Pediatric airway tumors: A report from the International Network of Pediatric Airway Teams (INPAT)

Abstract

Objective Primary tracheobronchial tumors (PTTs) are rare heterogeneous lesions arising from any part of the tracheobronchial tree. Nonspecific symptoms may lead to delayed diagnosis that requires more aggressive surgical treatment. An analysis of cases collected by the International Network of Pediatric Airway Team was undertaken to ensure proper insight into the behavior and management of PTTs. Methods Patients <18 years of age with a histological confirmation of PTT diagnosed from 2000 to 2015 were included in this multicenter international retrospective study. Medical records, treatment modalities, and outcomes were analyzed. The patient presentation, tumor management, and clinical course were compared between malignant and benign histotypes. Clinical and surgical variables that might influence event‐free survival were considered. Results Among the 78 children identified, PTTs were more likely to be malignant than benign; bronchial carcinoid tumor (n = 31; 40%) was the most common histological subtype, followed by inflammatory myofibroblastic tumor (n = 19; 25%) and mucoepidermoid carcinoma (n = 15; 19%). Regarding symptoms at presentation, wheezing (P = 0.001) and dyspnea (P = 0.03) were more often associated with benign growth, whereas hemoptysis was more frequently associated with malignancy (P = 0.042). Factors that significantly worsened event‐free survival were age at diagnosis earlier than 112 months (P = 0.0035) and duration of symptoms lasting more than 2 months (P = 0.0029). Conclusion The results of this international study provide important information regarding the clinical presentation, diagnostic workup, and treatment of PTTs in children, casting new light on the biological behavior of PTTs to ensure appropriate treatments. Level of Evidence NA Laryngoscope, 130:E243–E251, 2020

Extra‐appendicular neuroendocrine tumors: A report from the TREP project (2000‐2020)

“…The rarity of NETs and their peculiar feature to originate from different sites and causing variable, nonspecific symptoms may lead to an extremely delayed diagnosis that has been estimated by some authors to reach up to 10 years 3,14,16,28 . This has been confirmed by our findings.…”

“…Lung‐sparing surgery (sleeve resections and bronchoplasty for central NETs) 33 is considered the treatment of choice in bronchial NET. The endoscopic enucleation of selected tumors is also possible, but it has been described mostly in adults series, 33 and only anecdotally in children 28,34–36 . This approach is still debated 37,38 because of the risk of intraoperative bleeding and the impossibility to perform lymphadenectomy.…”

Case Report: Resection of Giant Endotracheal Hamartoma by Electrosurgical Snaring via Fiberoptic Bronchoscopy in a 9-Year-Old Boy

“…The presenting symptoms including cough, dyspnea, hemoptysis, chest pain, purulent sputum, and fever are induced by tracheobronchial obstruction and can lead to recurrent pneumonia (10). As the clinical symptoms are not specific and depend on the size and localization of the tumor, endotracheal hamartomas are often misdiagnosed as more benign conditions, delaying definitive treatment (6,11). Clinically, the differential diagnoses include aspirated foreign bodies, ectopic thyroid tissue, mucosal webs, cysts, as well as malignancies, including lipoma and lymphoma.…”

“…Tracheal or endobronchial hamartomas are traditionally treated by thoracotomy with bronchotomy or lung resection. At present, the firstline widely recommended approach in adults is endoscopic treatment; however, similar treatments have rarely been reported in children (6). We herein describe a case in which electrosurgical snaring via fiberoptic bronchoscopy (FB) was applied to successfully resect a rare tracheal hamartoma in a young boy, avoiding the need for bronchotomy.…”

Imaging of Airway Obstruction in Children

“…The most commonly seen lesions in school age children are carcinoid tumor (Figure 7), inflammatory myofibroblastic tumor and mucoepidermoid carcinoma (28). In general, these are best evaluated with CT.…”