RHABDOMYOMATOSIS OF THE NEWBORN LUNG UNASSOCIATED WITH OTHER MALFORMATIONS

Aims: To describe a case of rhabdomyomatosis of the lung unassociated with other external or visceral malformations in a newborn infant. Met hods and results: A 16 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 sequestra tion were excluded after laboratory and arteriographic studies, No vis ceral anomalies were found, ift the age of 9 months the patient underw ent a partial lobectomy and he is free of disease 39 months after surg ery Histological examination demonstrated the presence of numerous bun dles of striated fibres arranged haphazardly in the pulmonary intersti tium in a background of a type-II congenital cystic adenomatoid malfor mation-like morphology of the resected lung. Conclusion: The presence of striated muscle fibres in the lung not necessarily represents a let hal congenital malformation, As this case shows, rhabdomyomatosis of t he lung fan affect a single pulmonary lobe, and resection of the affec ted 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 l ung.