Background and Aims: Primary lung carcinomas are very rare childhood
tumors with an incidence of <2/1,000,000 per year as defined
by the European Cooperative Study Group for Pediatric Rare Tumors
(EXPeRT). They represent a challenge to treating physicians as there
are few reports on these diseases at this age and no specific recommendations
are available. This collaborative analysis of the EXPeRT
group was conducted to improve knowledge about the treatment and
prognosis of primary lung carcinomas in pediatric patients.
Methods:Aretrospective European analysis of all pediatric patients (0-
18 years) with primary lung carcinomas prospectively collected in the
EXPeRT databases between 2000 and 2021 was performed. Clinical
data including outcomes were analyzed.
Results: Thirty-eight patientswere identified with a median age of 12.8
years at diagnosis (range 0 to 17 years). Mucoepidermoid carcinoma
(MEC) was the most common entity (n=20), followed by adenocarcinoma
(n=12), squamous cell carcinoma (SCC; n=4), adenosquamous
carcinoma (n=1) and small-cell lung cancer (n=1). Lymph node metastases
occurred rarely in patients with MEC (2 cases), and 19 patients
achieved durable remission after surgical resection only. One patient
with MEC died after progression of metastasis. Patients with histology
other thanMEC often presentedwith advanced disease (stage IV in 14
of 18 cases) and needed multimodality treatment. They had a combined
survival rate of 44%. While all patients with SCC died, the 12 patients
with adenocarcinoma had a survival rate of 50%.
Conclusions: Primary lung carcinoma occur rarely in children. While
patients with MEC have a favorable outcome with a survival rate of
95%, patients with other lung carcinoma entities have an unfavorable
outcome despite multimodality treatment strategies. This analysis will
help propose consensus guidelines for diagnosis and therapy