Role of Neurosurgery in the Treatment of Langerhans Cell Histiocytosis

Abstract

  Langerhans cell histiocytosis (LCH) is a relatively rare disease that can result in late complications in the central nervous system (CNS). Recent advancement in clinical findings and chemotherapies has changed the treatment algorithm for LCH.
  We retrospectively reviewed the clinical course and outcome of patients with LCH treated at the National Center for Child Health and Development from March 2005 to September 2010. There were 14 patients (8 boys, 6 girls ; median age : 5 years 4 months, range : 6 months to 14 years 2 months) who underwent neurosurgical treatments. Subclassification of the LCH type was single-system single-site (SS-s : n=8), single-system multisite (SS-m : n=2), and multisystem (MS : n=4).
  The most common initial symptom was a painful, slow-growing skull mass. Preceding head trauma at the site of LCH was reported in 3 patients. Except for the first patient who underwent total resection, the remaining 13 patients received chemotherapy. Complete remission was obtained in 11 patients while reactivation developed in 2 patients during the follow-up period (mean duration : 48 months).
  Since LCH can develop in multiple systems and late reactivation is common, systemic examination and treatment seems indispensable. The prevention of late CNS complications is also important for long-term prognosis. Our limited experience manifested the possible risks of surgical treatment alone. Nearly half of the patients had involvement in multiple regions and organs, and despite chemotherapy, LCH was reactivated in 2 of them. Long-term follow-up in collaboration with pediatric oncologists is strongly recommended.