Wilm tumor and Vasculitis
Heather Borders, MD
over 4 years ago
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Langerhans Cell Histiocytosis - Thymus
Joseph Junewick, MD FACR
|Diagnostic Category: Neoplasia Malignant
|Created: over 5 years ago
|Updated: over 5 years ago
8 month old male with skin rash.
Langerhans Cell Histiocytosis
CT – Anterior mediastinal mass with macrolobulated margins, microcystic areas of low attenuation and coarse calcifications.
Langerhans’ cell histiocytosis (LCH) is a disorder of immunoregulation. The Langerhans’ cell is a nonlymphoid antigen presenting cell involved in T-cell mediated immune responses. Overproliferation of the Langerhans’ cell results in disease. Involvement may be focal or systemic. Prognosis is related to the number of organ systems involved and inversely related to patient age at diagnosis. Thymus involvement by LCH is common, especially with multisystem disease, and manifest by gland enlargement, contour distortion, cystic degeneration, air cysts and calcification. The thymus normalizes with treatment.
Junewick JJ, Fitzgerald NE. The thymus in Langerhans’ cell histicytosis. Pediatr Radiol (1999); 29(12):904-907.