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Maryam Abolhasani, Sareh Salarinejad, Mohammad Kazem Moslemi
INTRODUCTION: Ewing sarcoma/Primitive neuroectodermal tumor of the kidney (ES/PNET) is a member of Ewing's sarcoma family, occurring in young adults and has aggressive clinical behavior and poor prognosis. However, its discrimination from the renal cell carcinoma (RCC) is very difficult preoperatively. We present three cases of this rare disease that were managed in two academic centers. PRESENTATION OF CASES: Herein we report three cases of ES/PNET of the kidney, 2 young men complaining of right flank pain and gross hematuria and one young woman complaining of left subcostal pain...
October 18, 2016: International Journal of Surgery Case Reports
Hideki Ushiku, Keishi Yamashita, Hiroshi Kawamata, Mina Waraya, Hiroshi Katoh, Keigo Yokoi, Toshimichi Tanaka, Satoru Ishii, Nobuyuki Nishizawa, Mariko Kikuchi, Naoko Minatani, Ken Kojo, Hiroshi Tajima, Ryo Nishiyama, Takashi Kaizu, Yusuke Kumamoto, Masahiko Watanabe
OBJECTIVES: We have identified homeobox-only protein (HOPX) as a tumor suppressor gene in various human cancer, and its expression was reduced by promoter DNA hypermethylation. Homeobox-only protein is strongly expressed on pancreatic islet cells; however, clinical relevance of HOPX expression has remained elusive in pancreatic neuroendocrine tumor (pNET). METHODS: We investigated 36 patients with pNET who undertook surgical resection between 1988 and 2012 for HOPX expression and DNA methylation to reveal its clinical significance...
October 2016: Pancreas
Janez Cernelc
No abstract text is available yet for this article.
February 2016: Pathology
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
Ersin Özaslan, Halit Karaca, Sinan Koca, Alper Sevinç, Bekir Hacioğlu, Metin Özkan, Melike Özçelik, Ayşe O Duran, İlhan Hacibekiroğlu, Yaşar Yildiz, Özgür Tanriverdi, Serkan Menekşe, Asude Aksoy, Oktay Bozkurt, Semiha Urvay, Mükremin Uysal, Hacer Demir, Aydin Çiltaş, Faysal Dane
The objectives of this study were to compare progression-free survival (PFS) with somatostatin analog (SSA) versus chemotherapy (CTx) in first-line therapy and to determine the patient group in which these treatments were more effective in neuroendocrine tumors (NETs) with a Ki-67 index of 20% or less. Patients who received SSA or CTx and had unresectable locally advanced and metastatic NETs with a Ki-67 index of 20% or less were retrospectively selected from 13 centers in the Turkish database between 2000 and 2015...
October 20, 2016: Anti-cancer Drugs
Nancy Sharma, Boris G Naraev, Eric G Engelman, M Bridget Zimmerman, David L Bushnell, Thomas M OʼDorisio, M Sue OʼDorisio, Yusuf Menda, Jan Müller-Brand, James R Howe, Thorvardur R Halfdanarson
OBJECTIVES: The objective of this study was to describe the outcomes of patients in the University of Iowa Neuroendocrine Tumor (NET) Database treated with peptide receptor radionuclide therapy (PRRT). METHODS: One hundred thirty-five patients from the University of Iowa NET Database who received PRRT were analyzed, their characteristics were described, and survival was calculated. RESULTS: The median age at diagnosis was 51 years, and 64% were men...
October 18, 2016: Pancreas
Afshin Pourmokhtarian, Charles T Driscoll, John L Campbell, Katharine Hayhoe, Anne M K Stoner
Assessments of future climate change impacts on ecosystems typically rely on multiple climate model projections, but often utilize only one downscaling approach trained on one set of observations. Here, we explore the extent to which modeled biogeochemical responses to changing climate are affected by the selection of the climate downscaling method and training observations used at the montane landscape of the Hubbard Brook Experimental Forest, New Hampshire, USA. We evaluated three downscaling methods: the delta method (or the change factor method), monthly quantile mapping (Bias Correction-Spatial Disaggregation, or BCSD), and daily quantile regression (Asynchronous Regional Regression Model, or ARRM)...
