title: [Erdheim-Chester disease, an incredible simulator. Cases reports and review of literature].
abstract: Erdheim-Chester disease is a non-Langerhans histiocytosis. Until 2014 at least 550 cases have been reported. According to European Rare Disease Organization and National Organization for Rare Disorders it is a rare disease. The most common symptom is bone pain in the lower extremities and it usually appears between the 5th and 7th decades of life. The diagnostic is based on immunohistochemical results: S100(+/-), CD68(+), and CD1a(-), the latter 2 are mandatory. The best treatment nowadays is alpha-interferon or pegylated alpha-2. The overall survival is 96% at one year and 68% at 5 years. Central nervous system involvement is associated with a worse outcome. Two cases are presentedwith central nervous system lesions in the absence of lesions in other organs on their onset. Very few cases have been reported with this kind of presentation. We also noted that these patients had recurrences or new lesions at 8 months. A follow-up is proposed with brain MRI and thoraco-abdominal PET every 3-4 months.
authors.affiliation: Servicio de Neurocirugía, Instituto Neurociencias, Hospital Clínico San Carlos, Madrid, España. Electronic address: ferrascon@hotmail.com.
authors.forename: Fernando J
authors.initials: FJ
authors.lastname: Rascón-Ramírez
authors.affiliation: Servicio de Neurocirugía, Instituto Neurociencias, Hospital Clínico San Carlos, Madrid, España.
authors.forename: Josué M
authors.initials: JM
authors.lastname: Avecillas-Chasín
authors.affiliation: Servicio de Neurocirugía, Instituto Neurociencias, Hospital Clínico San Carlos, Madrid, España.
authors.forename: Gregorio
authors.initials: G
authors.lastname: Rodríguez-Boto
authors.affiliation: Servicio de Neuropatología, Instituto Neurociencias, Hospital Clínico San Carlos, Madrid, España.
authors.forename: Issa
authors.initials: I
authors.lastname: Subhi-Issa
authors.affiliation: Servicio de Neurocirugía, Instituto Neurociencias, Hospital Clínico San Carlos, Madrid, España.
authors.forename: Osman A
authors.initials: OA
authors.lastname: Salazar A
authors.affiliation: Servicio de Neurocirugía, Instituto Neurociencias, Hospital Clínico San Carlos, Madrid, España.
authors.forename: Kita
authors.initials: K
authors.lastname: Sallabanda D
mesh_terms: D002675:Child, Preschool; D031249:Erdheim-Chester Disease / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D010146:Pain / Q000209:etiology
keywords: Brain diseases; Brain metastasis; Enfermedad de Erdheim-Chester; Enfermedades cerebrales; Enfermedades linfáticas; Erdheim-Chester disease; Lymphatic diseases; Metástasis cerebral; Regresión neoplásica espontánea; Spontaneous neoplasm regression