Bronchial carcinoid tumor, while rare, remains the most common primary malignant lung tumor in children. We present a retrospective analysis of 7 patients with typical bronchial carcinoid tumors diagnosed at 2 pediatric tertiary care referral centers between 1990 and 2014. The most common presenting symptom was pneumonia, followed by respiratory distress. Somatostatin scans were performed in selected patients. All patients had negative resection margin following surgery and were alive without disease at last follow-up. Typical carcinoid tumors have a good prognosis following definitive surgical resection. A review of published literature on pediatric bronchial carcinoid tumors is provided.
Purpose
We performed a phase 2 study in children with recurrent or refractory leptomeningeal leukemia to determine the objective response rate after treatment with intrathecal topotecan.
Patients and Methods
Patients received age-adjusted intrathecal topotecan (0.4 mg/dose for patients > 3 years of age) administered twice weekly (every 3 to 4 days) for six weeks during induction, weekly for 4 weeks during consolidation, and twice monthly for 4 months and then monthly thereafter during maintenance.
Results
Twenty-two patients enrolled in the study, of whom 20 were eligible and assessable for toxicity and 16 were assessable for response. Of 16 patients, 6 (38%) had a complete response, 8 (50%) had stable disease, and 2 (13%) had progressive disease. The median EFS time (95% CI) was 3.1 (1.6 – 10.3) months and the median overall survival time (95% CI) was 18.0 (7.3 – 38.3) months. Eight patients (40%) experienced grade 3 or 4 adverse events. There were no grade 4 neurological events (Table III). Four patients experienced a total of 6 grade 3 neurological events including an olfactory seizure, a headache, transient grade 3 speech impairment, muscle weakness, motor neuropathy, and ataxia. Headache was the most common grade ≤ 2 neurologic event and two patients developed grade ≤ 2 arachnoiditis.
Conclusion
Intrathecal administration of topotecan was tolerable on this dose and schedule. The majority of adverse events were mild to moderate, reversible side effects. Complete central nervous system remissions were achieved in a subset of children with recurrent or refractory CNS leukemia.
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