TY  - JOUR
A1  - Dietzsch, Stefan
A1  - Braesigk, Annett
A1  - Seidel, Clemens
A1  - Remmele, Julia
A1  - Kitzing, Ralf
A1  - Schlender, Tina
A1  - Mynarek, Martin
A1  - Geismar, Dirk
A1  - Jablonska, Karolina
A1  - Schwarz, Rudolf
A1  - Pazos, Montserrat
A1  - Weber, Damien C.
A1  - Frick, Silke
A1  - Gurtner, Kristin
A1  - Matuschek, Christiane
A1  - Harrabi, Semi Ben
A1  - Glück, Albrecht
A1  - Lewitzki, Victor
A1  - Dieckmann, Karin
A1  - Benesch, Martin
A1  - Gerber, Nicolas U.
A1  - Obrecht, Denise
A1  - Rutkowski, Stefan
A1  - Timmermann, Beate
A1  - Kortmann, Rolf-Dieter
T1  - Types of deviation and review criteria in pretreatment central quality control of tumor bed boost in medulloblastoma—an analysis of the German Radiotherapy Quality Control Panel in the SIOP PNET5 MB trial
JF  - Strahlentherapie und Onkologie
N2  - Purpose
In Germany, Austria, and Switzerland, pretreatment radiotherapy quality control (RT-QC) for tumor bed boost (TB) in non-metastatic medulloblastoma (MB) was not mandatory but was recommended for patients enrolled in the SIOP PNET5 MB trial between 2014 and 2018. This individual case review (ICR) analysis aimed to evaluate types of deviations in the initial plan proposals and develop uniform review criteria for TB boost.

Patients and methods
A total of 78 patients were registered in this trial, of whom a subgroup of 65 patients were available for evaluation of the TB treatment plans. Dose uniformity was evaluated according to the definitions of the protocol. Additional RT-QC criteria for standardized review of target contours were elaborated and data evaluated accordingly.

Results
Of 65 initial TB plan proposals, 27 (41.5%) revealed deviations of target volume delineation. Deviations according to the dose uniformity criteria were present in 14 (21.5%) TB plans. In 25 (38.5%) cases a modification of the RT plan was recommended. Rejection of the TB plans was rather related to unacceptable target volume delineation than to insufficient dose uniformity.

Conclusion
In this analysis of pretreatment RT-QC, protocol deviations were present in a high proportion of initial TB plan proposals. These findings emphasize the importance of pretreatment RT-QC in clinical trials for MB. Based on these data, a proposal for RT-QC criteria for tumor bed boost in non-metastatic MB was developed.
KW  - brain tumor
KW  - pediatric
KW  - focal radiotherapy
KW  - quality assurance
KW  - individual case review
Y1  - 2022
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-307812
SN  - 0179-7158
SN  - 1439-099X
VL  - 198
IS  - 3
ER  - 
TY  - JOUR
A1  - Karastaneva, Anna
A1  - Lanz, Sofia
A1  - Wawer, Angela
A1  - Behrends, Uta
A1  - Schindler, Detlev
A1  - Dietrich, Ralf
A1  - Burdach, Stefan
A1  - Urban, Christian
A1  - Benesch, Martin
A1  - Seidel, Markus G.
T1  - Immune thrombocytopenia in two unrelated Fanconi anemia patients - a mere coincidence?
JF  - Frontiers in Pediatrics
N2  - Thrombocytopenia and pancytopenia, occurring in patients with Fanconi anemia (FA), are interpreted either as progression to bone marrow failure or as developing myelodysplasia. On the other hand, immune thrombocytopenia (ITP) represents an acquired and often self-limiting benign hematologic disorder, associated with peripheral, immune-mediated, platelet destruction requiring different management modalities than those used in congenital bone marrow failure syndromes, including FA. Here, we describe the clinical course of two independent FA patients with atypical – namely immune – thrombocytopenia. While in one patient belonging to complementation group FA-A, the ITP started at 17 months of age and showed a chronically persisting course with severe purpura, responding well to intravenous immunoglobulins (IVIG) and later also danazol, a synthetic androgen, the other patient (of complementation group FA-D2) had a self-limiting course that resolved after one administration of IVIG. No cytogenetic aberrations or bone marrow abnormalities other than FA-typical mild dysplasia were detected. Our data show that acute and chronic ITP may occur in FA patients and impose individual diagnostic and therapeutic challenges in this rare congenital bone marrow failure/tumor predisposition syndrome. The management and a potential context of immune pathogenesis with the underlying marrow disorder are discussed.
KW  - immune thrombocytopenia
KW  - bone marrow failure syndrome
KW  - Evans syndrome
KW  - danazol
KW  - FANCA
KW  - FANCD2
KW  - Fanconi anemia
KW  - DNA repair defect
Y1  - 2015
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-149837
VL  - 3
IS  - 50
ER  -