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Pediatr Blood Cancer. 2013 Nov;60(11):1759-65. doi: 10.1002/pbc.24603. Epub 2013 Jun 29.

Thymus and mediastinal node involvement in childhood Langerhans cell histiocytosis: long-term follow-up from the French national cohort.

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  • 1Service D'hémato Oncologie Pédiatrique, CHU de Strasbourg, Strasbourg, France; Service D'hémato Oncologie Pédiatrique, CHU de Bordeaux, Bordeaux, 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.

© 2013 The Authors. Pediatric Blood & Cancer Published by Wiley Periodicals, Inc.

KEYWORDS:

Langerhans cell histiocytosis; mediastinal nodes; survey; thymus

PMID:
23813854
[PubMed - indexed for MEDLINE]
PMCID:
PMC3824083
Free PMC Article
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