鼻软骨间充质错构瘤(NCMH)是一种非常罕见的,良性鼻窦肿瘤通常影响婴儿。在本文中,除了对NCMH的文献进行系统回顾之外,我们还报道了1例青少年患者发生NCMH的罕见病例.遵循PRISMA指南进行的系统审查。PubMed,利用EMBASE和通过相关出版物参考文献的手动搜索来收集所有已发表的NCMH病例报告。从每个病例报告中收集的患者人口统计数据,NCMH的位置和大小,临床表现,合并症,诊断方法,治疗方案和随访方法。系统审查收集了62例NCMH(包括我们的病例)的病例报告,涉及42名男性和21名女性(男女比例为2:1)。平均年龄为5.1岁(年龄范围:1天至70岁)。肿瘤的解剖部位为:鼻腔(n=17),鼻旁窦(n=30),轨道区(n=17),和头骨的底部(n=16)。报告的临床表现为鼻塞或充血(n=29),鼻肿块(n=27),鼻出血(n=6),眼眶症状(n=14)。NCMH是婴儿和幼儿鼻腔肿块的非常罕见的原因。我们的病例和以前的病例报告证实,NCMH可以模仿其他良性和恶性肿瘤,因此,我们应该警惕导致鼻腔肿块的罕见病理。最近,已经建立了DIECR1突变与NCMH之间的联系,因此,任何有DICER1相关肿瘤谱病史的鼻部或眼眶症状患者均应考虑NCMH.
Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign sinonasal tract tumor commonly affecting infants. In this paper, in addition to presenting a systematic review of the literature on NCMH, we also report an unusual
case of NCMH in an adolescent patient. A systematic review conducted following the PRISMA guidelines. PubMed, EMBASE and manual search through references of relevant publication were utilised to gather all published
case-
reports of NCMH. Data collected from each
case-report for patient demographics, site and size of NCMH, clinical presentation, co-morbidities, diagnostic methods, treatment options and follow-up methods. The systemic review collected sixty-two
case-
reports of NCMH (including our
case) affecting 42 men and 21 women (2:1 male to female ratio). Mean average age was 5.1 years (age range: 1 day to 70 years). The anatomical sites of the tumor were: nasal cavity (n = 17), paranasal sinuses (n = 30), orbital region (n = 17), and the base of the skull (n = 16). The reported clinical manifestations were nasal obstruction or congestion (n = 29), nasal mass (n = 27), epistaxis (n = 6), orbital symptoms (n = 14). NCMH is a very rare cause of nasal masses in infants and toddlers. Our
case and previous
case reports confirm that NCMH can mimic other benign and malignant tumors, therefore we should be vigilant for rare pathologies that lead to nasal masses. Recently the link between DIECR1 mutation with NCMH has been established, so NCMH should be considered in any patient with nasal or orbital symptoms with a history of DICER1-related tumor spectrum.