Adenocarcinoma of the Lung in Childhood and Adolescence: A Systematic Review.

Primary lung cancer is extremely rare in children. It often presents with metastatic disease and carries a poor prognosis. Adenocarcinoma is the most common type of bronchogenic carcinoma in children and adults. Our aim was to systematically review the presenting features, approach to diagnosis and management, as well as the outcomes of primary pediatric adenocarcinoma of the lung. This systematic review was prospectively registered with PROSPERO. The following databases were searched: Medline, Embase, Web of Science, and Scopus for English language cases of primary pediatric adenocarcinoma of the lung. Forty-eight studies were included, comprising 62 patients with adenocarcinoma and 21 cases of adenocarcinoma in situ. Presenting features were nonspecific, with cough and dyspnea the main symptoms at diagnosis. The majority of patients with adenocarcinoma had metastatic disease at diagnosis. Surgery was the most common form of management. More than half the patients with adenocarcinoma had died at final follow-up, whereas 5 of 21 with adenocarcinoma in situ died. Medical management did not improve outcomes, except for two ALK receptor tyrosine kinase (ALK)-rearranged adenocarcinomas that responded to ALK inhibitor therapy alone. Primary pediatric adenocarcinoma of the lung is a rare entity which often presents with metastatic disease and portends a poor prognosis. Surgery is associated with disease-free status, although new agents such as ALK-inhibitors are able to prolong life without surgical management.