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Craniospinal Langerhans cell histiocytosis in children: 30 years' experience at a single institution
Laurence Davidson1, J Gordon McComb, Ira Bowen
1Division of Neurosurgery, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, USA. ldavidso@usc.edu
Insights
Langerhans cell histiocytosis (LCH) craniospinal lesions in children often require chemotherapy, especially in younger patients. Surgery may suffice for unifocal LCH, and some lesions resolve spontaneously.
Area of Science:
- Pediatric Neurosurgery
- Pediatric Oncology
- Histiocytosis
Background:
- Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the abnormal proliferation of Langerhans cells.
- Craniospinal lesions represent a significant manifestation of LCH, impacting disease course and treatment strategies.
Purpose of the Study:
- To evaluate the long-term outcomes and treatment efficacy for pediatric patients with craniospinal Langerhans cell histiocytosis.
- To identify risk factors associated with disease progression and dissemination in LCH.
Main Methods:
- Retrospective review of 44 pediatric patients diagnosed with LCH and craniospinal lesions between 1976 and 2006.
- Analysis of patient demographics, lesion characteristics, treatment modalities, and follow-up data.
Main Results:
- Of 44 patients, 61% presented with unifocal bone lesions and 27% with multifocal bone disease. Younger patients (≤2 years) were at higher risk for multifocal disease and dissemination.
- Chemotherapy was effective in controlling multifocal or disseminated LCH, while surgery alone was sufficient for some unifocal cases. Spontaneous resolution occurred in 3 patients.
Conclusions:
- Unifocal LCH in children can be managed effectively with surgery alone.
- Younger patients with LCH require chemotherapy due to increased risk of multifocal disease and dissemination.
- Biopsy is recommended for diagnostic purposes, but resection of spontaneously resolving lesions is unnecessary.
Objectives:
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.