Objective: To explore the clinical characteristics, pathological features, and diagnosis and treatment strategies of nasal chondromesenchymal hamartoma (NCMH) in infants and young children. Methods: A retrospective analysis was conducted on seven cases of NCMH infants and young children admitted to Beijing Children's Hospital, Capital Medical University from April 2015 to January 2022. The cohort included 5 males and 2 females, aged from 6 days to 2 years and 3 months. General information, clinical symptoms, imaging findings, treatment plans, postoperative complications, recurrence and follow-up time were collected, summarized and analyzed. Additionally, immunohistochemical characteristics of the lesion were examined. Results: The clinical symptoms of 7 children included nasal congestion, runny nose, open mouth breathing, snoring during sleep, difficulty feeding, and strabismus. All patients underwent electronic nasopharyngoscopy examination, with 5 cases of tumors located in the right nasal cavity and 2 cases in the left nasal cavity. No case of bilateral nasal cavity disease was found. All 7 patients underwent complete imaging examinations, with 5 patients underwent MRI and CT examinations, 1 patient underwent CT examination only, and 1 patient underwent MRI examination only. The CT results showed that all tumors were broad-based, with uneven density, multiple calcifications and bone remodeling, and some exhibited multiple cystic components. The MRI results showed that the tumor showed low signal on T1 weighted imaging and high or slightly high signal on T2 weighted imaging. All patients were diagnosed through histopathological examination and immunohistochemistry, including 7 cases of Ki-67 and SMA (+), 5 cases of S-100 and Vimentin (+), and all EMA and GFAP were negative. All patients underwent endoscopic resection surgery through the nasal approach, with 3 cases using navigation technology. Five cases of tumors were completely removed, and two cases of tumors were mostly removed. No nasal packing was performed after surgery, and no postoperative nasal, ocular, or intracranial complication occurred in all patients. Follow up assessments conducted 6 to 84 months post-surgery revealed no instances of tumor recurrence in any of the patients. Conclusions: The clinical symptoms of children with NCHM mainly depend on the size and location of the tumor. Nasal endoscopic surgery is the main treatment method. In cases where critical structures like the skull base or orbit are implicated, staged surgical interventions may be warranted. Long-term follow-up is strongly advised to monitor for any potential recurrence or complications.
目的： 探讨婴幼儿鼻软骨间叶错构瘤（nasal chondromesenchymal hamartoma，NCMH）的临床特点、病理特征及诊治策略。 方法： 回顾性分析2015年4月至2022年1月期间，首都医科大学附属北京儿童医院收治的7例NCMH婴幼儿病例，年龄6 d至2岁3个月，男性5例，女性2例。收集患儿一般资料、临床症状、影像学表现、治疗方案、术后并发症、复发及随访时间，以及病变的免疫组织化学特点等临床资料进行总结分析。 结果： 7例患儿的临床症状包括鼻塞、流涕、张口呼吸、睡眠打鼾、喂养困难及斜视。所有患儿均行电子鼻咽镜检查，肿物位于右侧鼻腔5例，左侧鼻腔2例，未见双侧鼻腔患病者。7例患儿均完善了影像学检查，5例患儿行MRI及CT检查，1例仅行CT检查，1例仅行MRI检查。CT结果显示所有肿瘤均为广基，密度不均匀，多有钙化灶和骨重塑，部分表现为多个囊性成分。MRI结果显示，肿物在T1加权像呈低信号，在T2加权像呈高或稍高信号。所有患儿通过组织病理学检查和免疫组织化学确诊，其中7例Ki-67、SMA（+），5例S-100、Vimentin（+），所有EMA和GFAP均为阴性。对所有患儿采用经鼻内镜入路切除手术，其中3例使用了导航技术。5例肿瘤完全切除，2例肿瘤大部分切除，术后均未行鼻腔填塞，所有患儿无术后鼻腔、眼部或颅内并发症出现。术后随访6~84个月，均未见复发。 结论： NCMH患儿的临床症状主要取决于肿瘤大小及部位，经鼻内镜手术是主要治疗方法，累及颅底或眼眶等重要结构时可考虑分期手术，建议长期随访。.