NCI Definition: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001) [1]

Langerhans cell histiocytosiss most frequently harbor alterations in BRAF [2].

Most Commonly Altered Genes in Langerhans Cell Histiocytosis

BRAF Mutation, BRAF V600E, BRAF Exon 15 Mutation, and BRAF Codon 600 Missense are the most common alterations in langerhans cell histiocytosis [2].

Top Alterations in Langerhans Cell Histiocytosis

Significant Genes in Langerhans Cell Histiocytosis


FLT3 +

Disease Details

Histiocytosis X, Langerhans Cell Granulomatosis, Soft tissue histiocytosis Langerhans, LCH
Histiocytic and Dendritic Cell Neoplasm
Letterer-Siwe Disease, Hand-Schuller-Christian Disease, and Langerhans Cell Histiocytosis, Monostotic
OncoTree Name
Langerhans Cell Histiocytosis
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.