The case of a 22-month-old boy with alveolar rhabdomyosarcoma of the l
ung is presented. Brain metastasis and recurrence of the right pulmona
ry hilum and parietal pleura developed 6, 11, and 24 months (respectiv
ely) after tumor resection. Chemotherapy and radiotherapy were effecti
ve. Neuron-specific enolase was very helpful in detecting metastasis a
nd disease recurrence. Primary pulmonary rhabdomyosarcoma can be divid
ed into two groups: tumor in the normal lung, and tumor in cystic lesi
ons of the lung. Copyright (C) 1995 by W.B. Saunders Company.