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https://www.readbyqxmd.com/read/28099344/laparoscopic-total-pancreatectomy-case-report-and-literature-review
#1
Xin Wang, Yongbin Li, Yunqiang Cai, Xubao Liu, Bing Peng
RATIONALE: Laparoscopic total pancreatectomy is a complicated surgical procedure and rarely been reported. This study was conducted to investigate the safety and feasibility of laparoscopic total pancreatectomy. PATIENTS AND METHODS: Three patients underwent laparoscopic total pancreatectomy between May 2014 and August 2015. We reviewed their general demographic data, perioperative details, and short-term outcomes. General morbidity was assessed using Clavien-Dindo classification and delayed gastric emptying (DGE) was evaluated by International Study Group of Pancreatic Surgery (ISGPS) definition...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28092495/nonhypervascular-pancreatic-neuroendocrine-tumors-differential-diagnosis-from-pancreatic-ductal-adenocarcinomas-at-mr-imaging-retrospective-cross-sectional-study
#2
Sun Kyung Jeon, Jeong Min Lee, Ijin Joo, Eun Sun Lee, Hyun Jeong Park, Jin-Young Jang, Ji Kon Ryu, Kyung Bun Lee, Joon Koo Han
Purpose To determine useful magnetic resonance (MR) imaging features to differentiate nonhypervascular pancreatic neuroendocrine tumors (PNETs) from pancreatic ductal adenocarcinomas (PDACs). Materials and Methods The institutional review board approved this retrospective study and waived the informed consent requirement. Seventy-four patients with surgically confirmed PNETs and 82 patients with PDACs who underwent gadobutrol-enhanced MR imaging were included. Two radiologists independently evaluated the morphologic characteristics and temporal enhancement patterns of each tumor...
January 13, 2017: Radiology
https://www.readbyqxmd.com/read/28050377/primary-pulmonary-primitive-neuro-ectodermal-tumour-pnet-in-an-eight-year-old-girl-a-rare-case
#3
Radhika Narayan, J Sreedevi, Farah Rana, Minakshi Mishra, Rajesh Mohanty
Primitive Neuro-Ectodermal-Tumours (PNET) and Ewing's sarcoma are part of the spectrum of Ewing's Family of Tumours (EFT) and show varying degrees of neuroectodermal differentiation. Both these tumours share similar histological and genetic features. PNETs arising primarily in the lungs without pleural or chest wall involvement are extremely rare. We report a case of pulmonary PNET in an eight-year-old girl. To the best of our knowledge, this is the youngest case of primary pulmonary PNET to be reported in paediatric age group in the Indian literature...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28043759/baseline-plasma-chromogranin-a-levels-in-patients-with-well-differentiated-neuroendocrine-tumors-of-the-pancreas-a-potential-predictor-of-postoperative-recurrence
#4
Yoshihide Nanno, Hirochika Toyama, Ippei Matsumoto, Kyoko Otani, Sadaki Asari, Tadahiro Goto, Tetsuo Ajiki, Yoh Zen, Takumi Fukumoto, Yonson Ku
BACKGROUND: The present study aimed to elucidate prognostic values of baseline plasma chromogranin A (CgA) concentrations in patients with resectable, well-differentiated pancreatic neuroendocrine tumors (PNETs). METHODS: Preoperative CgA levels in 21 patients with PNET were correlated with clinicopathological factors and patients' survival. RESULTS: Plasma CgA levels ranged 2.9-30.8 pmol/mL (median 6.0), and were significantly elevated in patients with post-operative recurrence (P = 0...
December 29, 2016: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/28039368/long-term-neurologic-health-and-psychosocial-function-of-adult-survivors-of-childhood-medulloblastoma-pnet-a-report-from-the-childhood-cancer-survivor-study
#5
Allison A King, Kristy Seidel, Chongzhi Di, Wendy M Leisenring, Stephanie Mabry Perkins, Kevin R Krull, Charles A Sklar, Daniel M Green, Gregory T Armstrong, Lonnie K Zeltzer, Elizabeth Wells, Marilyn Stovall, Nicole J Ullrich, Kevin C Oeffinger, Leslie L Robison, Roger J Packer
BACKGROUND: Medulloblastoma is the most common malignant childhood brain tumor, although long-term risks for chronic neurologic health and psychosocial functioning in aging adult survivors are incompletely characterized. METHODS: The Childhood Cancer Survivor Study (CCSS) includes 380 five-year survivors of medulloblastoma/primitive neuroectodermal tumor (PNET; median age at follow-up: 30 y, interquartile range 24-36) and sibling comparison (n = 4031). Cumulative incidence of neurologic health conditions was reported...
