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https://www.readbyqxmd.com/read/29629846/molecular-profiling-of-pancreatic-neuroendocrine-tumors-pnets-and-the-clinical-potential
#1
Massimiliano Camilli, Konstantinos Papadimitrio, Amanda Nogueira, Lorena Incorvaia, Antonio Galvano, Federica D'Antonio, Jose Ferri, Daniele Santini, Nicola Silvestris, Antonio Russo, Marc Peeters, Christian Rolfo
Pancreatic neuroendocrine tumors (pNETs) represent a small part of pancreatic neoplasms, and the knowledge about their indolent clinical course remains a subject of investigation. They occur sporadically or as part of familial cancer syndromes and are classified by WHO in 3 categories. There is ongoing research to understand their molecular profiling and leading mutations. Areas covered: The aim of this review is to clarify the overall aspects of tumorigenesis, to expose the latest developments in understanding the course of the disease and the possible therapeutic implications of these...
April 9, 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29616127/therapeutic-approach-in-glioblastoma-multiforme-with-primitive-neuroectodermal-tumor-components-case-report-and-review-of-the-literature
#2
Arsela Prelaj, Sara Elena Rebuzzi, Giovanni Caffarena, Julio Rodrigo Giròn Berrìos, Silvia Pecorari, Carmela Fusto, Alessandro Caporlingua, Federico Caporlingua, Annamaria Di Palma, Fabio Massimo Magliocca, Maurizio Salvati, Silverio Tomao, Vincenzo Bianco
Glioblastoma multiforme (GBM) is the most common and aggressive malignant glioma that is treated with first-line therapy, using surgical resection followed by local radiotherapy and concomitant/adjuvant temozolomide (TMZ) treatment. GBM is characterised by a high local recurrence rate and a low response to therapy. Primitive neuroectodermal tumour (PNET) of the brain revealed a low local recurrence rate; however, it also exhibited a high risk of cerebrospinal fluid (CSF) dissemination. PNET is treated with surgery followed by craniospinal irradiation (CSI) and platinum-based chemotherapy in order to prevent CSF dissemination...
May 2018: Oncology Letters
https://www.readbyqxmd.com/read/29520437/central-nervous-system-cns-neuroblastoma-a-case-based-update
#3
Federico Bianchi, Gianpiero Tamburrini, Marco Gessi, Paolo Frassanito, Luca Massimi, Massimo Caldarelli
BACKGROUND: Primary central nervous system (CNS) neuroblastoma is a rare intracranial tumor affecting children mainly in the first years of life. It is usually a supratentorial tumor with a wide spectrum of clinical presentation, seizures, and focal neurological deficits being the most common presenting signs. CASE DESCRIPTION: A 2-year-old child was admitted to our ward after a generalized seizure. Neurological examination was normal. Radiological studies showed a small DWI hyperintense lesion of the right rectus gyrus...
March 8, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29248974/management-and-outcome-of-children-and-adolescents-with-non-medulloblastoma-cns-embryonal-tumors-in-spain-room-for-improvement-in-standards-of-care
#4
Teresa de Rojas, Francisco Bautista, Miguel Flores, Lucía Igual, Raquel Rubio, Eduardo Bardón, Lucía Navarro, Laura Murillo, Raquel Hladun, Adela Cañete, Miguel Garcia-Ariza, Carmen Garrido, Ana Fernández-Teijeiro, Eduardo Quiroga, Carlota Calvo, Anna Llort, Inmaculada de Prada, Luis Madero, Ofelia Cruz, Lucas Moreno
Non-medulloblastoma CNS embryonal tumors (former PNET/Pineoblastomas) are aggressive malignancies with poor outcome that have been historically treated with medulloblastoma protocols. The purpose of this study is to present a tumor-specific, real-world data cohort of patients with CNS-PNET/PB to analyze quality indicators that can be implemented to improve the outcome of these patients. Patients 0-21 years with CNS-PNET treated in eight large institutions were included. Baseline characteristics, treatment and outcome [progression-free and overall survival (PFS and OS respectively)] were analyzed...
March 2018: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29151797/late-onset-of-primary-pulmonary-primitive-neuroectodermal-tumor-a-case-report
#5
Bilgin Bahadir Başgöz, Adem Aydin, Semra Ince, Ibrahim Demirci, Ayhan Özcan
Lungs are one of most metastatic areas for primitive neuroectodermal tumor (PNET), however primary pulmonary PNET is extremely rare. Here we present a case of a 58-year-old male patient with a tumor in the right lung that originated from the lung but not from chest wall. Patient was diagnosed with PNET following histological and immunohistochemical examination of CT-guided percutaneous tru-cut needle biopsy and no distant metastasis were detected in PET-CT scan. As advised recently in published literature, surgical resection following neoadjuvant chemotherapy protocol is preferred in the treatment of our patient as it has better success of complete resection leading to higher 5-year survival rates...
