Langerhans Cell Histiocytosis (LCH) Ophthalmic Invasion, Ophthalmic Invasion, Orbital Tumor

 

 

Langerhans Cell Histiocytosis (LCH) is a rare, systemic disorder characterized by clonal proliferation of abnormal Langerhans cells, affecting bone, skin, lymph nodes, and internal organs.
Orbital involvement is found in about 20–23% of LCH cases, with proptosis and superolateral bone erosion being classic findings.

🔹 Classification & History

• Previously grouped as Histiocytosis X (Eosinophilic Granuloma, Hand-Schüller-Christian, Letterer-Siwe)
• Now considered a spectrum of a single disease based on shared pathology
• Different clinical severity and prognosis depending on extent

🔹 Diagnosis

• Histology: Grooved nucleus, eosinophils, plasma cells
• EM: Birbeck granules = “tennis racket” shaped organelles
• IHC: CD1a and S100 positivity
• Imaging: CT/MRI reveals bone destruction, soft tissue mass

🔹 Clinical Manifestations & Management

• Localized lesion: biopsy, steroid injection
• Multifocal/multisystem: radiation + chemotherapy (prednisone + vinblastine)
• Most commonly affects frontal bone, causing superolateral orbital mass and displacement

🔹 Prognosis

• Worse with younger age (<2y) and multisystem involvement
• Mortality: ~55–60% under 2 years; ~15% over 3 years
• Diabetes insipidus indicates risk of chronic disease/recurrence

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📋 LCH Subtypes Summary Table (English)

SubtypeKey FeaturesPrognosis

Eosinophilic Granuloma	Localized to bone, 4–7 y/o, uni/multifocal lesions	Favorable
Hand-Schüller-Christian	Bone + soft tissue, proptosis + skull lesions + DI	Intermediate
Letterer-Siwe	Acute, systemic, multiorgan (liver, spleen, marrow)	Poor
Orbital LCH	Superolateral bone erosion on imaging	Depends on dissemination
Diagnostic Markers	CD1a(+), S100(+), Birbeck granules	Mandatory for diagnosis