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Aparna Govindan, Muralikrishnan Vp, Jacob Paul Alapatt
Embryonal tumor with multilayered rosettes(ETMR) is a new entity in the group of Primitive Neuroectodermal tumors of the central nervous system. It combines histopathological features of neuroblastoma and ependymoblastoma. This tumor occurs mostly in the first five years of life. A hallmark cytogenetic abnormality at the chromosome locus 19q13.42 has been identified. The authors report a case of ETMR in the parietooccipital region in a 3 year old girl who presented with features of raised intracranial tension...
January 8, 2017: Turkish Neurosurgery
Katarzyna Modzelewska, Elena F Boer, Timothy L Mosbruger, Daniel Picard, Daniela Anderson, Rodney R Miles, Mitchell Kroll, William Oslund, Theodore J Pysher, Joshua D Schiffman, Randy Jensen, Cicely A Jette, Annie Huang, Rodney A Stewart
Malignant brain tumors are the leading cause of cancer-related deaths in children. Primitive neuroectodermal tumors of the CNS (CNS-PNETs) are particularly aggressive embryonal tumors of unknown cellular origin. Recent genomic studies have classified CNS-PNETs into molecularly distinct subgroups that promise to improve diagnosis and treatment; however, the lack of cell- or animal-based models for these subgroups prevents testing of rationally designed therapies. Here, we show that a subset of CNS-PNETs co-express oligoneural precursor cell (OPC) markers OLIG2 and SOX10 with coincident activation of the RAS/MAPK (mitogen-activated protein kinase) pathway...
October 25, 2016: Cell Reports
Jinglan Liu, Matthew P Keisling, Ayman Samkari, Gregory Halligan, Judy M Pascasio, Christos D Katsetos
Central nervous system (CNS) tumors exhibiting dual features of malignant glioma (MG) and primitive neuroectodermal tumor (PNET) are rare and diagnostically challenging. Previous studies have shown that MG-PNET carry MYCN or MYC gene amplifications within the PNET component concomitant with glioma-associated alterations, most commonly 10q loss, in both components [9]. Here we confirm and extend the profile of molecular genetic findings in a MG-PNET involving the left frontal lobe of a 12-year-old male. Histologically, the PNET-like component showed morphological features akin to anaplastic medulloblastoma highlighted by widespread immunoreactivity for βIII-tubulin (TUBB3) and nonphosphorylated neurofilament protein, and to a lesser degree, Neu-N, synaptophysin, and CD99, whereas the gliomatous component was demarcated by glial fibrillary acidic protein (GFAP) labeling...
November 2016: Clinical Neuropathology
Tanner M Johanns, Christopher A Miller, Ian G Dorward, Christina Tsien, Edward Chang, Arie Perry, Ravindra Uppaluri, Cole Ferguson, Robert E Schmidt, Sonika Dahiya, George Ansstas, Elaine R Mardis, Gavin P Dunn
: We present the case of a patient with a left frontal glioblastoma with primitive neuroectodermal tumor features and hypermutated genotype in the setting of a POLE germline alteration. During standard-of-care chemoradiation, the patient developed a cervical spine metastasis and was subsequently treated with pembrolizumab. Shortly thereafter, the patient developed an additional metastatic spinal lesion. Using whole-exome DNA sequencing and clonal analysis, we report changes in the subclonal architecture throughout treatment...
November 2016: Cancer Discovery
Changshu Ke 柯昌庶, Qiuhong Duan 段秋红, Hui Yang, Feng Zhu, Meng Yan, San-Peng Xu, Sheng Zhou, Feng Wan, Kai Shu, Ting Lei, Li-Ming Xia
Meningeal Ewing Sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) is a rare diagnostically challenging small round cell tumor in the CNS. This study investigates the clinical pathological features of four cases of this tumor from archives of 6 years in our hospital. Patients were within the median age of 21.5 years and male to female ratio was 1:1. The tumors distributed at the supra-tentorial location, posterior fossa and lumbar vertebral canal, usually presenting as the dura-sited nodule or having close connection with the meninges within the cranium or vertebral canal...
