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Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution
- Source :
- Journal of Neurosurgery: Pediatrics. 1:187-195
- Publication Year :
- 2008
- Publisher :
- Journal of Neurosurgery Publishing Group (JNSPG), 2008.
-
Abstract
- Object The goal of this study was to review a large series of patients with Langerhans cell histiocytosis (LCH) who had craniospinal lesions to assess the long-term course, outcome, and efficacy of treatment of the disease. Methods Forty-four patients with LCH who presented to a single pediatric neurosurgical department between 1976 and 2006 were retrospectively reviewed. Results This series included 29 boys and 15 girls, ranging in age from 2 months to 13 years, with a mean follow-up duration of 4.5 years. Twenty-seven patients (61%) had unifocal bone lesions, 12 (27%) had multifocal bone disease, 2 (5%) had solitary hypothalamic–pituitary axis lesions, and 3 (7%) had multiple organ involvement at presentation. Five (19%) of the 27 patients with unifocal bone disease and 4 (33%) of the 12 patients with multifocal bone disease had delayed development of new bone lesions during the follow-up period. The time to development of new bone lesions ranged from 1 month to 1 year. Two of the 3 patients with multiple-organ LCH died. Patient age ≤ 2 years at the time of initial presentation was a risk factor for both initial multifocality and eventual dissemination. In all patients with initial multifocal bone involvement or later dissemination of unifocal bone disease, LCH was controlled by chemotherapy, except for 2 who were treated by surgery alone. Three patients had histological evidence of spontaneous resolution of their lesions. Conclusions Patients with unifocal LCH can be effectively treated with surgery alone. Very young patients are more likely to have multifocal disease and disseminations, and will usually require chemotherapy to control their disease. Spontaneously regressing lesions need not be resected; however, a biopsy procedure can be performed for diagnostic purposes.
- Subjects :
- Male
Hypothalamo-Hypophyseal System
medicine.medical_specialty
Time Factors
Adolescent
Bone disease
Remission, Spontaneous
Disease
Letterer–Siwe disease
Langerhans cell histiocytosis
Eosinophilic granuloma
Humans
Medicine
Hand–Schüller–Christian disease
Child
Retrospective Studies
business.industry
Skull
Age Factors
Infant
General Medicine
medicine.disease
Surgery
Survival Rate
Histiocytosis, Langerhans-Cell
Treatment Outcome
El Niño
Child, Preschool
Disease Progression
Female
Spinal Diseases
Radiology
Bone Diseases
business
Craniospinal
Diabetes Insipidus
Hypothalamic Diseases
Follow-Up Studies
Subjects
Details
- ISSN :
- 19330715 and 19330707
- Volume :
- 1
- Database :
- OpenAIRE
- Journal :
- Journal of Neurosurgery: Pediatrics
- Accession number :
- edsair.doi.dedup.....ca240a30bb2d21b318bbc53788e4734a