TY - JOUR
T1 - Adult langerhans cell histiocytosis of bones
T2 - A rare cancer network study
AU - Atalar, Banu
AU - Miller, Robert C.
AU - Dincbas, Fazilet Oner
AU - Geismar, Jan Henning
AU - Micke, Oliver
AU - Akyurek, Serap
AU - Ozyar, Enis
PY - 2010/10
Y1 - 2010/10
N2 - Langerhans Cell Histiocytosis (LCH) is an uncommon benign bone tumour typically seen in children. LCH of bones in adults has been reported as solitary cases. The aim of the current study is to analyze different treatment approaches and the role of radiotherapy (RT) in adult LCH. Thirty patients from five Rare Network Cancer centers were included in this retrospective study. Median age was 30 years. The localization of tumours was skull bones in 12 (40%), lower extremity in 6 (20%), thoracic bones in 4 (133%), spine in 3 (10%), pelvis in 2 (6.7%) and multiple sites in 3 (10%) patients. Primary treatment was surgery in 1 (33%), surgery+ radiotherapy (RT) in 15 (50%), RT in 12 (40%), RT + CHT in 1 (33%) and corticosteroids in 1 (33%) patient. Median follow-up was 58 months. Complete remission was obtained in 21 (70%), partial remission in 4 (133%) ; 2 lesions were stable (6.7%) and progression was noted in 2 (6.7%) of the patients. Nine patients (30%) had recurrent disease. Recurrence rates were significantly lower in patients who were treated with surgery and RT (p < 0.003). Surgery plays a major role in the treatment of adult LCH of bones ; radiotherapy should be considered in the adjuvant setting and palliation.
AB - Langerhans Cell Histiocytosis (LCH) is an uncommon benign bone tumour typically seen in children. LCH of bones in adults has been reported as solitary cases. The aim of the current study is to analyze different treatment approaches and the role of radiotherapy (RT) in adult LCH. Thirty patients from five Rare Network Cancer centers were included in this retrospective study. Median age was 30 years. The localization of tumours was skull bones in 12 (40%), lower extremity in 6 (20%), thoracic bones in 4 (133%), spine in 3 (10%), pelvis in 2 (6.7%) and multiple sites in 3 (10%) patients. Primary treatment was surgery in 1 (33%), surgery+ radiotherapy (RT) in 15 (50%), RT in 12 (40%), RT + CHT in 1 (33%) and corticosteroids in 1 (33%) patient. Median follow-up was 58 months. Complete remission was obtained in 21 (70%), partial remission in 4 (133%) ; 2 lesions were stable (6.7%) and progression was noted in 2 (6.7%) of the patients. Nine patients (30%) had recurrent disease. Recurrence rates were significantly lower in patients who were treated with surgery and RT (p < 0.003). Surgery plays a major role in the treatment of adult LCH of bones ; radiotherapy should be considered in the adjuvant setting and palliation.
KW - Bone
KW - Langerhans Cell Histiocytosis
UR - http://www.scopus.com/inward/record.url?scp=78549269747&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=78549269747&partnerID=8YFLogxK
M3 - Article
C2 - 21138223
AN - SCOPUS:78549269747
SN - 0001-6462
VL - 76
SP - 663
EP - 668
JO - Acta Orthopaedica Belgica
JF - Acta Orthopaedica Belgica
IS - 5
ER -