Domizio P, Liesner R J, Dicks-Mireaux C, Risdon R A
Department of Histopathology, Hospital for Sick Children, London, United Kingdom.
Pediatr Pathol. 1990;10(5):785-97. doi: 10.3109/15513819009064712.
Primary lung tumors are uncommon in children, and malignant mesenchymal tumors form only a small proportion of these. Leiomyosarcomas occur more commonly than rhabdomyosarcomas, whereas malignant mesenchymomas are exceedingly rare. Of the total number of primary pulmonary rhabdomyosarcomas and malignant mesenchymomas of lung reported in children, 50% have occurred in association with congenital lung cysts. The relationship between abnormal morphogenesis and neoplasia is well documented in the kidney. A similar relationship may exist in the lung between cystic parenchymal maldevelopment and embryonal mesenchymal tumors. We report a 4-year-old boy with a malignant mesenchymoma of lung arising within a congenital lung cyst; one similar case has previously been reported to our knowledge.
原发性肺肿瘤在儿童中并不常见,恶性间叶性肿瘤仅占其中的一小部分。平滑肌肉瘤比横纹肌肉瘤更常见,而恶性间叶瘤极为罕见。在儿童报告的原发性肺横纹肌肉瘤和肺恶性间叶瘤总数中,50%与先天性肺囊肿有关。肾脏中形态发生异常与肿瘤形成之间的关系已有充分记录。在肺中,囊性实质发育异常与胚胎性间叶肿瘤之间可能也存在类似关系。我们报告一名4岁男孩,其先天性肺囊肿内发生了肺恶性间叶瘤;据我们所知,此前曾报告过1例类似病例。