Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution.
OBJECTIVE
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 < or = 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.
Davidson
Laurence
L
Division of Neurosurgery, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, USA. ldavidso@usc.edu
McComb
J Gordon
JG
Bowen
Ira
I
Krieger
Mark D
MD
D000293:Adolescent; D000367:Age Factors; D001847:Bone Diseases / Q000188:drug therapy / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003919:Diabetes Insipidus / Q000150:complications; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy / Q000601:surgery*; D006801:Humans; D007027:Hypothalamic Diseases / Q000188:drug therapy / Q000601:surgery; D007030:Hypothalamo-Hypophyseal System / Q000187:drug effects / Q000601:surgery; D007223:Infant; D008297:Male; D012075:Remission, Spontaneous; D012189:Retrospective Studies; D012886:Skull / Q000187:drug effects / Q000601:surgery*; D013122:Spinal Diseases / Q000188:drug therapy / Q000601:surgery*; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome