title: Pediatric langerhans cell histiocytosis: single center experience over a 17-year period.
abstract: This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases. The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy. Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.
authors.affiliation: Department of Pediatric Oncology, Dokuz Eylül University Institute of Oncology, İzmir.
authors.forename: Dilek
authors.initials: D
authors.lastname: İnce
authors.affiliation: Department of Pediatric Hematology-Oncology Clinic, Dokuz Eylül University Institute of Oncology, İzmir.
authors.forename: Bengü
authors.initials: B
authors.lastname: Demirağ
authors.affiliation: Department of Pediatric Hematology-Oncology Clinic, Dokuz Eylül University Institute of Oncology, İzmir.
authors.forename: Gülcihan
authors.initials: G
authors.lastname: Özek
authors.affiliation: Division of Pediatric Oncology, Başkent University Faculty of Medicine, Adana, Turkey.
authors.forename: Ayşe
authors.initials: A
authors.lastname: Erbay
authors.affiliation: Department of Pathology, İzmir University Faculty of Medicine, İzmir.
authors.forename: Ragıp
authors.initials: R
authors.lastname: Ortaç
authors.affiliation: Department of Pediatric Hematology-Oncology Clinic, Dokuz Eylül University Institute of Oncology, İzmir.
authors.forename: Yeşim
authors.initials: Y
authors.lastname: Oymak
authors.affiliation: Department of Radiation Oncology, Ege University Faculty of Medicine, İzmir.
authors.forename: Serra
authors.initials: S
authors.lastname: Kamer
authors.affiliation: Department of Pediatric Hematology-Oncology Clinic, Dokuz Eylül University Institute of Oncology, İzmir.
authors.forename: Yöntem
authors.initials: Y
authors.lastname: Yaman
authors.affiliation: Dermatology Clinic, Dr Behçet Uz Children's Hospital, İzmir.
authors.forename: Selcen
authors.initials: S
authors.lastname: Kundak
authors.affiliation: Department of Pediatric Hematology-Oncology Clinic, Dokuz Eylül University Institute of Oncology, İzmir.
authors.forename: Canan
authors.initials: C
authors.lastname: Vergin
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D012008:Recurrence; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Langerhans cell histiocytosis; children; treatment