July 2016: Ecological Applications: a Publication of the Ecological Society of America
Surbhi Goyal, Kiran Mishra, Urvee Sarkar, Satendra Sharma, Anita Kumari
BACKGROUND & OBJECTIVES: Renal tumours constitute about 7 per cent of all neoplasms in children. It is important to differentiate Wilms' tumour (commonest tumour) from non-Wilms' tumours. The aim of this study was to evaluate the immunoexpression and diagnostic role of Wilms' tumour-1 protein (WT1) in paediatric renal tumours. METHODS: A total of 53 cases of renal tumours in children (below 18 yr) who underwent total nephrectomy were included in this retrospective study...
May 2016: Indian Journal of Medical Research
Rie Tadokoro, Shotaro Sato, Fumiko Otsuka, Makoto Ueno, Shinichi Ohkawa, Hideki Katakami, Matsuo Taniyama, Shoichiro Nagasaka
The patient was a 61-year-old woman who had a well-differentiated pancreatic neuroendocrine tumor (PNET) with lymph node metastasis. After 15 months of octreotide treatment, glucose control deteriorated and pigmentation of the tongue and moon face developed, leading to the diagnosis of ectopic adrenocorticotropic hormone (ACTH) syndrome. An abnormal secretion of growth hormone (GH) was identified, and the plasma growth hormone-releasing hormone (GHRH) level was elevated. A tumor biopsy specimen positively immunostained for ACTH and GHRH...
2016: Internal Medicine
Jessica E Maxwell, Scott K Sherman, James R Howe
Pancreatic neuroendocrine tumors (PNET) are rare tumors, but have been increasing in incidence. Although typically thought of as indolent, more than half of patients present with metastatic disease. For many years, the only mutations commonly known in these tumors were those in the MEN1 gene. Recently, the genetics underlying PNETs have been further defined through exome sequencing. The most frequent alterations found in sporadic PNETs are in MEN1, DAXX/ATRX, and a variety of genes in the mTOR pathway. Confirmation of these mutations has prompted trials with a number of drugs active in these pathways, and two drugs were eventually approved in 2011-sunitinib and everolimus...
October 15, 2016: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
E Bertani, N Fazio, D Radice, C Zardini, G Spinoglio, A Chiappa, D Ribero, R Biffi, S Partelli, M Falconi
BACKGROUND: The role of primary tumour surgery in pancreatic neuroendocrine tumours (PNETs) with unresectable liver metastases is controversial and international guidelines do not recommend surgery in such cases. Resectability of the primary tumour has never been considered in outcome comparisons between operated and non-operated patients. METHODS: From two institutional prospective databases of patients affected by PNET and unresectable liver metastases, 63 patients who underwent a left-pancreatectomy at diagnosis were identified and compared with a group of 30 patients with a potentially resectable but not-resected primary tumour located in the body or tail...
September 30, 2016: European Journal of Surgical Oncology
Avas Chandra Ray, Subhra Aditya, Pulak Kumar Jana, Apratim Chatterjee, Anup Sarkar, Jay Mehta, Jotideb Mukhopadhyay
A young male labourer developed pain at the site of blunt trauma over back of chest followed by fever, cough with expectoration, breathlessness and hemorrhagic pleural effusion in the side of injury. What could have been passed as a sequel of trauma turned out to be the consequences of an underlying rare and aggressive malignant tumor of the chest wall known as Askin tumor or Primitive Neuroectodermal Tumor (PNET). CT thorax with guided FNAC, debulking operation, histopathological examination followed by immunohistochemistry of the tumor tissue led to the final diagnosis...
March 2016: Journal of the Association of Physicians of India
Young-Bem Se, Dong Gyu Kim, Sung Kwon Kim, Jin Wook Kim, Yong Hwy Kim, Chul-Kee Park, Hyun-Tai Chung, Sun-Ha Paek
BACKGROUND: Supratentorial primitive neuroectodermal tumors (PNETs) are highly malignant and rare tumors of the central nervous system. OBJECTIVES: The aim of this study was to determine the role of Gamma Knife surgery (GKS) as a salvage treatment option for patients with recurrent or residual supratentorial PNETs. METHODS: Between 1998 and 2014, 11 patients with supratentorial PNETs were retrospectively analyzed. This series consisted of 7 male and 4 female patients...
