Ep. Tagge et al., CHILDHOOD PLEUROPULMONARY BLASTOMA - CAUTION AGAINST NONOPERATIVE MANAGEMENT OF CONGENITAL LUNG CYSTS, Journal of pediatric surgery, 31(1), 1996, pp. 187-190
Pulmonary blastoma is a rare and aggressive malignant tumor that affec
ts children and adults. Recently a 3-year-old boy with a 2-year histor
y of bilateral unilocular pulmonary cysts was transferred for evaluati
on of a cough and high spiking fever. A chest radiogram showed left pu
lmonary consolidation with pleural effusion, but thoracentesis was uns
uccessful. Computerized tomography (CT) was suggestive of a pulmonary
abscess, but CT-guided drainage did not yield any purulent fluid. Perc
utaneous biopsies were performed, and the cytology showed malignant ce
lls. During thoracotomy, a large tumor involving the left lower lobe a
nd pleural space was found, and a biopsy was performed. A frozen secti
on showed blastemal and mesenchymal components devoid of neoplastic ep
ithelium, consistent with the pleural variant of pulmonary blastoma. A
left lower lobectomy, with tumor decortication of the pleural space,
achieved total gross tumor removal. The child received aggressive mult
iagent chemotherapy, and midway through it he underwent elective excis
ion of the opposite lung cyst. It has been 17 months since the lobecto
my; he is off chemotherapy and has no evidence of disease. A review of
the literature showed that a large number of pediatric pulmonary blas
tomas are associated with cystic lung disease. Because total tumor rem
oval offers the only chance of a good long-term outcome, surgical exci
sion or close follow-up of pulmonary cysts in children is strongly rec
ommended. Copyright (C) 1996 by W.B. Saunders Company.