title: Does adjunctive chemotherapy reduce remission rates compared to cortisone alone in unifocal or multifocal histiocytosis of bone?
abstract: BACKGROUND
  Langerhans cell histiocytosis (LCH) is a rare disorder that can affect almost any organ, including bone. Treatment options include local corticosteroid infiltration in isolated bone lesions and oral corticosteroids and chemotherapy in multifocal bone lesions. Several studies show local corticosteroid injection in unifocal bone lesions heal in more than 75% of patients with minimal side effects. Therefore, it is unclear whether chemotherapy adds materially to the healing rate.
  
  
  OBJECTIVE
  We therefore compared overall survival, remission rate, and recurrence rate in patients with bone LCH treated with chemotherapy and corticosteroids or corticosteroids alone.
  
  
  METHODS
  We retrospectively reviewed the records of 198 patients with LCH since 1950. Median age at diagnosis was 5 years, male-to-female ratio was 1.33, and the most frequent symptom was local pain (95%). We recorded the disease presentation, demographics, treatment, and clinical evolution of each patient. Minimum followup was 4 months (median, 24 months; range, 4-360 months).
  
  
  RESULTS
  The survival rate of the systemic disease group was 76.5% (65 of 85) while the survival rate in the unifocal and multifocal bone involvement groups was 100% at a median 5-year followup. All patients with unifocal bone involvement and 40 of 43 (93%) with multifocal bone involvement had complete remission. One of 30 patients with multifocal bone involvement treated with chemotherapy and oral corticosteroids did not achieve remission whereas two of six receiving only corticosteroids did not achieve remission.
  
  
  CONCLUSIONS
  Our observations suggest intralesional corticosteroid injection without adjunctive chemotherapy achieves remission in unifocal bone LCH but may not do so in multifocal single-system bone involvement. Larger series would be required to confirm this observation.
  
  
  METHODS
  Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.
authors: Department of Orthopaedics, University of São Paulo, Rua Barata Ribeiro, 490 Conjunto 33, Bela Vista, São Paulo 01308-000, Brazil. andre.baptista@uol.com.br
authors: André Mathias
authors: AM
authors: Baptista
authors: André Ferrari França
authors: AF
authors: Camargo
authors: Olavo Pires
authors: OP
authors: de Camargo
authors: Vicente
authors: V
authors: Odone Filho
authors: Alejandro Enzo
authors: AE
authors: Cassone
mesh_terms: D000293:Adolescent; D000328:Adult; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D001847:Bone Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D003348:Cortisone / Q000008:administration & dosage; D003430:Cross-Sectional Studies; D005260:Female; D006615:Hip / Q000000981:diagnostic imaging; D006646:Histiocytosis, Langerhans-Cell / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000401:mortality; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011859:Radiography; D012074:Remission Induction; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D003348:Cortisone