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Alice Indini, Elisabetta Schiavello, Veronica Biassoni, Luca Bergamaschi, Maria Chiara Magni, Nadia Puma, Stefano Chiaravalli, Federica Pallotti, Ettore Seregni, Barbara Diletto, Emilia Pecori, Lorenza Gandola, Geraldina Poggi, Maura Massimino
To assess the long-term safety of administering growth hormone (GH) in patients with GH deficiency due to treatment for childhood medulloblastoma and primitive neuroectodermal tumor (PNET). Data were retrospectively retrieved on children receiving GH supplementation, assessing their disease-free and overall survival outcomes and risk of secondary malignancies using Kaplan-Meier and Cox models. Overall 65 children were consecutively collected from May 1981 to April 2013. All patients had undergone craniospinal irradiation (total dose 18-39 Gy), and subsequently received GH for a median (interquartile range, IQR) of 81 (50...
October 21, 2016: Journal of Neuro-oncology
Kathrin Fielitz, Kristina Althoff, Katleen De Preter, Julie Nonnekens, Jasmin Ohli, Sandra Elges, Wolfgang Hartmann, Günter Klöppel, Thomas Knösel, Marc Schulte, Ludger Klein-Hitpass, Daniela Beisser, Henning Reis, Annette Eyking, Elke Cario, Johannes H Schulte, Alexander Schramm, Ulrich Schüller
Amplification or overexpression of MYCN is involved in development and maintenance of multiple malignancies. A subset of these tumors originates from neural precursors, including the most aggressive forms of the childhood tumors, neuroblastoma and medulloblastoma. In order to model the spectrum of MYCN-driven neoplasms in mice, we transgenically overexpressed MYCN under the control of the human GFAP-promoter that, among other targets, drives expression in neural progenitor cells. However, LSL-MYCN;hGFAP-Cre double transgenic mice did neither develop neural crest tumors nor tumors of the central nervous system, but presented with neuroendocrine tumors of the pancreas and, less frequently, the pituitary gland...
October 19, 2016: Oncotarget
Emmanuel Jouglar, Antoine Wagner, Grégory Delpon, Loïc Campion, Philippe Meingan, Valérie Bernier, Charlotte Demoor-Goldschmidt, Marc-André Mahé, Thomas Lacornerie, Stéphane Supiot
OBJECTIVES: Late abdominal irradiation toxicity during childhood included renal damage, hepatic toxicity and secondary diabetes mellitus. We compared the potential of conformal radiotherapy (CRT), helical tomotherapy (HT) and proton beam therapy (PBT) to spare the abdominal organs at risk (pancreas, kidneys and liver- OAR) in children undergoing abdominal irradiation. METHODS: We selected children with abdominal tumors who received more than 10 Gy to the abdomen...
2016: PloS One
P Papanagiotou, M Politi
Various types of brain tumor can occur in the region of the posterior fossa. Brain metastases in adults are the most common malignancies at this localization. Ependymomas, medulloblastomas and pilocytic astrocytomas occur mostly in children and only rarely in adults. Other tumors that occur in the posterior fossa are meningiomas, schwannomas, hemangioblastomas, brain stem gliomas and epidermoid tumors. Due to the fact that the various tumors of the posterior fossa have different treatment approaches and prognoses, an accurate and specific diagnosis is mandatory...
October 18, 2016: Der Radiologe
Aruna Nambirajan, Mehar C Sharma, Madhu Rajeshwari, Aanchal Kakkar, Vaishali Suri, Chitra Sarkar
Ependymomas are gliomas that recapitulate normal ependymal cells. The epithelial membrane antigen (EMA) shows "dot-like" and "ring-like" staining patterns, highlighting "microlumens" or intracytoplasmic rosettes, a pathognomonic ultrastructural feature. NHERF1/EBP50, an adaptor protein localized at the apical plasma membrane of human epithelia, has been found to localize to these microlumens. We aimed to analyze the staining patterns of EMA and EBP50 in ependymomas and other tumors, and thereby compare their diagnostic utility...
