Alkheder Ahmad, Al-Ghabra Yasser, Azar Adel, Dmirieh Ahmad, Mohsen Adham Bader Aldeen, Yousfan Abdulmajeed
Department of Otorhinolaryngology, Al Mouwasat University Hospital, Damascus University, Damascus, Syria.
Faculty of Medicine, Damascus University, Damascus, Syria.
Ear Nose Throat J. 2025 Jun 25:1455613251353637. doi: 10.1177/01455613251353637.
Inflammatory myofibroblastic tumor (IMT) is an uncommon mesenchymal neoplasm rarely encountered in the pediatric larynx, with the subglottis representing an atypical subsite. This report details a diagnostically instructive case of a 10-year-old girl presenting with 18 months of progressive dyspnea and exertional stridor, notably without hoarseness. Fibroendoscopic laryngoscopy identified a well-circumscribed right subglottic mass, confirmed on computed tomography scan to cause partial airway narrowing. The lesion's firm intraoperative consistency contrasted with common benign mimics. Histopathology revealed spindle cell proliferation with mixed inflammation (histiocytes, giant cells), while immunohistochemistry demonstrated focal anaplastic lymphoma kinase (ALK) and smooth muscle actin (SMA) positivity, CD68+ histiocytes, and CD1A negativity-collectively confirming IMT and excluding Langerhans cell histiocytosis. Complete endoscopic excision was achieved. This case underscores critical learning points: (1) IMT must be considered in children with progressive stridor/dyspnea, even lacking hoarseness, particularly with subglottic localization, and firm consistency; (2) Subglottic IMTs primarily manifest airway obstruction rather than voice changes typical of glottic lesions; (3) Focal ALK expression, present here, supports clonality but does not preclude a favorable outcome with complete resection; and (4) Immunohistochemistry (ALK/SMA/CD68/CD1A) is indispensable for accurate diagnosis and avoiding misdirected therapy. Despite excellent early postoperative results consistent with the generally favorable prognosis of pediatric IMT, vigilance for recurrence-highest within the first 2 years-remains essential. This report reinforces complete conservative resection as the cornerstone of management for localized disease, reserving adjuvant therapies for complex or recurrent scenarios. Multidisciplinary collaboration optimizes outcomes while preserving laryngeal integrity in children.
炎性肌纤维母细胞瘤（IMT）是一种罕见的间叶性肿瘤，在小儿喉部很少见，声门下是一个非典型的部位。本报告详细介绍了一例具有诊断指导意义的病例，一名10岁女孩出现进行性呼吸困难和劳力性喘鸣18个月，值得注意的是没有声音嘶哑。纤维喉镜检查发现一个边界清晰的右侧声门下肿物，计算机断层扫描证实该肿物导致部分气道狭窄。病变在术中质地坚硬，与常见的良性模仿病变不同。组织病理学显示梭形细胞增殖并伴有混合性炎症（组织细胞、巨细胞），而免疫组织化学显示局灶性间变性淋巴瘤激酶（ALK）和平滑肌肌动蛋白（SMA）阳性，CD68+组织细胞，CD1A阴性，共同证实为IMT并排除朗格汉斯细胞组织细胞增多症。通过内镜完全切除肿物。该病例强调了关键的学习要点：（1）对于进行性喘鸣/呼吸困难的儿童，即使没有声音嘶哑，特别是声门下定位且质地坚硬时，必须考虑IMT；（2）声门下IMT主要表现为气道梗阻，而非声门病变典型的声音改变；（3）此处存在的局灶性ALK表达支持克隆性，但并不排除完全切除后有良好的预后；（4）免疫组织化学（ALK/SMA/CD68/CD1A）对于准确诊断和避免治疗方向错误是必不可少的。尽管术后早期结果良好，与小儿IMT总体良好的预后一致，但对复发的警惕性（头2年内最高）仍然至关重要。本报告强化了完全保守切除作为局限性疾病管理的基石，将辅助治疗保留用于复杂或复发情况。多学科协作可优化治疗结果，同时保留儿童的喉部完整性。
