Abstract
The most common malignant pulmonary neoplasms in children are metastases from other primary tumors, whereas primary pulmonary malignancies in children are rare. This chapter describes diverse neoplasms that originate from intrathoracic organs or structures or the chest wall, that have been described in children. Most of these neoplasms are malignant, and some are diagnosed almost exclusively in children, especially pleuropulmonary blastoma, which is the most common primary malignancy of the lung in children. Children with immunodeficiency or genetic syndromes such as the DICER1 syndrome, tuberous sclerosis complex, and others may be predisposed to some of these tumors. Evaluation of these tumors requires adequate tissue and at times unique expertise of a pathologist with prior experience in such tumors. New genomic modalities require fresh tissue at times both for diagnosis and for the identification of biomarkers and potential therapeutic targets. The tumors are described in sections which include the mediastinum, heart, and lungs and a brief discussion of tumors of the chest wall, diaphragm, and pleura. These are often arbitrary distinctions since delineation of the exact site of origin may be difficult and these neoplasms are found in all areas of the thorax.
Original language | English |
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Title of host publication | Textbook of Uncommon Cancer |
Subtitle of host publication | Fifth Edition |
Publisher | wiley |
Pages | 1095-1125 |
Number of pages | 31 |
ISBN (Electronic) | 9781119196235 |
ISBN (Print) | 9781119196204 |
DOIs | |
State | Published - Jan 1 2024 |
Keywords
- Chest wall
- Germ cell tumors
- Heart
- IMT
- Inflammatory myofibroblastic tumor
- Lungs
- Mediastinum
- Neurogenic tumors
- Pleuropulmonary blastoma
- PPB
- Thymoma