Clinical characteristics and treatment outcomes of neuroblastoma with pulmonary metastases.
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OBJECTIVE: To describe the clinical characteristics, treatment processes, and prognosis of primary neuroblastoma (NB) with concurrent pulmonary metastases. METHODS: Clinical data were collected retrospectively from 10 patients with primary NB and concurrent pulmonary metastases in our institution from March 2019 to October 2025. We collected and analyzed clinical and pathological features, imaging characteristics, and therapeutic responses. RESULTS: This cohort comprised seven boys and three girls, with a median age of 45.5 months (range 29-200 months). All patients were classified as stage M and high-risk group. N-myc proto-oncogene (MYCN) amplification was seen in four cases and gain in three cases. Heterogeneous responses to induction chemotherapy were observed. The primary tumor and bone marrow metastases showed favorable responses, whereas pulmonary metastases exhibited low sensitivity to chemotherapy. Nine patients died with a median survival of 17 months (range 5-64 months); one patient survived for 62 months. In the later stages of treatment, tumor progression or recurrence occurred in 9 patients, with bone (n=4), liver (n=4), lung (n=4), primary tumor site (n=3), distant lymph nodes (n=2), brain (n=2), and bone marrow (n=1) being the sites of first progression or recurrence. CONCLUSIONS: Primary NB with pulmonary metastases is uncommon, frequently associated with MYCN amplification and multiple non-pulmonary metastases; prognosis is poor. Current clinical management predominantly involves systemic therapy, with limited local interventions for pulmonary metastases. Treatment responses vary across primary tumors and different metastatic sites.