Kim J Y, Jung W H, Yoon C S, Kim M J, Kim H K, Kim K D, Cho S H
Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.
Yonsei Med J. 2000 Oct;41(5):615-22. doi: 10.3349/ymj.2000.41.5.615.
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.
胸壁间叶性错构瘤是一种罕见肿瘤,英文文献中报道约53例。我们回顾了4例患者的6例胸壁间叶性错构瘤,其中2例为多发病变,特别关注了影像学与病理学的相关性。所有病例均发生于新生儿或婴儿,年龄从7小时至7个月不等。诊断方法包括胸部平片(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1例无囊性改变的小病例外,所有病例在影像学和病理学上均表现出间叶性错构瘤的典型特征。影像学上,它们为边界清晰的肿块,有实性和囊性成分,伴有多个液-液平面,伴有单根或多根肋骨破坏或改变。CT扫描显示了胸壁错构瘤的典型表现,MRI在T1加权和T2加权图像上显示肿块信号强度不均匀。MRI还更清晰地显示了T2加权图像上伴有多个液-液平面的继发性动脉瘤样骨囊肿形成。显微镜下,它们显示出软骨岛和原始间叶细胞增生交替出现的区域,这与影像学表现中的实性成分相符。骨形成区域和充满血液的囊性空间分别与钙化或骨化密度以及囊性成分相对应。1例无囊性改变的小病例表现出类似骨软骨瘤的特殊影像学和病理学表现。总之,婴儿期胸壁间叶性错构瘤非常罕见,有时由于影像学上的骨质破坏表现以及显微镜下细胞丰富和有丝分裂活跃的特征,可能被误诊为恶性肿瘤,除非放射科医生和病理科医生了解其特征性的临床、影像学和病理学表现。影像学检查通常能与病理结果良好关联从而做出正确诊断。