Primary and metastatic lung tumors in the pediatric population: a review and 25-year experience at a large children's hospital.
ABSTRACT 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.
SourceAvailable from: Sjirk J Westra[Show abstract] [Hide abstract]
ABSTRACT: The incidental detection of small lung nodules in children is a vexing consequence of an increased reliance on CT. We present an algorithm for the management of lung nodules detected on CT in children, based on the presence or absence of symptoms, the presence or absence of elements in the clinical history that might explain these nodules, and the imaging characteristics of the nodules (such as attenuation measurements within the nodule). We provide suggestions on how to perform a thoughtfully directed and focused search for clinically occult extrathoracic disease processes (including malignant disease) that may present as an incidentally detected lung nodule on CT. This algorithm emphasizes that because of the lack of definitive information on the natural history of small solid nodules that are truly detected incidentally, their clinical management is highly dependent on the caregivers’ individual risk tolerance. In addition, we present strategies to reduce the prevalence of these incidental findings, by preventing unnecessary chest CT scans or inadvertent inclusion of portions of the lungs in scans of adjacent body parts. Application of these guidelines provides pediatric radiologists with an important opportunity to practice patient-centered and evidence-based medicine.Pediatric Radiology 02/2015; DOI:10.1007/s00247-014-3267-7 · 1.65 Impact Factor
[Show abstract] [Hide abstract]
ABSTRACT: Rare pediatric tumors are a heterogenous group of tumors that are rare at any age, or rare in children and common in adults. These are orphan diseases which have not benefitted from the significant advances seen in other pediatric malignancies. To improve our understanding of these tumors, rare tumor registries and tissue repositories have been established. The development of registries has followed two different models: in Europe, the focus has been on large co-operative group registries that enroll the breath of rare tumors, while in the USA, individual rare tumor registries have predominated. Both types of registries have significantly improved our understanding of these tumors. The co-operative group registries have provided valuable information on some very rare tumors, leading to the development of therapeutic guidelines, with the goal of standardizing treatment. On the other hand, the individual rare tumor registries have focused on single tumors, providing granular clinical and molecular information. They have defined the molecular pathogenesis of these tumors and provided new models to understanding carcinogenesis. We discuss the important contributions made by these registries.06/2015; 3(2). DOI:10.1007/s40124-015-0077-9
[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.Pediatric Radiology 02/2015; DOI:10.1007/s00247-014-3269-5 · 1.65 Impact Factor