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Mesenchymal hamartomas of the chest wall in infancy: radiologic and pathologic correlation

机译:婴儿胸壁间质错构瘤:放射学和病理学相关性

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Mesenchymal hamartoma of the chest wall is a rare tumor with about 53 reported cases in the English literature. We reviewed six chest wall mesenchymal hamartomas in four patients, including two cases with multiple lesions, with specific focus on the radiologic and pathologic correlation. All cases occurred in neonates or infants with ages ranging from seven hours to seven months. They were diagnosed with plain chest radiographs (n=6), ultrasonography (n=2), chest CT scan (n=6), whole body bone scan (n=2) and MRI (n=3). All cases except a small one without cystic change showed the typical features of mesenchymal hamartoma radiographically and pathologically. Radiologically they were well-circumscribed masses with solid and cystic components with multiple fluid-fluid levels in association with single or multiple rib destruction or change. The CT scan showed the typical findings of chest wall hamartoma, and the MR showed heterogeneous signal intensities of the mass on T1- and T2-weighted images. The MR also revealed more concisely a secondary aneurysmal bone cyst formation with multiple fluid-fluid levels on the T2-weighted image. Microscopically, they showed alternating areas of cartilaginous islands and primitive appearing mesenchymal proliferation, which corresponded well with the solid component on the radiologic findings. The areas of bone formation and blood-filled cystic spaces matched the calcified or ossified densities and the cystic components, respectively. A small case without cystic change showed peculiar radiological and pathological findings resembling an osteochondroma. In conclusion, mesenchymal hamartoma of the chest wall in infancy is quite rare and sometimes can be misdiagnosed as malignancy due to the bone-destroying radiographic appearance and the highly cellular and mitotically active microscopic features, unless the radiologists and pathologists are aware of the characteristic clinical, radiological, and pathological findings. Imaging studies can usually make a correct diagnosis with good correlation to the pathologic findings.
机译:胸壁间质错构瘤是一种罕见的肿瘤,在英国文献中报道了约53例。我们回顾了4例患者中的6例胸壁间质错构瘤,其中包括2例具有多个病变的病例,特别侧重于放射学和病理学相关性。所有病例均发生在新生儿或婴儿中,年龄从七个小时到七个月不等。他们被诊断出有胸部平片(n = 6),超声检查(n = 2),胸部CT扫描(n = 6),全身骨扫描(n = 2)和MRI(n = 3)。除一例无囊性改变的病例外,所有病例均在影像学和病理学上显示间充质错构瘤的典型特征。放射学上,它们是界限分明的肿块,具有固体和囊性成分,具有多种流体-水平,伴有单个或多个肋骨破坏或改变。 CT扫描显示了典型的胸壁错构瘤征象,而MR显示了在T1和T2加权图像上肿块的异质信号强度。 MR还更简洁地显示了在T2加权图像上继发性动脉瘤性骨囊肿的形成,并伴有多种流体水平。在显微镜下,他们显示出软骨岛的交替区域和原始出现的间充质增生,这与放射学发现中的固体成分非常吻合。骨形成区域和充血的囊性空间分别与钙化或骨化密度和囊性成分相匹配。一例没有囊性变化的病例显示出特殊的放射学和病理学发现,类似于骨软骨瘤。总之,婴儿期胸壁间质错构瘤非常少见,有时由于破坏骨的放射学表现以及高度细胞和有丝分裂活性的微观特征而可能被误诊为恶性肿瘤,除非放射线医师和病理学家意识到其特征性临床表现,放射学和病理学发现。影像学检查通常可以做出正确的诊断,并且与病理结果具有良好的相关性。

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