Schumacher R, Mai A, Gutjahr P
University Children's Hospital, Mainz, Federal Republic of Germany.
Eur J Pediatr. 1992 Jun;151(6):432-4. doi: 10.1007/BF01959357.
A relationship exists between tumours and malformations both generally and in particular combinations. This is also valid for minor errors of morphogenesis suggesting that embryonic tumours are an expression of aberrant intra-uterine morphogenesis. We speculated that these minor aberrations might also manifest in other morphological defects, especially in minor anomalies and malformations of the ribs. We reviewed chest roentgenographs of 1000 children with malignancies for rib anomalies and compared them to 200 patients with mainly infectious diseases. We found 242 rib anomalies in 218 children with tumours (21.8%) and 11 (5.5%) in children without malignancy. This difference was statistically highly significant (P less than 0.001). A high incidence of cervical ribs was found in neuroblastoma (33%), brain tumour (27.4%), leukaemia (26.8%), soft tissue sarcoma (24.5%), Wilms tumour (23.5%) and Ewing sarcoma (17.1%). Only neuroblastoma showed a high incidence of rib bifurcation (4.5%). The increased incidence of these mesenchymal defects in children with malignancies may be another clue for an altered morphogenesis in tumour origin. In neuroblastoma the rib anomaly may be another expression of neurocristopathy as proposed for the association of congenital heart disease and neuroblastoma.
肿瘤与畸形之间存在着普遍的关系，在特定组合中也是如此。这对于形态发生的微小错误同样适用，提示胚胎肿瘤是子宫内形态发生异常的一种表现。我们推测这些微小异常也可能表现在其他形态学缺陷中，尤其是肋骨的微小异常和畸形。我们回顾了1000例患有恶性肿瘤儿童的胸部X光片以检查肋骨异常情况，并将其与200例主要患有感染性疾病的患者进行比较。我们在218例患有肿瘤的儿童中发现了242处肋骨异常（21.8%），而在无恶性肿瘤的儿童中发现了11处（5.5%）。这种差异在统计学上具有高度显著性（P小于0.001）。在神经母细胞瘤（33%）、脑肿瘤（27.4%）、白血病（26.8%）、软组织肉瘤（24.5%）、肾母细胞瘤（23.5%）和尤因肉瘤（17.1%）中发现颈肋的发生率较高。只有神经母细胞瘤显示出较高的肋骨分叉发生率（4.5%）。恶性肿瘤患儿中这些间充质缺陷发生率的增加可能是肿瘤起源中形态发生改变的另一个线索。在神经母细胞瘤中，肋骨异常可能是神经嵴病的另一种表现，就如同先天性心脏病与神经母细胞瘤的关联所提出的那样。
