Primary lung tumors in children: a national retrospective study on pulmonary outcome.

<bold>Background and aims:</bold> Primary lung tumors (PLTs) in children are rare, with little data from the literature. The objective is to describe their clinical and therapeutic characteristics, and the respiratory impact following treatment. <bold>Methods:</bold> From a national French data base and through a national census, we developed a national cohort of pediatric PLTs. We analyzed clinical, histological, management and post-operative data from 2013 to 2023 in France. <bold>Results:</bold> Sixty-two patients are included. The median age at diagnosis is 3.6 years with symptoms in 54 patients (dyspnea, cough, chest pain, fever) and eight incidentalomas. In three patients, the tumors were discovered in a context of refractory asthma. Sixty patients had surgical removal. The lobectomy was majority (n=32), then wedge (n=15), segmentectomy (n=5) and pneumectomy (n=5). Thoracoscopic approach was preferential in only 14% of the cohort. Pleuropneumoblastoma represents 50% of the cohort. Other histologies are neuroendocrine tumors, mucoepidermoid carcinomas, inflammatory myofibroblastic tumors and others. Complications (infectious, orthopedic, and pulmonary) occurred in 16% of cases at six months and six percent at the last follow up. At the last follow-up, only 43% of eligible patients had postoperative pulmonary function tests and four of them had a FEV1 < 80%. Seven children had respiratory symptoms and four died. <bold>Conclusions:</bold> Pleuropneumoblastoma is the most frequent PLT and lobectomy the preferential surgical treatment. The respiratory impact seems early but difficult to assess because not much performed in clinical routine. A joint surgeon/ pulmonologist follow-up post-surgery seems important.

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.oa4690
Primary Topic
Congenital Diaphragmatic Hernia Studies
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article

Primary lung tumors in children: a national retrospective study on pulmonary outcome.

Varlet Francois, Arnaud Bonnard, Olivier Abbo, Sabine Sarnacki et al.
Congenital Diaphragmatic Hernia Studies
article

Primary lung tumors in children: a national retrospective study on pulmonary outcome.

Varlet Francois, Arnaud Bonnard, Olivier Abbo, Sabine Sarnacki, Philippe Buisson, Julien Rod, Yves Réguerre, Nicolas Kalfa, Jérémie Rouger, Brice Fresneau, L Mansuy, Édouard Habonimana, Guillaume Podevin, Marie Auger Hunault, Julien Lejeune, Estelle Thébaud, Frédéric Hameury, ANAROSA SIMO, Aurore Haffreingue, Claire Briandet, Marc‐David Leclair, Coralie Mallebranche, Daniel H. Orbach, Fanny Delehaye, Jean‐Louis Lemelle, Thierry Merrot, Erik Hervieux, Hélène Sudour, R. Besson, Cécile Verite, Marion Gambart, Cédric Agossah, Nicolas André, Hubert Lardy, Emmanuel Sapin, Florent Guérin, Aude Marie‐Cardine, Jacques Brouard, C. Piolat, Frédéric Millot, Flavie Godard
article en

Abstract

<bold>Background and aims:</bold> Primary lung tumors (PLTs) in children are rare, with little data from the literature. The objective is to describe their clinical and therapeutic characteristics, and the respiratory impact following treatment. <bold>Methods:</bold> From a national French data base and through a national census, we developed a national cohort of pediatric PLTs. We analyzed clinical, histological, management and post-operative data from 2013 to 2023 in France. <bold>Results:</bold> Sixty-two patients are included. The median age at diagnosis is 3.6 years with symptoms in 54 patients (dyspnea, cough, chest pain, fever) and eight incidentalomas. In three patients, the tumors were discovered in a context of refractory asthma. Sixty patients had surgical removal. The lobectomy was majority (n=32), then wedge (n=15), segmentectomy (n=5) and pneumectomy (n=5). Thoracoscopic approach was preferential in only 14% of the cohort. Pleuropneumoblastoma represents 50% of the cohort. Other histologies are neuroendocrine tumors, mucoepidermoid carcinomas, inflammatory myofibroblastic tumors and others. Complications (infectious, orthopedic, and pulmonary) occurred in 16% of cases at six months and six percent at the last follow up. At the last follow-up, only 43% of eligible patients had postoperative pulmonary function tests and four of them had a FEV1 < 80%. Seven children had respiratory symptoms and four died. <bold>Conclusions:</bold> Pleuropneumoblastoma is the most frequent PLT and lobectomy the preferential surgical treatment. The respiratory impact seems early but difficult to assess because not much performed in clinical routine. A joint surgeon/ pulmonologist follow-up post-surgery seems important.

University of Reunion Island (RE), Université de Poitiers (FR), Lille’s Cardiology Hospital (FR), Centre Population et Développement (FR), Laboratoire d'Economie Appliquée de Grenoble (FR), Centre Hospitalier Universitaire de Montpellier (FR), Centre Hospitalier Universitaire Amiens-Picardie (FR), Laboratoire Population Environnement Développement (FR), Centre d’Investigation Clinique Innovation Technologique de Nancy (FR), Bordeaux Population Health (FR), Laboratoire de Physique Corpusculaire de Caen (FR), Centre de Recherche en Automatique de Nancy (FR), Centre Hospitalier Universitaire de Poitiers (FR), Centre Hospitalier Universitaire d'Angers (FR), Centre d’Etudes Politiques Et sociaLes (FR), Institut de génétique et de développement de Rennes (FR), Thion Medical (France) (FR), Centre Hospitalier Universitaire de Clermont-Ferrand (FR), Pharmacochimie et Biologie pour le Développement (FR), Maison des Sciences sociales et des Humanités de Dijon (FR), Laboratoire de Génie des Procédés – Environnement – Agro-alimentaire (FR), Centre Hospitalier Universitaire de Saint-Étienne (FR), Onco Lille (FR), Institut Génétique, Reproduction et Développement (FR), Université d'Angers (FR), École d'Ingénieurs en Chimie et Sciences du Numérique (FR), Université de Rouen Normandie (FR)
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Congenital Diaphragmatic Hernia Studies
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