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Thymus and mediastinal node involvement in childhood langerhans cell histiocytosis: Long‐term follow‐up from the French national cohort
Author(s) -
Ducassou Stephane,
Seyrig Fanny,
Thomas Caroline,
Lambilliotte Anne,
MarecBerard Perrine,
Berger Claire,
Plat Genevieve,
Brugiere Laurence,
Ouache Marie,
Barkaoui Mohamed,
ArmariAlla Corinne,
Lutz Patrick,
Leverger Guy,
Rialland Xavier,
Mansuy Ludovic,
Pacquement Helene,
Jeziorski Eric,
Gandemer Virginie,
Chalard François,
Chateil Jean François,
Tazi Abdellatif,
Emile Jean François,
Donadieu Jean
Publication year - 2013
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.24603
Subject(s) - medicine , langerhans cell histiocytosis , pediatrics , histiocytosis , respiratory distress , cohort , b symptoms , radiology , surgery , lymphoma , disease
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.