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Pediatric pulmonary tumors are extremely uncommon entities without a unifying presenting symptomatology, clear diagnostic strategy, or definitive therapeutic regimens. Patients may present with all manner of symptoms or complaints, and these concerns are almost always nonspecific. Secondary to their rarity and the heterogeneous nature of the presenting findings, tumors are seldom thought of as the causative factor for a child presenting to a primary care provider with respiratory complaints, especially in light of the prevalence of other, far more common diseases in young patients such as reactive airway disease, upper and lower respiratory infections, or even inhaled/ingested aerodigestive foreign bodies. The published data document that the proportion of benign lesions to metastatic lesions to primary malignancies is on the order of 60:5:1.1,2 As such, the most critical factor in diagnosing these lesions is a high index of suspicion, especially if symptoms do not abate with the intended treatment strategies directed at the suspected underlying causative agent (i.e., asthma that is refractory to standard therapies or the respiratory infection that fails to resolve in a timely fashion with appropriate antimicrobial drugs). There is often a significant delay then, from the initial presentation to a healthcare provider and definitive histopathologic diagnosis. Staging, treatment strategies, and outcomes will be tumor-specific, but surgery generally plays a critical role if cure is to be achieved in all lesions.
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General Considerations
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Presentation/Evaluation
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Presenting symptoms in pediatric patients can range from the incidentally found tumor or lesion in the asymptomatic child to the child who presents in extremis with severe hemorrhage or cardiorespiratory collapse from parenchymal or mediastinal compression or invasion. The size, location, number, degree of vascularity, specific tracheobronchial anatomic location, associated organ (heart, great vessels) or structure (trachea) involvement (directly or indirectly), and malignant potential all are factors known to influence the precise constellation of presenting symptoms. Patients presenting with benign tumors are most commonly asymptomatic (24%).3,4 However, when patients with benign tumors do present with symptoms, fever, cough, and pneumonitis have been documented to have the highest reported frequency (~10%) in this cohort. Those pediatric patients who are found to have metastatic lung cancers are also most commonly asymptomatic, as these lesions are generally small, numerous, and peripherally located on staging studies. Seldom does the child with pulmonary metastases present with pulmonary symptoms as the initial physical complaint. Primary malignant tumors in children generally do present with symptoms, however, and two large series found only 6% of children were asymptomatic upon presentation.3,4 In fact, these reports document that one-third of patients present with a refractory cough, and a not so insignificant number (10%–25% per symptom) also present with evidence of fever, pneumonitis, respiratory distress, or hemoptysis.
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As previously stated, however, these symptoms are nonspecific, and definitive diagnosis is almost always delayed. A high index of suspicion with close, diligent follow-up is mandated in ...