Primary and metastatic lung tumors in the pediatric population: A review and 25-year experience at a large children's hospital

Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX 77030, USA.
Archives of pathology & laboratory medicine (Impact Factor: 2.84). 08/2008; 132(7):1079-103. DOI: 10.1043/1543-2165(2008)132[1079:PAMLTI]2.0.CO;2
Source: PubMed


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.

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    • "Common metastatic tumors include Wilms tumors , osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma [3] [4]. Previously described benign lesions include inflammatory myofibroblastic tumors, hamartomas, and chondromas [2] [4]. "
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    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.
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    • "In the case of FLIT, the bulk of the mass appears as a solid expansion of immature lung interstitium with uniform cell proliferation and without cartilaginous foci. In comparison, classic early PPB usually has grossly larger air-filled cysts and thin septa lacking an epithelial component (Dishop and coworkers, 2008, 2010). Based on these findings, the current case was diagnosed as FLIT. "
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    • "In a 25-year experience of Texas Children’s Hospital, carcinoid tumor of the lung was found to be the minority cause of lung masses. Surgical intervention was preferred as the primary treatment.54 "
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