February 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Anobel Y Odisho, Joseph T Rabban, Maxwell V Meng
A 29-year-old man presenting with a retroperitoneal mass was found to have a testis lesion consistent with mixed germ cell tumor and the RPLND specimen showed teratoma with an area of central nervous system-type primitive neuroectodermal tumor (PNET) not present in the testis. Whether such primitive tumor components represent a de novo tumor component or represent progression from existing neuroepithelial teratomatous elements is unclear. Given the high likelihood of residual tumor and possibility of malignant transformation, post-chemotherapy RPLND remains vital in treating patients with testis cancer...
May 2016: Urology Case Reports
L Fairley, S V Picton, R J Q McNally, S Bailey, M G McCabe, R G Feltbower
BACKGROUND: Medulloblastoma and primitive neuroectodermal tumours (PNET) are the most common central nervous system (CNS) embryonal tumours diagnosed in childhood. Survival outcomes are worse for children diagnosed with CNS PNET compared to medulloblastoma. Less is known about survival outcomes in teenagers and young adults (TYA). METHODS: Data were extracted from two population-based cancer registries of children and young people (0-24 years) in the north of England for all diagnoses of medulloblastoma and CNS PNET between 1990 and 2013...
July 2016: European Journal of Cancer
(no author information available yet)
A recent study found that a majority of pediatric patients diagnosed with central nervous system primitive neuroectodermal tumors may have other types of central nervous system cancers instead. Researchers also concluded that these rare brain tumors fall into four distinct molecular subtypes. The findings may lead to more accurate diagnoses and more-tailored treatment plans.
May 2016: Cancer Discovery
Jeroen F Vermeulen, Wim van Hecke, Wim G M Spliet, José Villacorta Hidalgo, Paul Fisch, Roel Broekhuizen, Niels Bovenschen
BACKGROUND: Central nervous system (CNS) primitive neuroectodermal tumors (PNETs) are malignant primary brain tumors that occur in young infants. Using current standard therapy, up to 80% of the children still dies from recurrent disease. Cellular immunotherapy might be key to improve overall survival. To achieve efficient killing of tumor cells, however, immunotherapy has to overcome cancer-associated strategies to evade the cytotoxic immune response. Whether CNS-PNETs can evade the immune response remains unknown...
2016: PloS One
Dominik Sturm, Brent A Orr, Umut H Toprak, Volker Hovestadt, David T W Jones, David Capper, Martin Sill, Ivo Buchhalter, Paul A Northcott, Irina Leis, Marina Ryzhova, Christian Koelsche, Elke Pfaff, Sariah J Allen, Gnanaprakash Balasubramanian, Barbara C Worst, Kristian W Pajtler, Sebastian Brabetz, Pascal D Johann, Felix Sahm, Jüri Reimand, Alan Mackay, Diana M Carvalho, Marc Remke, Joanna J Phillips, Arie Perry, Cynthia Cowdrey, Rachid Drissi, Maryam Fouladi, Felice Giangaspero, Maria Łastowska, Wiesława Grajkowska, Wolfram Scheurlen, Torsten Pietsch, Christian Hagel, Johannes Gojo, Daniela Lötsch, Walter Berger, Irene Slavc, Christine Haberler, Anne Jouvet, Stefan Holm, Silvia Hofer, Marco Prinz, Catherine Keohane, Iris Fried, Christian Mawrin, David Scheie, Bret C Mobley, Matthew J Schniederjan, Mariarita Santi, Anna M Buccoliero, Sonika Dahiya, Christof M Kramm, André O von Bueren, Katja von Hoff, Stefan Rutkowski, Christel Herold-Mende, Michael C Frühwald, Till Milde, Martin Hasselblatt, Pieter Wesseling, Jochen Rößler, Ulrich Schüller, Martin Ebinger, Jens Schittenhelm, Stephan Frank, Rainer Grobholz, Istvan Vajtai, Volkmar Hans, Reinhard Schneppenheim, Karel Zitterbart, V Peter Collins, Eleonora Aronica, Pascale Varlet, Stephanie Puget, Christelle Dufour, Jacques Grill, Dominique Figarella-Branger, Marietta Wolter, Martin U Schuhmann, Tarek Shalaby, Michael Grotzer, Timothy van Meter, Camelia-Maria Monoranu, Jörg Felsberg, Guido Reifenberger, Matija Snuderl, Lynn Ann Forrester, Jan Koster, Rogier Versteeg, Richard Volckmann, Peter van Sluis, Stephan Wolf, Tom Mikkelsen, Amar Gajjar, Kenneth Aldape, Andrew S Moore, Michael D Taylor, Chris Jones, Nada Jabado, Matthias A Karajannis, Roland Eils, Matthias Schlesner, Peter Lichter, Andreas von Deimling, Stefan M Pfister, David W Ellison, Andrey Korshunov, Marcel Kool
Primitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are highly aggressive, poorly differentiated embryonal tumors occurring predominantly in young children but also affecting adolescents and adults. Herein, we demonstrate that a significant proportion of institutionally diagnosed CNS-PNETs display molecular profiles indistinguishable from those of various other well-defined CNS tumor entities, facilitating diagnosis and appropriate therapy for patients with these tumors. From the remaining fraction of CNS-PNETs, we identify four new CNS tumor entities, each associated with a recurrent genetic alteration and distinct histopathological and clinical features...
