TY  - JOUR
AB  - 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.
AU  - Kriz, Jan
AU  - Eich, Hans-Theodor
AU  - Eich, Hans Theodor
AU  - Eich, Hans Theodor
AU  - Eich, Hans Theodor
AU  - Bruns, Frank
AU  - Heyd, Reinhard
AU  - Heyd, R.
AU  - Schäfer, Ulrich
AU  - Haverkamp, Uwe
AU  - Haverkamp, U.
AU  - Büntzel, Jens
AU  - Seegenschmiedt, Michael H.
AU  - Seegenschmiedt, Michael Heinrich
AU  - Seegenschmiedt, Michael-Heinrich
AU  - Seegenschmiedt, M. Heinrich
AU  - Seegenschmiedt, M. H.
AU  - Micke, Oliver
DA  - 2013-10-09
DO  - 10.1186/1748-717X-8-233
KW  - Langerhans cell histiocytosis
KW  - Radiotherapy
KW  - Benign disorder
KW  - Effectivity
KW  - Rare disease
LA  - eng
N1  - Finanziert durch den Open-Access-Publikationsfonds 2013/2014 der Deutschen Forschungsgemeinschaft (DFG) und der Westfälischen Wilhelms-Universität Münster (WWU Münster).
N1  - Radiation Oncology 8  (2013) 233
PY  - 2013-10-09
TI  - Radiotherapy in langerhans cell histiocytosis - a rare indication in a rare disease
UR  - https://nbn-resolving.org/urn:nbn:de:hbz:6-84309663089
Y2  - 2024-12-26T19:29:54
ER  -