2017: Clujul Medical (1957)
https://www.readbyqxmd.com/read/29089709/co-existence-of-diffuse-serous-cystadenoma-and-pancreatic-neuroendocrine-tumor
#6
Mallika Tewari, Shashikant Patne, Richa Katiyar, Dipanjan Biswas, H S Shukla
Diffuse serous cystic neoplasm (SCN) associated with pancreatic neuroendocrine tumor (PNET) is a rare finding reported previously in only three patients to the best of our knowledge. We herein present one such interesting report of a diffuse serous cystic adenoma (SCA) and co-existent PNET in a 25-year old lady who presented with abdominal pain for past 6 months. A triple-phase pancreatic protocol computed tomography (CT) scan revealed multiple cysts involving the entire pancreas. The cysts were thin walled, ranging from 2 to 8 cm in width, with no calcification or central scar that was confirmed at laparotomy...
October 2017: Indian Journal of Surgery
https://www.readbyqxmd.com/read/28256369/anti-hu-paraneoplastic-brainstem-encephalitis-caused-by-a-pancreatic-neuroendocrine-tumor-presenting-with-central-hypoventilation
#7
Marc Najjar, Andrew Taylor, Surbhi Agrawal, Tito Fojo, Alexander E Merkler, Marc K Rosenblum, Laura Lennihan, Michael D Kluger
Paraneoplastic neurological syndromes are rare autoimmune manifestations of malignancies associated with specific antibodies. Anti-Hu associated brainstem encephalitis, a well-described syndrome, usually presents subacutely with preferential involvement of the medulla. Anti-Hu antibodies target intraneuronal antigens and are therefore highly correlated with neurological syndromes when present concomitantly with a neoplasm. Reported is a case of anti-Hu brainstem encephalitis associated with a pancreatic neuroendocrine tumor (PNET) presenting with central hypoventilation...
June 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/27174220/current-knowledge-on-the-sensitivity-of-the-68-ga-somatostatin-receptor-positron-emission-tomography-and-the-suvmax-reference-range-for-management-of-pancreatic-neuroendocrine-tumours
#8
REVIEW
Irene Virgolini, Michael Gabriel, Alexander Kroiss, Elisabeth von Guggenberg, Rupert Prommegger, Boris Warwitz, Bernhard Nilica, Llanos Geraldo Roig, Margarida Rodrigues, Christian Uprimny
Physiologically increased pancreatic uptake at the head/uncinate process is observed in more than one-third of patients after injection of one of the three (68)Ga-labelled octreotide-based peptides used for somatostatin (sst) receptor (r) imaging. There are minor differences between these (68)Ga-sstr-binding peptides in the imaging setting. On (68)Ga-sstr-imaging the physiological uptake can be diffuse or focal and usually remains stable over time. Differences in the maximal standardised uptake values (SUVmax) reported for the normal pancreas as well as for pancreatic neuroendocrine tumour (PNET) lesions may be related to several factors, including (a) differences in the peptide binding affinities as well as differences in sstr subtype expression of pancreatic α- and β-cells, and heterogeneity / density of tumour cells, (b) differences in scanner resolution, image reconstruction techniques and acquisition protocols, (c) mostly retrospective study designs, (d) mixed patient populations, or (e) interference with medications such as treatment with long-acting sst analogues...
October 2016: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/27161746/po-60-renal-tumors-with-extensive-vascular-disease-management-challenges-in-a-pediatric-series-from-the-hospital-for-sick-children
#9
G Zamperlini-Netto, A Zanette, E Wehbi, S Williams, R M Grant, L R Brandao
INTRODUCTION: Venous thrombotic events (VTE) are becoming more and more common in children, particularly in the hospital setting. To date, 1 in 200 children admitted to tertiary pediatric hospitals are now being recognized to develop VTE. Amongst those patients with an identified thrombotic occlusion, pediatric patients diagnosed with renal tumors have long been recognized, but their ideal management in the instances of vascular invasion remains controversial. AIM: We describe the clinical behavior of patients diagnosed with renal tumors and extra renal vascular involvement at The Hospital for Sick Children in Toronto, Canada...
