Nelson Textbook of Pediatrics 22nd Edition | Primary pulmonary tumors in children are rare. Bronchial carcinoid (most common primary malignant tumor, low-grade, may metastasize), mucoepidermoid carcinoma (salivary gland type), inflammatory myofibroblastic tumor (pseudotumor, benign but locally invasive), pleuropulmonary blastoma (PPB, aggressive, associated with DICER1 mutations, cystic or solid). Metastatic tumors are more common: Wilms tumor, osteosarcoma, germ cell tumors, hepatoblastoma. Clinical: cough, hemoptysis, wheezing (unresponsive to bronchodilators), recurrent pneumonia (same location). Diagnosis: CXR, CT, bronchoscopy, biopsy. Treatment: surgical resection (lobectomy, bronchoscopic excision), chemotherapy for PPB, DICER1 genetic testing and family screening.
🌐 paeds.online — Pakistan's Pediatric Platform| Tumor | Key Features | Treatment |
|---|---|---|
| Bronchial carcinoid | Most common primary malignant, low-grade, may metastasize, central or peripheral | Surgical resection (lobectomy, bronchoscopic excision), surveillance |
| Mucoepidermoid carcinoma | Salivary gland type, low-grade, central airway | Surgical resection |
| Inflammatory myofibroblastic tumor (IMT) | Benign, locally invasive, may recur | Surgical excision (complete resection) |
| Pleuropulmonary blastoma (PPB) | Aggressive, DICER1 mutations, cystic or solid, types I-IV | Surgery + chemotherapy (if type II/III/IV), DICER1 genetic testing, family screening |
| Metastatic tumors | Wilms tumor, osteosarcoma, germ cell tumors, hepatoblastoma | Treat primary tumor, metastasectomy if limited |