCTION: We presented a case of tracheal ROS1 translocated inflammatory myofibroblastic tumour rich in histiocyte demonstrating rapid local recurrence and was misdiagnosed as asthma. CASE PRESENTATION: A 49-year-old lady presented with dyspnea and productive cough for 2 weeks. Physical examination showed stridor and expiratory wheeze. Chest X-ray showed suspected lesion at trachea. Flexible laryngoscopy showed clear airway down to vocal cords. Patient was treated as asthma and discharged with oral steroid. A scheduled spirometry demonstrated Empey index of 14.5 and expiratory disproportion index (EDI) of 87, with flow volume loop suspicious of fixed upper airway obstruction. She was readmitted for persistent dyspnea. Arterial blood gas revealed compensated type 2 respiratory failure. Contrast computed tomography of neck and thorax identified a 2x2x1.2cm enhancing polypoid nodule at the cervical trachea located at 2.6cm inferior to the level of vocal cords. Rigid bronchoscopy found a large fleshy tumor 3cm with 80% obstruction of the trachea. Mechanical coring was performed.The tumour measured 2.5 cm x 1 cm x 1cm. Microscopic examination showed a submucosal histiocyte-rich lesion, with neoplastic spindle cells arranged in fascicles, intimately associated with mixed populations of plasma cells, lymphocytes and abundant histiocytes. The neoplastic spindle cells possessed plump vesicular nuclei, distinct nucleoli and purplish cytoplasm. Mitotic activity was readily identified and tumour necrosis was focally seen. Immunostaining for histiocytic markers (CD163 and CD68) showed apparent diffuse strong staining in tumour tissue, however, additional immunostaining of PU.1 clarified the non-histiocytic nature of the neoplastic spindle cells. On immunostaining, the spindle cells showed patchy SMA and diffuse strong cytoplasmic expression of ROS1, while pan-cytokeratin (MNF116), CAM5.2, p40, pan-TRK, ALK, BRAFV600E(VE1), S100 and SOX10 were negative. Fluorescent in-situ hybridization (FISH) confirmsed the presence of ROS1 gene rearrangement and lack of RET gene translocation. The diagnosis was inflammatory myofibroblastic tumour (IMT).A follow-up bronchoscopy showed residual tumor growth. Excision was deemed not suitable due to the extension of the tumour to esophagus and thyroid. Mechanical coring was repeated. The patient received radiotherapy for local disease control. DISCUSSION: In this case initially presented with stridor suspicious of severe upper airway obstruction with a normal laryngoscopy and subtle Chest X-ray abnormality, an early spirometry can help screening out cases that need emergent attention. Apart from the classical flow-volume-loop pattern, an EDI of 87 would alert the physician to arrange an urgent bronchoscopy (1). Inflammatory myofibroblastic tumour is characterized by spindle myofibroblastic proliferation in background of prominent inflammatory lymphoplasmacytic infiltrate. The current case demonstrates several pathological features uncommon to IMT, regarding its uncommon tracheal localization, histiocyte-rich histological pattern and rare genetic aberration of ROS1 gene rearrangement (2). The neoplastic myofibroblasts are obscured by histiocytes, mimicking a histiocytic neoplasm. Multinucleated Touton giant cells are seen in foci. The more typical pattern of fascicular proliferation of myofibroblastic spindle cells is only observed in focal areas. The tumour harbors ROS1 gene rearrangement, instead of the more common ALK gene rearrangement. CONCLUSIONS: Physicians and pathologists should be aware of tracheal tumor as asthma mimic and histiocyte rich ROS1 translocated IMT respectively. REFERENCE #1: Nouraei SA, Nouraei SM, Patel A, Murphy K, Giussani DA, Koury EF, et al. Diagnosis of laryngotracheal stenosis from routine pulmonary physiology using the expiratory disproportion index. Laryngoscope. 2013;123(12):3099-104. REFERENCE #2: Yamamoto H, Yoshida A, Taguchi K, Kohashi K, Hatanaka Y, Yamashita A, et al. ALK, ROS1 and NTRK3 gene rearrangements in inflammatory myofibroblastic tumours. Histopathology. 2016;69(1):72-83. DISCLOSURES: No relevant relationships by Chin Tong Kwok No relevant relationships by Jason Tsang" @default.
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• W4387265816 date "2023-10-01" @default.
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• W4387265816 title "RAPIDLY RECURRING TRACHEAL INFLAMMATORY MYOFIBROBLASTIC TUMOR WITH HISTIOCYTE-RICH HISTOLOGICAL PATTERN AND RARE ROS1 GENE REARRANGEMENT PRESENTING AS ASTHMA MIMIC" @default.
• W4387265816 doi "https://doi.org/10.1016/j.chest.2023.07.3018" @default.
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