October 8, 2016: Stereotactic and Functional Neurosurgery
Claudius Conrad, Onur C Kutlu, Arvind Dasari, Jennifer A Chan, Jean-Nicolas Vauthey, David B Adams, Michael Kim, Jason B Fleming, Matthew H G Katz, Jeffrey E Lee
BACKGROUND: The impact of lymph node (LN) status and lymphadenectomy (LA) on survival in pancreatic neuroendocrine tumors (pNETs) remains controversial. We evaluated the impact of tumor extension and grade on nodal metastasis and survival. METHODS: Surgical pNET patients were queried in the Surveillance Epidemiology and End Results (SEER) database (1998-2012, N = 981). Factors associated with LN status were analyzed by logistic regression and by Cox analyses...
October 6, 2016: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
Fara Uccelli, F Gavazzi, G Capretti, M Virdis, M Montorsi, A Zerbi
Enucleation (EN) and middle pancreatectomy (MP) have been proposed as a treatment for G1 and G2 pancreatic neuroendocrine tumors (PNET). The aim of this study is to analyze the outcomes of parenchyma-sparing surgery (PSS) for PNET in an Italian high-volume center. All patients with a histological diagnosis of PNET who underwent surgical resection in our center between January 2010 and January 2016 were included in the study. Demographic, perioperative, and discharge data were collected in a prospective database...
October 5, 2016: Updates in Surgery
Ivan Zokalj, Jasminka Igrec, Antonio Plesnar
INTRODUCTION: Primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (EWS) belongs to a family of neoplasms that are presumed to originate from the neuroectodermal crest. PNET/EWSs are highly aggressive malignancies that usually present in the form of bone or soft tissue masses and usually affect adolescents and young adults. Primary PNET/EWS of the kidney is very rare. CASE PRESENTATION: We present the case of a 32-year-old female patient with primary renal PNET/EWS diagnosed nine years and eight months earlier...
July 2016: Nephro-urology Monthly
Javier A Cienfuegos, Fernando Rotellar, Joseba Salguero, Miguel Ruiz-Canela, Jorge M Núñez Córdoba, Iosu Sola, Alberto Benito, Gabriel Zozaya, F Pardo, José Luis Hernández Lizoáin
BACKGROUND: Pancreatic neuroendocrine tumors (pNETs) comprise a heterogeneous group of tumors with a varied biological behavior. In the present study, we analyzed the experience of 79 pNETs resected between 1999 and 2014. The pathologic prognostic factors (European Neuroendocrine Tumor Society, ENETS; and AJCC) classification, vascular invasion (VI), proliferation index (ki-67) and the presence of necrosis were retrospectively reviewed. METHODS: The clinical data of 79 patients with pNETs who underwent surgery were retrospectively analyzed...
October 5, 2016: Revista Española de Enfermedades Digestivas
Xin Jin, Jianfeng Cao, Yong Liu, Fang Bian, Qingqing Zhao, Yan Wang, Xu Lv, Yayong Huang
Primitive neuroectodermal tumors (PNETs) are small, round cell tumors that may be classified as peripheral or central, based on their site of origin. PNETs often arise in the soft tissue or bone of young adults. Although not common, PNETs have been described in other organs, including the gonads, kidneys, myocardium and pancreas, but rarely in the lungs without chest wall or pleural involvement. The present study reports a rare case of peripheral PNET (pPNET), which originated in the lung. A 37-year-old female patient presented at Xuzhou Central Hospital (Xuzhou, China) with a history of a dry cough, mild dyspnea and slight pain in the left chest...
October 2016: Oncology Letters
Raffaele Manta, Elisabetta Nardi, Nico Pagano, Claudio Ricci, Mariano Sica, Danilo Castellani, Helga Bertani, Micaela Piccoli, Barbara Mullineris, Alberto Tringali, Francesco Marini, Ugo Germani, Vincenzo Villanacci, Riccardo Casadei, Massimiliano Mutignani, Rita Conigliaro, Gabrio Bassotti, Angelo Zullo
BACKGROUND AND AIMS: Diagnosis of pancreatic neuroendocrine tumors (p-NETs) is frequently challenging. We describe a large series of patients with p-NETs in whom both pre-operative Computed Tomography (CT) and Endoscopic Ultrasonography (EUS) were performed. METHODS: This was a retrospective analysis of prospectively collected sporadic p-NET cases. All patients underwent both standard multidetector CT study and EUS with fine-needle aspiration (FNA). The final histological diagnosis was achieved on a post-surgical specimen...
September 2016: Journal of Gastrointestinal and Liver Diseases: JGLD
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 PNET 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. Furthermore, a persistently high neoantigen load was observed within all tumors...
September 28, 2016: Cancer Discovery
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