TY  - JOUR
A1  - Gaab, Christine
A1  - Adolph, Jonas E.
A1  - Tippelt, Stephan
A1  - Mikasch, Ruth
A1  - Obrecht, Denise
A1  - Mynarek, Martin
A1  - Rutkowski, Stefan
A1  - Pfister, Stefan M.
A1  - Milde, Till
A1  - Witt, Olaf
A1  - Bison, Brigitte
A1  - Warmuth-Metz, Monika
A1  - Kortmann, Rolf-Dieter
A1  - Dietzsch, Stefan
A1  - Pietsch, Torsten
A1  - Timmermann, Beate
A1  - Sträter, Ronald
A1  - Bode, Udo
A1  - Faldum, Andreas
A1  - Kwiecien, Robert
A1  - Fleischhack, Gudrun
T1  - Local and systemic therapy of recurrent medulloblastomas in children and adolescents: results of the P-HIT-REZ 2005 Study
JF  - Cancers
N2  - Recurrent medulloblastomas are associated with survival rates <10%. Adequate multimodal therapy is being discussed as having a major impact on survival. In this study, 93 patients with recurrent medulloblastoma treated in the German P-HIT-REZ 2005 Study were analyzed for survival (PFS, OS) dependent on patient, disease, and treatment characteristics. The median age at the first recurrence was 10.1 years (IQR: 6.9–16.1). Median PFS and OS, at first recurrence, were 7.9 months (CI: 5.7–10.0) and 18.5 months (CI: 13.6–23.5), respectively. Early relapses/progressions (<18 months, n = 30/93) found mainly in molecular subgroup 3 were associated with markedly worse median PFS (HR: 2.34) and OS (HR: 3.26) in regression analyses. A significant survival advantage was found for the use of volume-reducing surgery as well as radiotherapy. Intravenous chemotherapy with carboplatin and etoposide (ivCHT, n = 28/93) showed improved PFS and OS data and the best objective response rate (ORR) was 66.7% compared to oral temozolomide (oCHT, n = 47/93) which was 34.8%. Intraventricular (n = 43) as well as high-dose chemotherapy (n = 17) at first relapse was not related to a significant survival benefit. Although the results are limited due to a non-randomized study design, they may serve as a basis for future treatment decisions in order to improve the patients' survival.
KW  - medulloblastoma
KW  - refractory
KW  - recurrent
KW  - children
KW  - chemotherapy
KW  - surgery
KW  - radiotherapy
KW  - re-irradiation
KW  - intraventricular therapy
Y1  - 2022
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-254809
SN  - 2072-6694
VL  - 14
IS  - 3
ER  - 
TY  - JOUR
A1  - Linsenmann, Thomas
A1  - Monoranu, Camelia M.
A1  - Alkonyi, Balint
A1  - Westermaier, Thomas
A1  - Hagemann, Carsten
A1  - Kessler, Almuth F.
A1  - Ernestus, Ralf-Ingo
A1  - Löhr, Mario
T1  - Cerebellar liponeurocytoma - molecular signature of a rare entity and the importance of an accurate diagnosis
JF  - Interdisciplinary Neurosurgery
N2  - Background:
Cerebellar liponeurocytoma is an extremely rare tumour entity of the central nervous system. It is histologically characterised by prominent neuronal/neurocytic differentiation with focal lipidisation and corresponding histologically to WHO grade II. It typically develops in adults, and usually shows a low proliferative potential. Recurrences have been reported in almost 50% of cases, and in some cases the recurrent tumour may display increased mitotic activity and proliferation index, vascular proliferations and necrosis. Thus pathological diagnosis of liponeurocytoma is challenging. This case presentation highlights the main clinical, radiographic and pathological features of a cerebellar liponeurocytoma.

Case presentation:
A 59-year-old, right-handed woman presented at our department with a short history of persistent headache, vertigo and gait disturbances. Examination at presentation revealed that the patient was awake, alert and fully oriented. The cranial nerve status was normal. Uncertainties were noted in the bilateral finger-to-nose testing with bradydiadochokinesis on both sides. Strength was full and no pronator drift was observed. Sensation was intact. No signs of pyramidal tract dysfunction were detected. Her gait appeared insecure. The patient underwent surgical resection. Afterward no further disturbances could be detected.

Conclusions:
To date >40 cases of liponeurocytoma have been reported, including cases with supratentorial location. A review of the 5 published cases of recurrent cerebellar. Liponeurocytoma revealed that the median interval between the first and second relapse was rather short, indicating uncertain malignant potential. The most recent WHO classification of brain tumours (2016) classifies the cerebellar liponeurocytoma as a separate entity and assigns the tumour to WHO grade II. Medulloblastoma is the most important differential diagnosis commonly seen in children and young adults. In contrast, cerebellar liponeurocytoma is typically diagnosed in adults. The importance of accurate diagnosis should not be underestimated especially in the view of possible further therapeutic interventions and for the determination of the patient's prognosis.
KW  - liponeurocytoma
KW  - neurocytoma
KW  - medulloblastoma
KW  - molecular signature
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-177652
VL  - 16
ER  -