October 7, 2016: Applied Immunohistochemistry & Molecular Morphology: AIMM
Gang Liu, Jun Yang, Juan Wang, Xiaohua Liu, Wenjing Huang, Jie Li, Wenfu Tan, Ao Zhang
A series of novel Smo antagonists were developed either by directly incorporating the basic skeleton of the natural product artemisinin or by first breaking artemisinin into structurally simpler and stable intermediates and then reconstructing into diversified heterocyclic derivatives, equipped with a Smo-targeting bullet. 2-(2,5-Dimethyl-5,6,7,8-tetrahydroquinolin-8-yl)-N-arylpropanamide 65 was identified as the most potent with an IC50 value of 9.53 nM against the Hh signaling pathway. Complementary mechanism studies confirmed that 65 inhibits Hh signaling pathway by targeting Smo and shares the same binding site as that of the tool drug cyclopamine...
October 13, 2016: Journal of Medicinal Chemistry
Mincione Gabriella, Di Marcantonio Maria Carmela, Tarantelli Chiara, Savino Luca, Ponti Donatella, Marchisio Marco, Lanuti Paola, Sancilio Silvia, Calogero Antonella, Di Pietro Roberta, Muraro Raffaella
Epidermal Growth Factor Receptor (EGFR), a member of the ErbB family of receptor tyrosine kinase (RTK) proteins, is aberrantly expressed or deregulated in tumors and plays pivotal roles in cancer onset and metastatic progression. ZNF216 gene has been identified as one of Immediate Early Genes (IEGs) induced by RTKs. Overexpression of ZNF216 protein sensitizes 293 cell line to TNF-α induced apoptosis. However, ZNF216 overexpression has been reported in medulloblastomas and metastatic nasopharyngeal carcinomas...
October 6, 2016: Oncotarget
Mwangala P Akamandisa, Rita Nahta, Robert C Castellino
No abstract text is available yet for this article.
2016: Oncoscience
Rebecca L Eccles, Maciej T Czajkowski, Carolin Barth, Paul Markus Müller, Erik McShane, Stephan Grunwald, Patrick Beaudette, Nora Mecklenburg, Rudolf Volkmer, Kerstin Zühlke, Gunnar Dittmar, Matthias Selbach, Annette Hammes, Oliver Daumke, Enno Klussmann, Sylvie Urbé, Oliver Rocks
Protein kinase A is a key mediator of cAMP signalling downstream of G-protein-coupled receptors, a signalling pathway conserved in all eukaryotes. cAMP binding to the regulatory subunits (PKAR) relieves their inhibition of the catalytic subunits (PKAC). Here we report that ARHGAP36 combines two distinct inhibitory mechanisms to antagonise PKA signalling. First, it blocks PKAC activity via a pseudosubstrate motif, akin to the mechanism employed by the protein kinase inhibitor proteins. Second, it targets PKAC for rapid ubiquitin-mediated lysosomal degradation, a pathway usually reserved for transmembrane receptors...
October 7, 2016: Nature Communications
Hee Jung Yoo, Hyery Kim, Hyeon Jin Park, Dong Seok Kim, Young Shin Ra, Hee Young Shin
The neurocognitive function and quality of life of 58 Korean survivors of childhood medulloblastoma were assessed after surgery, cranial radiation and chemotherapy. All patients were evaluated with a battery of neurocognitive function tests and the Pediatric Functional Assessment of Cancer Therapy-Brain Tumor Survivors, which consists of self-report questionnaires on quality of life. The mean full-scale intelligence quotient (IQ), verbal IQ, and performance IQ scores were 90.2, 97.1, and 84.16, respectively...
November 2016: Journal of Korean Medical Science
Ryuya Yamanaka, Azusa Hayano, Tomohiko Kanayama
By conducting a systemic search of the PubMed database, we performed a comprehensive literature review to characterize secondary gliomas following radiotherapy treatment and to determine the most appropriate treatment strategy. Our analysis included 296 cases of radiation-induced gliomas. The primary lesion was characterized as a hematological malignancy in 104 cases (35.1 %), pituitary adenoma in 35 (11.8 %), craniopharyngioma in 19 (6.4 %), medulloblastoma in 38 (12.8 %), germ cell tumor in 13 (4.3 %), low-grade glioma in 28 (9...
