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WHO Classification of Tumours
Langerhans cell histiocytosis
Nasal cavity and Sinuses


Definition

Langerhans cell histiocytosis (LCH) is a rare clonal neoplastic proliferation of Langerhans-type cells that express CD1a, langerin and S100 protein, and shows Birbeck granules by ultrastructural examination
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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (Eds.)
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues
4th Edition
International Agency for Research on Cancer: Lyon 2008



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Children are most frequently affected. There is a marked predilection for males (M:F ratio 3.7:1) and whites of northern European descent while the disease is uncommon in blacks. There is a high concordance rate for identical twins. The clinical course is generally related to the number of organs affected at presentation.
Langerhans cell histiocytosis of the lung is almost always a disease of smokers, usually non-clonal and thought to represent a reactive process {502, 2479}.

The following subtypes are distinguished:

Histiocytosis X, eosinophilic granuloma (solitary lesion)

Hand-Schüller-Christian disease (multiple lesions)

Letterer-Siwe disease (if disseminated or visceral involvement).