Bronchial carcinoid tumors in children and adolescents - A report and management considerations from the German MET studies

Lung Cancer. 2023 Sep:183:107320. doi: 10.1016/j.lungcan.2023.107320. Epub 2023 Aug 5.


Objectives: Bronchial carcinoid tumors (BC) are exceptionally rare in childhood, with an incidence of <0.2/1,000,000 per year. Typical low-grade BCs are distinguished from atypical, intermediate-grade BCs. Little is known about BCs in pediatric patients and management guidelines are missing. In this study, we explored characteristics and outcome of pediatric patients with BC prospectively registered with the Malignant Endocrine Tumor studies.

Material and methods: We performed a retrospective multicenter study in children, adolescents, and young adults (aged 0-20 years) with BC reported to the German MET registry between January 1997 and December 2022. Data were last updated on 28 of February 2023.

Results: Thirty-two patients were diagnosed at a median age of 15.0 years (range, 9.8-19.2). Atypical BCs (23.3%) were less frequent than typical, but more common than in adulthood. Lymph node metastases were present in 14.3% of cases (atypical BC: 28.6%, typical BC: 10.5%), distant metastases in one (3.1%) patient with atypical BC. 92.6% of patients were in complete remission after surgical resection (median follow-up: 2.7 years). The patient with metastatic spread and one patient with atypical BC and multiple recurrences were on treatment at last follow-up. 5-year event-free survival of typical BC was 100% and 83.3% in atypical BC.

Conclusions: Completely resected localized BCs in pediatric patients have a favorable outcome also with lung tissue sparing surgery. Atypical BC with risk of metastatic spread and recurrence occurred more frequently compared to adults. Interdisciplinary management and collaborative efforts are needed to improve our understanding and the management of pediatric BC.

Keywords: Bronchial carcinoid; Children; EXPeRT; Lung carcinoid; MET; Rare tumor.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Bronchial Neoplasms* / diagnosis
  • Bronchial Neoplasms* / epidemiology
  • Bronchial Neoplasms* / therapy
  • Carcinoid Tumor* / diagnosis
  • Carcinoid Tumor* / epidemiology
  • Carcinoid Tumor* / therapy
  • Child
  • Humans
  • Lung Neoplasms* / pathology
  • Lymphatic Metastasis
  • Pneumonectomy
  • Progression-Free Survival
  • Retrospective Studies
  • Young Adult