Thymus and mediastinal node involvement in childhood langerhans cell histiocytosis: Long‐term follow‐up from the French national cohort |
| |
| Authors: | Stephane Ducassou MD Fanny Seyrig MD Caroline Thomas MD Anne Lambilliotte MD Perrine Marec‐Berard MD Claire Berger MD Genevieve Plat MD Laurence Brugiere MD Marie Ouache MD Mohamed Barkaoui MSc Corinne Armari‐Alla MD Patrick Lutz MD PhD Guy Leverger MD Xavier Rialland MD Ludovic Mansuy MD Helene Pacquement MD Eric Jeziorski MD PhD Virginie Gandemer MD PhD François Chalard MD Jean François Chateil MD PhD Abdellatif Tazi MD PhD Jean François Emile MD PhD Jean Donadieu MD PhD the Investigators of the French LCH Study Group |
| |
| Affiliation: | 1. Service D'hémato Oncologie Pédiatrique, CHU de Strasbourg, , Strasbourg, France;2. Service D'hémato Oncologie Pédiatrique, CHU de Bordeaux, , Bordeaux, France;3. Service D'hémato Oncologie Pédiatrique, CHU de Nantes, , Nantes, France;4. Service D'hématologie Oncologie Pédiatrique, H?pital Jeanne de Flandres, , Lille, France;5. Institut D'hémato Oncologie Pédiatrique, Hospice civil de Lyon, , Lyon, France;6. Service D'hémato Oncologie Pédiatrique, CHU de Saint Etienne, , France;7. Service D'hémato Oncologie Pédiatrique Hopital Purpan, CHU de Toulouse, , France;8. Service D'oncologie Pédiatrique, Institut Gustave Roussy, , Villejuif, France;9. Service D'hématologie Pédiatrique, Hopital Robert Débré, , Paris, France;10. Service D'hémato Oncologie Pédiatrique, Centre de référence des histiocytoses, registre des histiocytoses, APHP H?pital A. Trousseau, , Paris, France;11. Service de Pédiatrie, Unité D'hémato Oncologie Pédiatrique, CHU Michallon, , Grenoble, France;12. Service de Pédiatrie, Unité d'hémato Oncologie Pédiatrique, CHU d'Angers, , Angers, France;13. Service de Médecine Infantile II, CHU de Brabois, Nancy, , France;14. Service de Pédiatrie, Institut Curie, , Paris, France;15. Service de Médecine Infantile, Hopital Arnaud de Villeneuve, CHU de Montpellier, , Montpellier, France;16. Service D'hémato Oncologie Pédiatrique, Hopital Sud CHU, , Rennes, France;17. Service de Radiologie APHP, Hopital Trousseau, , Paris, France;18. Service Radiologie Pédiatrique, CHU Bordeaux, , France;19. Service de Pneumologie, Centre de référence des histiocytoses, APHP H?pital Saint Louis, , Paris, France;20. Laboratoire D'anatomie et Cytologie Pathologique, APHP Hopital A Paré, , Boulognes, France |
| |
| Abstract: | Background Mediastinal involvement (MI) in Langerhans cell histiocytosis (LCH) has been rarely reported. Here, we describe the clinical, radiological, and biological presentation, and the outcome of childhood LCH with MI. Method From the French LCH register, which includes 1,423 patients aged less than 18 years, we retrieved the medical charts of patients with mediastinal enlargement detected on chest X‐rays. Results Thirty‐seven patients were retrieved, including 18 males; median age of diagnosis was 0.7 years, and median follow‐up time was 6.2 years. The prevalence of MI varied with the age at diagnosis, ranging from 7% below 1 year old to less than 1% at >5 years. Thirteen cases (35%) were diagnosed because of MI‐related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10). CT scans performed in 32 cases at diagnosis showed tracheal compression (N = 5), cava thrombosis (N = 2), and/or calcification (N = 16). All patients presented multi‐system disease at LCH diagnosis, and 35/37 were initially treated with vinblastine and corticosteroids. Death occurred in five cases, due to MI (N = 1) or hematological refractory involvement (N = 4). The overall 5‐year survival was 87.1%, and immunodeficiency was not detected as a sequel. Conclusions MI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications. Pediatr Blood Cancer 2013;60:1759–1765. © 2013 Wiley Periodicals, Inc. |
| |
| Keywords: | Langerhans cell histiocytosis mediastinal nodes survey thymus |
|
|
