Prenatal presentation and outcome of children with pleuropulmonary blastoma.
OBJECTIVE
Pleuropulmonary blastoma (PPB) is a rare primary neoplasm of pleuropulmonary mesenchyme. Fewer than 170 children have been reported, and few single institutions have reported more than several cases. Treatment for this condition is primarily surgical resection; however, increasing experience suggests that adjuvant chemotherapy may decrease recurrence and improve outcome.


METHODS
We reviewed the charts of all children with PPB treated at our institution since 1960. We reviewed the prenatal features, clinical presentation, operation, pathological findings, adjuvant treatment, and outcome.


RESULTS
Ten children (6 boys and 4 girls) were treated for PPB at a mean age of 3.2 +/- 4.3 years. In 2, a cystic lung mass was diagnosed prenatally, and in 8, a cystic or solid and cystic lung mass was diagnosed postnatally (right lung, 3; left lung, 4; and bilateral, 3). In no patient was PPB considered preoperatively. Surgical resection was performed at 1 day to 11 years (median, 23 months) of age. Seven children had complete resection; 1 had microscopic residual disease, and 2 had gross residual disease. Pathology showed type I PPB in 7, type II in 1, and type III in 2. Five patients received adjuvant chemotherapy with vincristine, actinomycin, and cyclophosphamide-based regimens. At follow-up (mean, 7.7 +/- 11.5 years; range, 1-456 months), children with type I PPB have no evidence of disease (n = 6) or are lost to follow-up (n = 1), whereas all those with type II/III PPB have died of the disease.


CONCLUSIONS
PPB must be included in the differential diagnosis of a fetus, neonate, or child with a cystic lung mass. This finding supports early resection of these lesions rather than observation or treatment with nonoperative strategies.
Miniati
Douglas N
DN
Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX 77030, USA.
Chintagumpala
Murali
M
Langston
Claire
C
Dishop
Megan K
MK
Olutoye
Oluyinka O
OO
Nuchtern
Jed G
JG
Cass
Darrell L
DL
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008175:Lung Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D008297:Male; D011296:Prenatal Diagnosis; D018202:Pulmonary Blastoma / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome