Hardisson D, García-Jiménez J A, Jiménez-Heffernan J A, Nistal M
Department of Pathology, University Hospital La Paz, Faculty of Medicine, Autonomous University of Madrid, Spain.
Histopathology. 1997 Nov;31(5):474-9. doi: 10.1046/j.1365-2559.1997.2830880.x.
To describe a case of rhabdomyomatosis of the lung unassociated with other external or visceral malformations in a newborn infant.
A 26 weeks gestation newborn male with no relevant medical or family history presented a well-circumscribed solid area in the posterior mediastinum occupying the upper lobe of the right lung. The possibility of neuroblastoma or an extralobar pulmonary sequestration were excluded after laboratory and arteriographic studies. No visceral anomalies were found. At the age of 9 months the patient underwent a partial lobectomy, and he is free of disease 39 months after surgery. Histological examination demonstrated the presence of numerous bundles of striated fibres arranged haphazardly in the pulmonary interstitium in a background of a type-II congenital cystic adenomatoid malformation-like morphology of the resected lung.
The presence of striated muscle fibres in the lung not necessarily represents a lethal congenital malformation. As this case shows, rhabdomyomatosis of the lung can affect a single pulmonary lobe, and resection of the affected lung parenchyma may be curative. It is important for pathologists to be aware of this entity, although it is exceptional, and to include it in the differential diagnosis of pulmonary masses in the newborn lung.
描述一例新生儿肺部横纹肌瘤病，该病例未伴有其他外部或内脏畸形。
一名孕26周的男婴，无相关病史及家族史，后纵隔出现一个边界清晰的实性区域，占据右肺上叶。经实验室检查和动脉造影研究后，排除了神经母细胞瘤或肺叶外隔离症的可能性。未发现内脏异常。患儿9个月时接受了部分肺叶切除术，术后39个月无疾病复发。组织学检查显示，在切除肺呈现II型先天性囊性腺瘤样畸形形态的背景下，肺间质中有大量随意排列的横纹肌纤维束。
肺内出现横纹肌纤维不一定代表致命的先天性畸形。如本病例所示，肺部横纹肌瘤病可累及单个肺叶，切除受累肺实质可能治愈疾病。尽管这种情况罕见，但病理学家认识到这一实体并将其纳入新生儿肺部肿块的鉴别诊断中很重要。
