Bronchial Carcinoid Tumors in Children and Adolescents.

PURPOSE

Carcinoid neoplasms, even rare, are the most common pulmonary tumors during childhood. They are classically divided into typical and atypical tumors according to pathology pattern. Cornerstone therapy is to obtain a complete tumor resection. The aim was to describe clinical characteristics and treatment of young patients diagnosed with bronchial carcinoid tumors (BCT) in France.

METHODS

Using the National Registry of Childhood Cancers and the pediatric very rare tumors FRACTURE (French Group for Rare Childhood Tumors) database, we conducted a nationwide retrospective multicenter study including patients aged under 18 years diagnosed with a BCT between 2011 and 2022.

RESULTS

Thirty-eight patients were included (median age: 15.6 years). Diagnosis was performed during bronchoscopy (n = 23) or by immediate resection (n = 14) (missing data [MD]: 1). Twenty-six patients (68%) had typical carcinoid tumors. Tumors were mainly localized (T1/T2-N0-M0; 28 cases); five had regional lymph node involvement (LN+) and none had metastatic disease. All patients underwent tumor resection, mainly with lobectomy (n = 29) associated to lymph node dissection (35 cases, including five LN+; MD: three). Pneumonectomy was required in three cases. Margin status was complete in 36 cases (microscopic residue: two cases). No patient received medical treatment as first-line therapy. With a median follow-up of 33 months (range: 0-120), one patient with N0 R0 typical BCT developed a distant relapse. Five-year progression-free and overall survivals were, respectively, 87.5% (95% confidence interval: 38.7-98.1) and 100%.

CONCLUSION

BCTs are rare and frequently "typical," with a very favorable prognosis in children after an exclusively surgical strategy, even in LN+ cases.