February 25, 2016: Cell
Wafik Zaky
Pediatric central nervous system primitive neuro-ectodermal brain tumors (CNS-PNETs) are rare tumors with ill-defined biological features. In this issue of Cell, Sturm et al. used state-of-the-art methods to interrogate these tumors' biology. Their integrated molecular analyses led them to propose a new molecular classification, with four new entities identified, that should get oncologists' attention.
February 25, 2016: Cell
Ben O'Leary, Henry C Mandeville, Naomi Fersht, Francesca Solda, Julie Mycroft, Stergios Zacharoulis, Sucheta Vaidya, Frank Saran
There is no standard treatment for glioblastoma with elements of PNET (GBM-PNET). Conventional treatment for glioblastoma is surgery followed by focal radiotherapy with concurrent temozolomide. Given the increased propensity for neuroaxial metastases seen with GBM-PNETs, craniospinal irradiation (CSI) with temozolomide (TMZ) could be a feasible treatment option but little is known regarding its toxicity. The clinical records of all patients treated at two UK neuro-oncology centres with concurrent CSI and TMZ were examined for details of surgery, radiotherapy, chemotherapy and toxicities related to the CSI-TMZ component of their treatment...
April 2016: Journal of Neuro-oncology
Nicole L Drezner, Roger J Packer
Embryonal tumors represent a heterogeneous group of malignancies characterized by poorly differentiated cells and generally aggressive behavior. Although advances in survival rates have been made in several of these tumor types, including Wilms' tumor, retinoblastoma, and medulloblastoma, survival of patients with central nervous system (CNS) embryonal tumors, including primitive neuro-ectodermal tumors (PNETs) and atypical teratoid rhabdoid tumors (AT/RT), are particularly poor. Advancing molecular analysis techniques and the development of gene expression profiles has led to the formulation of different subdivisions within many of the umbrella CNS tumor groups with clinical and prognostic implications...
January 2016: Translational pediatrics
G Egan, K A Cervone, P C Philips, J B Belasco, J L Finlay, S L Gardner
Recurrence of malignant brain tumors results in a poor prognosis with limited treatment options. High-dose chemotherapy with autologous hematopoietic cell rescue (AHCR) has been used in patients with recurrent malignant brain tumors and has shown improved outcomes compared with standard chemotherapy. Temozolomide is standard therapy for glioblastoma and has also shown activity in patients with medulloblastoma/primitive neuro-ectodermal tumor (PNET), particularly those with recurrent disease. Temozolomide was administered twice daily on days -10 to -6, followed by thiotepa 300 mg/m(2) per day and carboplatin dosed using the Calvert formula or body surface area on days -5 to -3, with AHCR day 0...
