Surgical treatment of primary tracheal tumors in children and adolescents
Pediatric primary tracheal tumors (PTTs) are rare entities, with an estimated incidence of 0.2% among all neoplasms in children and adolescents. Benign lesions and malignancies are rather equally represented, with subglottic hemangioma, granular cell tumor, carcinoid tumor, and low-grade mucoepidermoid carcinoma being the most common histotypes. Guidelines on management and treatment of pediatric PTTs are not available, and only a few reviews, case reports, and single surgeon/institute experiences have been published. A literature search was carried out focusing on the surgical approaches utilized in the most recent publications to manage pediatric PTTs, thus including case reports, single institute experiences, and reviews on surgical techniques. Moreover, we divided the available intervention modalities based on an anatomical classification, distinguishing between laryngotracheal, cervical tracheal, lower tracheal, and carinal scenarios. Endoscopic treatment is still underused, even for benign lesions: this is mainly due to the difficulties that surgeons may experience during airway management, appropriate tumor visualization, and its safe removal with adequate prevention/management of intraoperative complications. Considering open surgery, simple tumorectomy via median thyrotomy is the treatment of choice whenever possible (mainly for benign tumors), whereas in case of wider excisions, laryngotracheal resection and reconstruction with cartilage graft, end-to-end anastomosis, or neo-carinal reconstruction represent the most widely used procedures. All these techniques are similar to what described in adults. Strict cooperation with an experienced team of pediatric anesthesiologists and intensivists is of paramount importance for the adequate management of PTTs in young children. We are far from establishing a common guideline for treatment of PTTs. Mini-invasive procedures should be encouraged and developed since the majority of pediatric histotypes are benign or low-grade cancers. Moreover, patients should be addressed to specialized pediatric centers in order to improve and refine the existing techniques, laying the groundwork for the proposal of new procedures.