TY  - JOUR
A1  - Kriz, Jan
A1  - Eich, Hans Theodor
A1  - Bruns, Frank
A1  - Heyd, Reinhard
A1  - Schäfer, Ulrich
A1  - Haverkamp, Uwe
A1  - Büntzel, Jens
A1  - Seegenschmiedt, Heinrich
A1  - Micke, Oliver
T1  - Radiotherapy in langerhans cell histiocytosis : a rare indication in a rare disease
T2  - Radiation oncology
N2  - Introduction: Langerhans Cell Histiocytosis (LCH) represents a rare benign disorder, previously designated as "Histiocytosis X", "Type II Histiocytosis" or "Langerhans Cell Granulomatosis". Clinical presentation includes osteolysis, ulcerations of skin and soft tissues but also involvement of the CNS is described.Because treatment concepts are not well defined the German Cooperative Group on Radiotherapy for Benign Diseases performed a retrospective analysis.
Methods and material: Eight closely cooperating centres collected patients' data of the past 45 years. As study endpoints disease free survival, recurrent disease, death and therapy related side effects were defined.
Results: A total of 80 patients with histologically proven LCH were irradiated within the past 45 years. According to the LCH classification of Greenberger et al. 37 patients had stage Ia, 21 patients stage Ib, 13 patients stage II and 9 patients stage IIIb and the median age was 29 years. The median Follow up was 54 months (range 9-134 months). A total of 39 patients had a surgical intervention and 23 patients a chemotherapy regimen.Radiation treatment was carried out with a median total dose of 15 Gy (range 3-50.4 Gy). The median single fraction was 2 Gy (range 1.8-3 Gy).Overall, 77% patients achieved a complete remission and 12.5% achieved a partial remission. The long-term control rate reached 80%. Within an actuarial overall 5-year survival of 90% no radiogenic side and late effects ≥EORTC/RTOG II° were observed.
Conclusion: In the present study a large collective of irradiated patients was analysed. Radiotherapy (RT) is a very effective and safe treatment option and even low RT doses show sufficient local control.
KW  - Langerhans cell histiocytosis
KW  - Radiotherapy
KW  - Benign disorder
KW  - Effectivity
KW  - Rare disease
Y1  - 2013
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/32414
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-324141
SN  - 1748-717X
N1  - © 2013 Kriz et al.; licensee BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
VL  - 8
IS  - 233
PB  - BioMed Central
CY  - London
ER  -