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Joubert Syndrome
Joseph Junewick, MD FACR
over 7 years ago
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Case History


8 month old male with skin rash.


Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: CT
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Case Images


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Reveal Findings

CT – Anterior mediastinal mass with macrolobulated margins, microcystic areas of low attenuation and coarse calcifications.

Reveal Discussion

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.

Reveal Diagnosis

Langerhans Cell Histiocytosis

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