Luca Pio1, Patricio Varela2, Martin J Eliott3, Vincent Couloigner4, Gabriela Guillén Burrieza5, Irene Paraboschi6, Calogero Virgone7, Rebecca Maunsell8, Victor Rachkov9, Michael J Rutter10, Mariano Boglione11, Jaime Penchyna Grub12, Gastón Bellía Munzón13, Sabine Sarnacki1, Sabine Irtan14, Claudia Schweiger10, Michèle Larroquet14, Naziha Khen Dunlop1, Madhavan Ramaswamy3, Angela Pistorio15, Giovanni Cecchetto7, Andrea Ferrari16, Gianni Bisogno17, Michele Torre18. 1. Department of Pediatric Surgery, Necker Enfants-Malades Hospital-Université Paris Descartes, Sorbonne Paris Cité, Paris, France. 2. Department of Pediatric Surgery, Clinica Las Condes Medical Center, Hospital de Niños Calvo Mackenna, University of Chile, Santiago, Chile. 3. Tracheal Team, Great Ormond Street Children's Hospital, London, United Kingdom. 4. Pediatric Ear, Nose, and Throat Department, Necker Hospital for Sick Children, Public Assistance-Hospitals of Paris, Paris, France. 5. Pediatric Surgery Department, Hospital Vall d'Hebron, Barcelona, Spain. 6. DINOGMI University of Genoa, Genoa, Italy. 7. Pediatric Surgery, Department of Women's and Children's Health, University of Padua, Padua, Italy. 8. Department of Otorhinolaryngology, Head and Neck Surgery, University of Campinas, Campinas, São Paulo, Brazil. 9. Chair of Pediatric Surgery of N.I. Pirogov Russian Research Medical University, Dmitry Rogachev Federal Research Center of Pediatric Hematology, Oncology and Immunology; ZAO European Medical Center, Moscow, Russia. 10. Division of Pediatric Otolaryngology-Head and Neck Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, U.S.A. 11. Department of General Surgery, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Pichincha, 1850, Ciudad De Buenos Aires. 12. Department of Thoracic Surgery and Endoscopy, Hospital Infantil de México, Federico Gómez, Mexico. 13. Department of Surgery, Fundación Hospitalaria, Private Children's Hospital, Ciudad Autónoma de Buenos Aires, Buenos Aires, Argentina. 14. Department of Surgery, Hôpital Trousseau-Hôpitaux Universitaires de l'Est Parisien (AP-HP), Université Pierre et Marie Curie, Paris, France. 15. Epidemiology, and Biostatistics Unit, Genoa, Italy. 16. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy. 17. Pediatric Hematology and Oncology Division, University of Padua, Padua, Italy. 18. Airway Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
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.
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.