TY  - JOUR
A1  - Dietl, Sebastian
A1  - Schwinn, Stefanie
A1  - Dietl, Susanne
A1  - Riedl, Simone
A1  - Deinlein, Frank
A1  - Rutkowski, Stefan
A1  - von Bueren, Andre O.
A1  - Krauss, Jürgen
A1  - Schweitzer, Tilmann
A1  - Vince, Giles H.
A1  - Picard, Daniel
A1  - Eyrich, Matthias
A1  - Rosenwald, Andreas
A1  - Ramaswamy, Vijay
A1  - Taylor, Michael D.
A1  - Remke, Marc
A1  - Monoranu, Camelia M.
A1  - Beilhack, Andreas
A1  - Schlegel, Paul G.
A1  - Wölfl, Matthias
T1  - MB3W1 is an orthotopic xenograft model for anaplastic medulloblastoma displaying cancer stem cell- and Group 3-properties
JF  - BMC Cancer
N2  - Background
Medulloblastoma is the most common malignant brain tumor in children and can be divided in different molecular subgroups. Patients whose tumor is classified as a Group 3 tumor have a dismal prognosis. However only very few tumor models are available for this subgroup.

Methods
We established a robust orthotopic xenograft model with a cell line derived from the malignant pleural effusions of a child suffering from a Group 3 medulloblastoma.

Results
Besides classical characteristics of this tumor subgroup, the cells display cancer stem cell characteristics including neurosphere formation, multilineage differentiation, CD133/CD15 expression, high ALDH-activity and high tumorigenicity in immunocompromised mice with xenografts exactly recapitulating the original tumor architecture.

Conclusions
This model using unmanipulated, human medulloblastoma cells will enable translational research, specifically focused on Group 3 medulloblastoma.
KW  - cancer stem cells
KW  - anaplastic medulloblastoma
KW  - group 3
KW  - orthotopic xenograft
KW  - animal model
KW  - brain tumor
KW  - children
Y1  - 2016
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-145877
VL  - 16
IS  - 115
ER  - 
TY  - JOUR
A1  - Sabel, Magnus
A1  - Fleischhack, Gudrun
A1  - Tippelt, Stephan
A1  - Gustafsson, Göran
A1  - Doz, François
A1  - Kortmann, Rolf
A1  - Massimino, Maura
A1  - Navajas, Aurora
A1  - von Hoff, Katja
A1  - Rutkowski, Stefan
A1  - Warmuth-Metz, Monika
A1  - Clifford, Steven C.
A1  - Pietsch, Torsten
A1  - Pizer, Barry
A1  - Linnering, Birgitta
T1  - Relapse patterns and outcome after relapse in standard risk medulloblastoma: a report from the HIT-SIOP-PNET4 study
JF  - Journal of Neurooncology
N2  - The HIT-SIOP-PNET4 randomised trial for standard risk medulloblastoma (MB) (2001-2006) included 338 patients and compared hyperfractionated and conventional radiotherapy. We here report the long-term outcome after a median follow up of 7.8 years, including detailed information on relapse and the treatment of relapse. Data were extracted from the HIT Group Relapsed MB database and by way of a specific case report form. The event-free and overall (OS) survival at 10 years were 76 +/- 2 % and 78 +/- 2 % respectively with no significant difference between the treatment arms. Seventy-two relapses and three second malignant neoplasms were reported. Thirteen relapses (18 %) were isolated local relapses in the posterior fossa (PF) and 59 (82 %) were craniospinal, metastatic relapses (isolated or multiple) with or without concurrent PF disease. Isolated PF relapse vs all other relapses occurred at mean/median of 38/35 and 28/26 months respectively (p = 0.24). Late relapse, i.e. > 5 years from diagnosis, occurred in six patients (8 %). Relapse treatment consisted of combinations of surgery (25 %), focal radiotherapy (RT 22 %), high dose chemotherapy with stem cell rescue (HDSCR 21 %) and conventional chemotherapy (90 %). OS at 5 years after relapse was 6.0 +/- 4 %. In multivariate analysis; isolated relapse in PF, and surgery were significantly associated with prolonged survival whereas RT and HDSCR were not. Survival after relapse was not related to biological factors and was very poor despite several patients receiving intensive treatments. Exploration of new drugs is warranted, preferably based on tumour biology from biopsy of the relapsed tumour.
KW  - High-dose chemotherapy
KW  - Childhood medulloblastoma
KW  - Adolescents
KW  - Primitive neuroectodermal
KW  - Tumors
KW  - Recurrent medulloblastoma
KW  - Childrens-cancer
KW  - Phase-II
KW  - Trial
KW  - Therapy
KW  - Reirradiation
KW  - Medulloblastoma
KW  - Relapse
KW  - Survival
KW  - Treatment
KW  - Clinical trial
KW  - Chemotherapy
KW  - Radiotherapy
KW  - Paediatric
KW  - Secondary tumours
Y1  - 2016
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-187498
VL  - 129
IS  - 3
ER  -