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External Auditory Atresia
Joseph Junewick, MD FACR
over 7 years ago
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ARSt Case Repository

Orbital Langerhans Cell Histiocytosis

Case Detail

Anatomy: Neck-Face
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Neoplasia Malignant
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: MR CT
Activities:
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History

10 year old with persistent orbital swelling and erythema 2 months after minor trauma.


Case Images


Diagnosis

Orbital Langerhans Cell Histiocytosis

Findings

CT – Axial bone algorithm images demonstrate destructive process of lateral left orbital wall.

MR – Axial T1 and fat-suppressed T2 and postgadolinium T1 images show isointense T1 and hyperintense T2 and postgadolinium lesion with mass effect upon the lateral rectus muscle in leptomeningeal involvement in the middle cranial fossa.

Discussion

Langerhans cell histiocytosis is a rare disease, which is most commonly seen in children. Boys are more likely to be affected than girls. The spectrum of manifestations of the disease is broad, ranging from a single lesion to involvement of multiple organs. Bone lesions are the most common manifestation. When there is extraosseous involvement, affected organs may include the skin, central nervous system, liver, spleen, lungs, lymph nodes, bone marrow, soft tissues, and bowel. Some lesions may spontaneously regress and others may respond to chemotherapy, whereas the disease may be fatal in certain patients with multiple organ involvement. Orbital involvement in children has been well documented in the literature both as a solitary finding and as a part of multiple organ involvement. In patients with solitary lesions, findings typically include a lytic lesion centered in the orbital roof with a soft tissue mass extending into the orbit. These lesions respond very well to biopsy and curettage with or without intralesional injection of corticosteroids. Complete resolution is the result in most cases.

Reference

Schmidt S, Eich G, Geoffray A, Hanquinet S. Extraosseous Langerhans Cell Histiocytosis in Children. RadioGraphics (2008); 28:707–726.

Harris GJ. Langerhans cell histiocytosis of the orbit: A need for interdisciplinary dialogue. American journal of Ophthalmology (2006); 141(2): 374-378.

Contributor

Douglas Lukins, MD



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