Pulmonary metastases at diagnosis of neuroblastoma in pediatric patients: CT findings and prognosis.

Abstract

We undertook this study to determine the frequency, CT appearance, and clinical implications of the rare occurrence of pulmonary metastases among children presenting with neuroblastoma. A search of the Children's Cancer Group database revealed 21 of 567 children with reported lung metastases at original diagnosis of neuroblastoma. CT examinations available for 17 of these patients were analyzed retrospectively to determine if lung metastases were present, and if so, to characterize their radiographic features. Seventeen (3%) of 567 patients presenting with Evans stage IV neuroblastoma had confirmed pulmonary metastases at diagnosis. All had metastases to at least one site other than the lungs. The most common CT appearance of pulmonary lesions was of up to five, small, bilateral, noncalcified nodules. In nine patients (53%), the pulmonary nodules initially resolved with treatment. In this cohort, six children developed progressive disease and died, and three are still alive. All eight children whose lung lesion did not completely respond to treatment died. Overall, children with pulmonary metastases had unfavorable Shimada histology, a higher association with amplification of the MYCN oncogene (p = 0.0002), and a decreased event-free survival (p < 0.001) when compared with all children with stage IV neuroblastoma without pulmonary metastases. The search for neuroblastoma lung metastases, which occur more frequently than previously reported, is clinically important because their presence portends a poor prognosis.