April 2016: Thrombosis Research
https://www.readbyqxmd.com/read/26919653/pancreatic-incidentaloma-differentiating-nonfunctioning-pancreatic-neuroendocrine-tumors-from-intrapancreatic-accessory-spleen
#10
Ester Osher, Erez Scapa, Joseph Klausner, Yona Greenman, Karen Tordjman, Alla Melhem, Ido Nachmany, Yael Sofer, Ravit Geva, Arye Blachar, Naftali Stern, Erwin Santo
OBJECTIVE: To improve the preoperative assessment of pancreatic incidentalomas (PIs) by analysis of 1 index case and characterization of the published features of intrapancreatic accessory spleen (IPAS) compared to pancreatic neuroendocrine tumor (PNET). METHODS: A search of the literature using the online database MEDLINE. RESULTS: In all, 46 cases of IPAS have been described to date: 17 were "presumed" as IPAS based on technetium-99m (Tc-99m) scanning, fine-needle aspiration (FNA) stain for CD8, or contrast-enhanced sonography; 29 were misdiagnosed as PNET and underwent surgery...
July 2016: Endocrine Practice
https://www.readbyqxmd.com/read/26674063/clinical-presentation-and-outcome-of-nonfunctional-pancreatic-neuroendocrine-tumors-in-a-modern-cohort
#11
Duncan C Watley, Quan P Ly, Geoffrey Talmon, Chandrakanth Are, Aaron R Sasson
BACKGROUND: The natural history of nonfunctional pancreatic neuroendocrine tumors (NF-PNETs) is largely unstudied due to its rarity. The primary goal of this study was to characterize clinical features and outcomes of incidental NF-PNETs. METHODS: An institutional review board-approved retrospective study of patients with NF-PNET evaluated by the Surgical Oncology of University of Nebraska Medical Center was performed. Patients were evaluated with dedicated pancreatic and liver imaging using multiphasic computed tomographic scan and dedicated magnetic resonance imaging protocols...
December 2015: American Journal of Surgery
https://www.readbyqxmd.com/read/26438544/embryonal-tumors-with-multilayered-rosettes-in-children-the-sfce-experience
#12
Meryl Horwitz, Christelle Dufour, Pierre Leblond, Franck Bourdeaut, Cécile Faure-Conter, Anne-Isabelle Bertozzi, Marie Bernadette Delisle, Gilles Palenzuela, Anne Jouvet, Didier Scavarda, Matthieu Vinchon, Laetitia Padovani, Jean Gaudart, Dominique Figarella Branger, Nicolas Andre
PURPOSES: The purpose of this study was to retrospectively study embryonal tumors with multilayered rosettes (ETMR), a rare new entity that gathers ETAN-TR (embryonal tumor with abundant neuropil and true rosettes), ependymoblastomas, and medulloepitheliomas, in order to improve their descriptions and try to better define therapeutic modalities. METHODS: Patients with ETMR, ETAN-TR, ependymoblastoma, and medulloepithelioma treated in SFCE centres (Société Française de lutte contre les Cancers et les leucémies de l'Enfant et de l'adolescent) since 2000 were collected...
February 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/26318820/differentiation-between-pancreatic-metastases-from-renal-cell-carcinoma-and-hypervascular-neuroendocrine-tumour-use-of-relative-percentage-washout-value-and-its-clinical-implication
#13
Tae Wook Kang, Seong Hyun Kim, Jisun Lee, Ah Yeong Kim, Kyung Mi Jang, Dongil Choi, Min Ji Kim
PURPOSE: To compare computed tomography (CT) findings in patients with pancreatic metastasis from renal cell carcinoma (pRCC) and patients with hypervascular pancreatic neuroendocrine tumour (pNET) with a focus on the relative percentage washout (RPW). METHODS: We evaluated 16 patients with 37 pRCCs and 28 patients with 31 hypervascular pNETs using a protocol consisting of arterial and portal phase CT. Imaging findings were analyzed for comparison between the two groups...
November 2015: European Journal of Radiology
https://www.readbyqxmd.com/read/26275259/primary-ewing-sarcoma-of-vulva-confirmed-with-molecular-cytogenetic-analysis-a-rare-case-report-with-diagnostic-and-treatment-implications
#14
Bharat Rekhi, Girish Chinnaswamy, Tushar Vora, Sneha Shah, Venkatesh Rangarajan
Primary vulvar Ewing sarcoma (ES)/PNET is an uncommonly documented tumor, especially with molecular results. A 10-year-old girl presented with left vulvar swelling, a year ago. Her abdominopelvic ultrasound revealed a 12 cm × 8 cm sized, mixed echogenic blood-filled lesion in the left vulva; radiologically considered as a hematoma. Vulvectomy revealed a multinodular grey-brown tumor, microscopically comprising malignant round cells. Immunohistochemically, tumor cells diffusely expressed MIC2/CD99 and Fli1 and subsequently displayed EWSR1 rearrangement, confirming diagnosis of ES/PNET...
