Primary and metastatic lung tumors in the pediatric population: A review and 25-year experience at a large children's hospital
Primary lung neoplasms are rare in children, but they comprise a broad and interesting spectrum of lesions, some of which are familiar from other tissue sites, and some of which are unique to the pediatric lung. To determine the relative incidence of primary and metastatic lung tumors in children and adolescents through a single-institution case series, to compare these data to reports in the medical literature, to discuss the clinical and pathologic features of primary tumors of the tracheobronchial tree and lung parenchyma in children, and to provide recommendations for handling pediatric lung cysts and tumors. A 25-year single institutional experience with pediatric lung tumors, based on surgical biopsies and resections at Texas Children's Hospital from June 1982 to May 2007, an additional 40 lung tumors referred in consultation, and a review of the medical literature. A total of 204 pediatric lung tumors were diagnosed at our institution, including 20 primary benign lesions (9.8%), 14 primary malignant lesions (6.9%), and 170 secondary lung lesions (83.3%). The ratio of primary benign to primary malignant to secondary malignant neoplasms is 1.4:1:11.6. The common types of lung cancer in adults are exceptional occurrences in the pediatric population. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumor. Other primary pediatric lung tumors include congenital peribronchial myofibroblastic tumor and other myofibroblastic lesions, sarcomas, carcinoma, and mesothelioma. Children with primary or acquired immunodeficiency are at risk for Epstein-Barr virus-related smooth muscle tumors, lymphoma, and lymphoproliferative disorders. Metastatic lung tumors are relatively common in children and also comprise a spectrum of neoplasia distinct from the adult population.
[Show abstract] [Hide abstract] ABSTRACT: Atypical adenomatous hyperplasia is a premalignant lesion reflecting a focal proliferation of atypical cells. These lesions are usually observed as incidental findings in lungs that have been resected due to other conditions, such as lung cancer. We report the youngest case of atypical adenomatous hyperplasia on record in a 12-year-old girl. In this patient, the lesion was found in association with pneumothorax. © The Korean Society for Thoracic and Cardiovascular Surgery. 2016. All right reserved.0Comments 0Citations
- "The ratio of primary benign to primary malignant to metastatic malignant tumors was 1.4:1:11.6 . Cohen and Kaschula  reported a ratio of primary tumors to metastatic tumors to non-neoplastic lesions of 1:5:60. "
[Show abstract] [Hide abstract] ABSTRACT: No guidelines are in place for the follow-up and management of incidentally detected pulmonary nodules on CT in the pediatric population. The Fleischner guidelines, which were developed for the older adult population, do not apply to children. This review summarizes the evidence collected by the Society for Pediatric Radiology (SPR) Thoracic Imaging Committee in its attempt to develop pediatric-specific guidelines. Small pulmonary opacities can be characterized as linear or as ground-glass or solid nodules. Linear opacities and ground-glass nodules are extremely unlikely to represent an early primary or metastatic malignancy in a child. In our review, we found a virtual absence of reported cases of a primary pulmonary malignancy presenting as an incidentally detected small lung nodule on CT in a healthy immune-competent child. Because of the lack of definitive information on the clinical significance of incidentally detected small solid lung nodules on CT in children, the management of those that do not have the typical characteristics of an intrapulmonary lymph node should be dictated by the clinical history as to possible exposure to infectious agents, the presence of an occult immunodeficiency, the much higher likelihood that the nodule represents a metastasis than a primary lung tumor, and ultimately the individual preference of the child’s caregiver. Nodules appearing in children with a history of immune deficiency, malignancy or congenital pulmonary airway malformation should not be considered incidental, and their workup should be dictated by the natural history of these underlying conditions.0Comments 1Citation
- "In the most recent review article on pediatric lung cancer  , there is no reported initial presentation as an incidental pulmonary nodule — these rare tumors are often aggressive and tend to be symptomatic at the time of diagnosis11121314. Primary bronchogenic carcinoma is extremely rare in children and adolescents, and rare reported cases of squamous cell carcinoma present with a large tumor with a secondary respiratory infection . "
[Show abstract] [Hide abstract] ABSTRACT: The purpose is to delineate the clinical and pathological characteristics of rare primary malignant pulmonary tumors in children. Utilizing the National Cancer Data Base (NCDB), we analyzed all children (≤18years) with a primary malignant pulmonary tumor from 1998 to 2011 to identify factors associated with better survival. Of 211 children identified, the most common histology was carcinoid tumor (n=133, 63%) followed by mucoepidermoid carcinoma (MEC) (n=37, 18%), squamous cell carcinoma (SCC) (n=19, 9%), adenocarcinoma (n=16, 8%), bronchoalveolar carcinoma (BAC) (n=4, 2%), and small cell carcinoma (SCLC) (n=2, <1%). Factors that significantly affected survival include histology, race, tumor size, lymph node status, and extent of surgery. Patients with MEC and carcinoid tumors had a better overall survival compared to patients with other histologies (p<0.0001). The 5-year overall survival for MEC and carcinoid tumors was 100% and 95% (95% CI 87-98), respectively, versus 50% (95%CI 1-91) for BAC, 28% (95%CI 9-52) for SCC, and 26% (95%CI 5-55) for adenocarcinoma. The majority of pediatric patients with a primary malignant pulmonary tumor present with carcinoid tumor or MEC and have an excellent prognosis. Lung cancers which are common in adults, but rare in children, have a worse prognosis. Copyright © 2015. Published by Elsevier Inc.0Comments 3Citations
- "The OS of pulmonary carcinoid tumors in pediatric patients is excellent and in our study was 95%. Mucopidermoid carcinoma (MEC) is the second most common primary malignant epithelial pulmonary tumor seen in children [4,17]. In this study, MEC represented 18% of all primary epithelial lung tumors in children b18 years of age. "