A case of Erdheim-Chester disease with spinal cord compression and sphenoid sinus involvement

K. Bunaux , Henri Sevestre (1) , J. -F. Emile , C. Capel , L. Chenin , J. Peltier

1 LPCM - Laboratoire de Physiologie Cellulaire et Moléculaire - UR UPJV 4667

Erdheim-Chester disease is a rare form of non-Langerhans cell histiocytosis. It is an inflammatory disorder associated with BRAF V600E mutation in 50% of cases. This multisystem disease is rarely associated with spinal involvement. Neurological involvement is an independent predictive factor of poor prognosis. The diagnosis is histopathological based on CD68-positive and CD1A-negative histiocytes. Treatment with interferon-alpha is an independent predictor of survival in Erdheim-Chester disease and vemurafenib has also been shown to be effective for BRAF V600E mutation. We report a clinical case of a 51-year-old patient with multiple and rare locations of Erdheim-Chester disease, particularly at the sphenoid sinus. (C) 2018 Elsevier Masson SAS. All rights reserved.

Domaines: Sciences du Vivant [q-bio]

K. Bunaux, Henri Sevestre, J. -F. Emile, C. Capel, L. Chenin, et al.. A case of Erdheim-Chester disease with spinal cord compression and sphenoid sinus involvement. Neurochirurgie, 2018, 64 (6), pp.439-441. ⟨10.1016/j.neuchi.2018.08.002⟩. ⟨hal-03607761⟩