July 2015: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/26185384/efficacy-of-endoscopic-ultrasonography-guided-fine-needle-aspiration-for-pancreatic-neuroendocrine-tumor-grading
#15
COMPARATIVE STUDY
Mitsuru Sugimoto, Tadayuki Takagi, Takuto Hikichi, Rei Suzuki, Ko Watanabe, Jun Nakamura, Hitomi Kikuchi, Naoki Konno, Yuichi Waragai, Hiroyuki Asama, Mika Takasumi, Hiroshi Watanabe, Katsutoshi Obara, Hiromasa Ohira
AIM: To evaluate the efficacy of endoscopic ultrasonography-guided fine needle aspiration (EUS-FNA) for grading pancreatic neuroendocrine tumors (PNETs). METHODS: A total of 22 patients were diagnosed with PNET by EUS-FNA between October 2001 and December 2013 at Fukushima Medical University Hospital. Among these cases, we targeted 10 PNET patients who were evaluated according to the World Health Organization (WHO) 2010 classification. Surgery was performed in eight patients, and chemotherapy was performed in two patients due to multiple liver metastases...
July 14, 2015: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/25935542/streptozocin-5-fluorouracil-chemotherapy-is-associated-with-durable-response-in-patients-with-advanced-pancreatic-neuroendocrine-tumours
#16
Lisa-Marie Dilz, Timm Denecke, Ingo G Steffen, Vikas Prasad, Ludwig Fischer von Weikersthal, Ulrich-Frank Pape, Bertram Wiedenmann, Marianne Pavel
BACKGROUND: The role of systemic chemotherapy for pancreatic neuroendocrine tumours (pNET) is controversially discussed. Objective response rates (RR) reported for streptozocin (STZ)-based chemotherapy are variable and novel targeted drugs have recently been approved. However, the sequence of treatment remains unclear. We aimed to evaluate the efficacy of STZ plus 5-fluorouracil (STZ/5-FU) in a large pNET cohort. METHODS: Data from 96 pNET patients treated with STZ/5-FU were analysed retrospectively...
July 2015: European Journal of Cancer
https://www.readbyqxmd.com/read/25723725/neoadjuvant-chemotherapy-for-brain-tumors-in-infants-and-young-children
#17
Junya Iwama, Hideki Ogiwara, Chikako Kiyotani, Keita Terashima, Kentaro Matsuoka, Hideto Iwafuchi, Nobuhito Morota
OBJECT: Because of their large size and high vascularity, complete removal of brain tumors in infants and young children is often difficult. In most cases the degree of resection is associated with prognosis. Neoadjuvant chemotherapy may facilitate resection by reducing the vascularity of the tumor. The authors evaluated the effectiveness of neoadjuvant chemotherapy in the management of these tumors. METHODS: The authors performed a retrospective review of infants and young children who underwent tumor removal after neoadjuvant chemotherapy...
May 2015: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/25704363/outcome-and-prognostic-factors-for-children-with-supratentorial-primitive-neuroectodermal-tumors-treated-with-carboplatin-during-radiotherapy-a-report-from-the-children-s-oncology-group
#18
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
https://www.readbyqxmd.com/read/25697539/long-term-survival-in-a-case-of-etantr-with-histological-features-of-neuronal-maturation-after-therapy
#19
Manila Antonelli, Andrey Korshunov, Angela Mastronuzzi, Francesca Diomedi Camassei, Andrea Carai, Giovanna S Colafati, Stefan M Pfister, Marcel Kool, Felice Giangaspero
Embryonal tumor with abundant neuropil and true rosettes (ETANTR) is a rare subtype of primitive neuroectodermal tumors and one of the most aggressive brain tumors in children. The neoplasm harbors a specific genetic alteration, amplification of the miRNA cluster C19MC at 19q13.42. We report a case of a 21-month-old boy with a mass in the left fronto-opercular region. The lesion was partially resected and pathology examination revealed an ETANTR with immunoreativity for LIN28A protein and amplification of the C19MC locus...
May 2015: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/25688512/rationale-and-protocol-of-the-metnet-1-trial-a-prospective-single-center-phase-ii-study-to-evaluate-the-activity-and-safety-of-everolimus-in-combination-with-octreotide-lar-and-metformin-in-patients-with-advanced-pancreatic-neuroendocrine-tumors
#20
RANDOMIZED CONTROLLED TRIAL
Sara Pusceddu, Filippo de Braud, Laura Concas, Cristina Bregant, Livia Leuzzi, Barbara Formisano, Roberto Buzzoni
Abnormal PI3K-AKT-mTOR pathway signalling and autocrine activation of the mTOR pathway, mediated through insulin-like growth factor-1, have been implicated in the proliferation of pancreatic neuroendocrine tumor (pNET) cells. Everolimus, an mTOR inhibitor, has shown antitumor benefit in pNETs alone and in combination with octreotide LAR in RADIANT-1 and RADIANT-3 studies. Although everolimus-based phase II/III trials have improved progression-free survival for pNET, its use has not impacted on prolonging overall survival...
November 2014: Tumori
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