儿童软骨间叶性错构瘤的影像表现及临床病理特征

Imaging manifestations and clinicopathological features of chondromesenchymal hamartoma in children

目的探讨儿童软骨间叶性错构瘤(CMH)的影像表现及临床病理特征。方法收集并分析8例经手术病理证实的儿童CMH的影像资料及其临床病理资料。结果8例患儿中,男6例,女2例;其中4例表现位于鼻部,4例位于胸壁;发病年龄为出生1 d至3岁。鼻部病变的症状主要为鼻腔肿物、鼻塞及眼球突出;CT表现为混杂密度软组织肿块伴钙化,可向颅内或眼眶侵蚀;磁共振成像(MRI)呈T1WI低、T2WI高信号为主的混杂信号表现,增强后明显不均匀强化。胸壁病变多发生于肋骨,临床主要表现为胸壁肿块;CT检查显示边界清楚的膨胀性骨质破坏,伴边缘为主的钙化或骨化;MRI显示肿块呈囊实性混杂信号,部分可见继发性动脉瘤样骨囊肿的出血性液-液平面。组织病理学可见大小不等的透明软骨伴软骨内骨化,免疫组织化学检查显示软骨细胞主要表达波形蛋白(Vimentin)和S-100蛋白,梭形间质细胞表达平滑肌肌动蛋白(SMA)。结论CMH是一种多见于婴幼儿鼻部与胸壁的罕见良性病变,具有独特的影像学及临床病理特点,术前准确诊断对于患儿的治疗和预后至关重要。

Objective To investigate the imaging manifestations and clinicopathological features of chondromesenchymal hamartoma(CMH)in children.Methods The clinicopathological data of 8 cases of CMH child confirmed by surgery and pathology were collected and analyzed.Results Among 8 cases,6 cases were male and 2 cases were female.Among them,4 cases were located in the nose and 4 cases in the chest wall.The onset age ranged from 1 d after birth to 3 years old.The symptoms of nasal lesion were mainly nasal cavity mass,nasal congestion and exophthalmos.CT showed the mixed density soft tissue mass accompanied by calcification,which could erode into the intracranial cavity or orbit.MRI showed the mixed signals with low T1WI and high T2WI signals,and obviously uneven enhancement after enhancement.Its chest wall lesion mostly occur in the ribs,and the main clinical manifestation was the chest wall mass.The CT examination showed the expansive bone destruction with clear boundary,accompanied by calcification or ossification mainly at the edge.MRI showed that the mass showed the mixed signal of cystic and solid,and the hemorrhagic fluid-liquid level of secondary aneurysmal bone cyst could be seen in some cases.Histopathology showed varying sizes of hyaline cartilage with endochondral ossification,and the immunohistochemistry showed that chondrocytes mainly expressed Vimentin and S-100 protein,and spindle interstitial cells expressed smooth muscle actin(SMA).Conclusion CMH is a rare benign lesion in the nose and chest wall of infants and young children.It has unique imaging and clinicopathological features.Accurate diagnosis before operation is very important for the treatment and prognosis of the children patients.