April 2016: Bone Marrow Transplantation
Cara L Sedney, Sanjay Bhatia, Fahad Bafakih, Stephan Paul, Kymberly Gyure
Secondary, radiation-induced, supratentorial primitive neuroectodermal tumors (PNETs) are extremely rare entities which may present in survivors of childhood cancers after central nervous system radiation. These lesions have been described after a number of pediatric cancers and demonstrate unique treatment problems and an accelerated clinical course compared to primary PNETs. We present a case of a sixteen year old male with a history of non-Hodgkin's lymphoma who presented with a radiation-induced PNET, and describe our treatment for this lesion...
July 2015: West Virginia Medical Journal
Jing Xie, Jin Wen, Ya-Lan Bi, Han-Zhong Li
Primitive neuroectodermal tumour (PNET) is a highly aggressive neoplasm that develops classically in the central nervous system. PNET of the kidney (rPNET) is extremely rare. Recently, a 23-year-old woman complained of left flank pain and intermittent hematuria for 3 months and was admitted to our hospital. A computed tomography (CT) scan and magnetic resonance imaging demonstrated a 5.1 × 4.4-cm heterogenous mass with unconspicuous reinforcement in the upper pole of the left kidney. F18-FDG positron emission tomography CT (PET-CT) revealed the mass as a benign lesion with internal extensive bleeding...
May 2015: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
Heather E Danysh, Laura E Mitchell, Kai Zhang, Michael E Scheurer, Philip J Lupo
BACKGROUND: Due to increasing concerns regarding air pollution and childhood cancer, we conducted a population-based study evaluating the association between traffic-related hazardous air pollutants (1,3-butadiene, benzene, diesel particulate matter [DPM]) and the incidence of childhood central nervous system (CNS) tumors. PROCEDURE: Information on children diagnosed with a CNS tumor at <15 years of age, in Texas, for the period of 2001-2009 (n = 1,949) was obtained from the Texas Cancer Registry...
September 2015: Pediatric Blood & Cancer
Johannes Nowak, Carolin Seidel, Torsten Pietsch, Balint Alkonyi, Taylor Laura Fuss, Carsten Friedrich, Katja von Hoff, Stefan Rutkowski, Monika Warmuth-Metz
BACKGROUND: Ependymoblastoma (EBL), ependymoma (EP), and primitive neuroectodermal tumors of the central nervous system not otherwise specified (CNS-PNET NOS) are pediatric brain tumors that can be differentiated by histopathology in the clinical setting. Recently, we described specific MRI features of EBL. In this study, we compare standardized MRI characteristics of EBL with EP and CNS-PNET NOS in a series comprising 22 patients in each group. METHODS: All 66 centrally reviewed cases were obtained from the database of the German multicenter HIT trials...
August 2015: Neuro-oncology
Sergey Malchenko, Simone Treiger Sredni, Hitoshi Hashimoto, Atsushi Kasai, Kazuki Nagayasu, Jianping Xie, Naira V Margaryan, Kaoru Seiriki, Rishi R Lulla, Richard E B Seftor, Lauren M Pachman, Herbert Y Meltzer, Mary J C Hendrix, Marcelo B Soares
There is growing evidence and a consensus in the field that most pediatric brain tumors originate from stem cells, of which radial glial cells constitute a subtype. Here we show that orthotopic transplantation of human radial glial (RG) cells to the subventricular zone of the 3rd ventricle--but not to other transplantation sites--of the brain in immunocompromised NOD-SCID mice, gives rise to tumors that have the hallmarks of CNS primitive neuroectodermal tumors (PNETs). The resulting mouse model strikingly recapitulates the phenotype of PNETs...
2015: PloS One
Regina I Jakacki, Peter C Burger, Mehmet Kocak, James M Boyett, Joel Goldwein, Minesh Mehta, Roger J Packer, Nancy J Tarbell, Ian F Pollack
BACKGROUND: Supratentorial PNETs (sPNET) are uncommon embryonal malignancies of the central nervous system whose prognosis has historically been poor. We evaluated the outcome and prognostic factors of children with sPNET treated prospectively on a Children's Oncology Group trial. PROCEDURE: Following surgery, patients received craniospinal radiotherapy with concurrent carboplatin followed by six months of maintenance chemotherapy with cyclophosphamide and vincristine...
May 2015: Pediatric Blood & Cancer
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