Synchronous Pulmonary Langerhans Cell Histiocytosis and Multiple Cutaneous Reticulohistiocytomas With a Common BRAF-V600E/D Mutation Driver

Histiocytoses constitute a group of heterogeneous disorders characterized by involvement of variable organs by neoplastic macrophage or dendritic cells. They may affect both adults and children with a predilection to the skin, bone, lungs, lymph nodes, and CNS. The coexistence of different types of histiocytoses in the same patient is an extremely rare phenomenon. We describe a very rare case of co-occurring pulmonary Langerhans cell histiocytosis with multiple cutaneous reticulohistiocytomas with a common BRAF- V600E mutation as the driver genetic event in both the lung and skin lesions. The presence of a common BRAF- V600E mutation provides evidence of their clonal relation and contributes to our understanding in the pathogenesis of multiple, co-occurring histiocytic proliferations.

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Publication Details

Journal
American Journal of Dermatopathology
Published
2024-11-05
DOI
https://doi.org/10.1097/dad.0000000000002871
Primary Topic
Histiocytic Disorders and Treatments
Type
article
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article

Synchronous Pulmonary Langerhans Cell Histiocytosis and Multiple Cutaneous Reticulohistiocytomas With a Common BRAF-V600E/D Mutation Driver

Ourania Parra, Jessica G. Wilson, Natalia Georgantzoglou, Donald P. Green et al.
American Journal of Dermatopathology
Histiocytic Disorders and Treatments
article

Synchronous Pulmonary Langerhans Cell Histiocytosis and Multiple Cutaneous Reticulohistiocytomas With a Common BRAF-V600E/D Mutation Driver

Ourania Parra, Jessica G. Wilson, Natalia Georgantzoglou, Donald P. Green, Konstantinos Linos
article en

Abstract

Histiocytoses constitute a group of heterogeneous disorders characterized by involvement of variable organs by neoplastic macrophage or dendritic cells. They may affect both adults and children with a predilection to the skin, bone, lungs, lymph nodes, and CNS. The coexistence of different types of histiocytoses in the same patient is an extremely rare phenomenon. We describe a very rare case of co-occurring pulmonary Langerhans cell histiocytosis with multiple cutaneous reticulohistiocytomas with a common BRAF- V600E mutation as the driver genetic event in both the lung and skin lesions. The presence of a common BRAF- V600E mutation provides evidence of their clonal relation and contributes to our understanding in the pathogenesis of multiple, co-occurring histiocytic proliferations.

American Journal of DermatopathologyVol. 47(1)
Dartmouth–Hitchcock Medical Center (US), Memorial Sloan Kettering Cancer Center (US)
Good health and well-being
Openalex Percentile: Top 73%
Histiocytic Disorders and Treatments
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