October 5, 2016: Neurosurgical Review
Ahitagni Biswas, Lakhan Kashyap, Aanchal Kakkar, Chitra Sarkar, Pramod Kumar Julka
Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal central nervous system tumor commonly affecting children <3 years of age. It roughly constitutes 1%-2% of all pediatric central nervous system tumors. Recent data show that it is the most common malignant central nervous system tumor in children <6 months of age. Management of this aggressive tumor is associated with a myriad of diagnostic and therapeutic challenges. On the basis of radiology and histopathology alone, distinction of AT/RT from medulloblastoma or primitive neuroectodermal tumor is difficult, and hence this tumor has been commonly misdiagnosed as primitive neuroectodermal tumor for decades...
2016: Cancer Management and Research
Marta Viana-Pereira, Gisele Caravina Almeida, João Norberto Stavale, Susana Malheiro, Carlos Clara, Patrícia Lobo, José Pimentel, Rui Manuel Reis
Hotspot activating mutations of the telomerase reverse transcriptase (hTERT) promoter region were recently described in several tumor types. These mutations lead to enhanced expression of telomerase, being responsible for telomere maintenance and allowing continuous cell division. Additionally, there are alternative telomere maintenance mechanisms, associated with histone H3 mutations, responsible for disrupting the histone code and affecting the regulation of transcription. Here, we investigated the clinical relevance of these mechanistically related molecules in medulloblastoma...
October 1, 2016: Pathobiology: Journal of Immunopathology, Molecular and Cellular Biology
Chizuru Tsuruoka, Benjamin Blyth, Takamitsu Morioka, Mutsumi Kaminishi, Mayumi Shinagawa, Yoshiya Shimada, Shizuko Kakinuma
Recently reported studies have led to a heightened awareness of the risks of cancer induced by diagnostic radiological imaging, and in particular, the risk of brain cancer after childhood CT scans. One feature of Ptch1(+/-) mice is their sensitivity to radiation-induced medulloblastomas (an embryonic cerebellar tumor) during a narrow window of time centered on the days around birth. Little is known about the dynamics of how dose protraction interacts with such narrow windows of sensitivity in individual tissues...
September 30, 2016: Radiation Research
Emil Lou, Matthew Schomaker, Jon D Wilson, Mary Ahrens, Michelle Dolan, Andrew C Nelson
Medulloblastoma is an aggressive primitive neuroectodermal tumor of the cerebellum that is rare in adults. Medulloblastomas fall into 4 prognostically significant molecular subgroups that are best defined by experimental gene expression profiles: the WNT pathway, sonic hedgehog (SHH) pathway, and subgroups 3 and 4 (non-SHH/WNT). Medulloblastoma of adults belong primarily to the SHH category. Vismodegib, an SHH-pathway inhibitor FDA-approved in 2012 for treatment of basal cell carcinoma, has been used successfully in the setting of chemorefractory medulloblastoma, but not as a first-line therapy...
August 12, 2016: Cancer Biology & Therapy
C Kart, S Guven, E Guvendag Güven
No abstract text is available yet for this article.
November 2015: Journal of Minimally Invasive Gynecology
D Raleigh, A L Krup, P K Choksi, J F Reiter
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
B H Kann, N H Lester-Coll, H S M Park, D N Yeboa, R S Bindra, K P Becker, J Baehring, K B Roberts
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
K D Kelley, C Powell, S Chakraborty, A C Wright, S B Al Dimassi, M Symons, R Ruggieri
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
C Carrie, S Bolle, L Padovani, V Bernier, S Supiot, E Leprise, J L Habrand, C Alapetite, P Y Bondiau, B Coche-Dequéant, G Truc, C Pasteuris, C Kerr, T D Nguyen, S Dussart
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
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