December 29, 2016: Neuro-oncology
https://www.readbyqxmd.com/read/28035372/surgery-management-for-sporadic-small-%C3%A2-2-cm-non-functioning-pancreatic-neuroendocrine-tumors-a-consensus-statement-by-the-chinese-study-group-for-neuroendocrine-tumors-csnet
#6
Guang Yang, Meng Ji, Jie Chen, Rufu Chen, Ye Chen, Deliang Fu, Baohua Hou, Heguang Huang, Liming Jiang, Kaizhou Jin, Nengwen Ke, Ying Li, Yong Li, Houjie Liang, An'an Liu, Jie Luo, Quanxing Ni, Chengwei Shao, Boyong Shen, Weiqi Sheng, Bin Song, Jian Sun, Chunlu Tan, Huangying Tan, Qiyun Tang, Yingmei Tang, Xiaodong Tian, Jian Wang, Jie Wang, Wei Wang, Wei Wang, Zheng Wu, Jin Xu, Qiang Yan, Ning Yang, Yinmo Yang, Xiaoyu Yin, Xianjun Yu, Chunhui Yuan, Shan Zeng, Guochao Zhang, Renchao Zhang, Zhiwei Zhou, Zhaohui Zhu, Chenghao Shao
The incidence of small (≤2 cm), non-functioning pancreatic neuroendocrine tumors (NF-pNETs) increased in the last decades. Before making appropriate strategy for patients with NF-pNETs ≤2 cm, pathological confirmation is vital. Incidentally diagnosed, sporadic small NF-pNETs may bring aggressive behavior and poor prognosis, such as extrapancreatic extension, lymph nodal metastasis, distant metastasis and recurrence, even causing disease-related death. Understanding and formulating an appropriate strategy for the patients with sporadic small, non-functioning pancreatic neuroendocrine tumors have been controversial for some time...
February 2017: International Journal of Oncology
https://www.readbyqxmd.com/read/28029420/early-dural-metastasis-from-a-case-of-glioblastoma-with-primitive-neuroectodermal-differentiation-a-case-report-and-literature-review
#7
Subhas K Konar, Shyamal C Bir, Tanmoy K Maiti, Devi Prasad Patra, Angela C DiPoto Brahmbhatt, Jamie A Jacobsohn, Anil Nanda
Glioblastoma with a primitive neuroectodermal (PNET) variant is a rare primary parenchymal tumor. Only a few cases of extraparenchymal metastasis are reported in world literature. Although the overall survival duration of glioblastoma multiforme (GBM) with primitive neuroectodermal tumor (PNET) variety may be prolonged in comparison to classical glioblastoma, the metastatic trend is completely different, and the prognosis is worse. We report an early dural metastasis of pure PNET component appearing in a case of primary glioblastoma with PNET variant...
January 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28008994/common-germline-variants-within-the-cdkn2a-2b-region-affect-risk-of-pancreatic-neuroendocrine-tumors
#8
Daniele Campa, Gabriele Capurso, Manuela Pastore, Renata Talar-Wojnarowska, Anna Caterina Milanetto, Luca Landoni, Evaristo Maiello, Rita T Lawlor, Ewa Malecka-Panas, Niccola Funel, Maria Gazouli, Antonio De Bonis, Harald Klüter, Maria Rinzivillo, Gianfranco Delle Fave, Thilo Hackert, Stefano Landi, Peter Bugert, Franco Bambi, Livia Archibugi, Aldo Scarpa, Verena Katzke, Christos Dervenis, Valbona Liço, Sara Furlanello, Oliver Strobel, Francesca Tavano, Daniela Basso, Rudolf Kaaks, Claudio Pasquali, Manuel Gentiluomo, Cosmeri Rizzato, Federico Canzian
Pancreatic neuroendocrine tumors (PNETs) are heterogeneous neoplasms which represent only 2% of all pancreatic neoplasms by incidence, but 10% by prevalence. Genetic risk factors could have an important role in the disease aetiology, however only a small number of case control studies have been performed yet. To further our knowledge, we genotyped 13 SNPs belonging to the pleiotropic CDKN2A/B gene region in 320 PNET cases and 4436 controls, the largest study on the disease so far. We observed a statistically significant association between the homozygotes for the minor allele of the rs2518719 SNP and an increased risk of developing PNET (ORhom = 2...
December 23, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27994516/genes-involved-in-angiogenesis-and-mtor-pathways-are-frequently-mutated-in-asian-patients-with-pancreatic-neuroendocrine-tumors
#9
Wen-Chi Chou, Po-Han Lin, Yi-Chen Yeh, Yi-Ming Shyr, Wen-Liang Fang, Shin-E Wang, Chun-Yu Liu, Peter Mu-Hsin Chang, Ming-Han Chen, Yi-Ping Hung, Chung-Pin Li, Yee Chao, Ming-Huang Chen
Introduction: To address the issue of limited data on and inconsistent findings for genetic alterations in pancreatic neuroendocrine tumors (pNETs), we analyzed sequences of known pNET-associated genes for their impact on clinical outcomes in a Taiwanese cohort. Methods: Tissue samples from 40 patients with sporadic pNETs were sequenced using a customized sequencing panel that analyzed 43 genes with either an established or potential association with pNETs. Genetic mutations and clinical outcomes were analyzed for potential associations...
2016: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/27975336/growth-rate-of-small-pancreatic-neuroendocrine-tumors-in-multiple-endocrine-neoplasia-type-1-results-from-an-endoscopic-ultrasound-based-cohort-study
#10
Wouter F W Kappelle, Gerlof D Valk, Max Leenders, Leon M G Moons, Auke Bogte, Peter D Siersema, Frank P Vleggaar
Background and aims In multiple endocrine neoplasia type 1 (MEN1), endoscopic ultrasound (EUS) is used for identification and follow-up of pancreatic neuroendocrine tumors (PNETs). The role of EUS in surveillance of small ( < 20 mm) PNETs is unclear, mostly because the natural course of these lesions is largely unknown. We aimed to determine annual growth and incidence rate of small PNETs in patients with MEN1 using EUS-based surveillance. Patients and methods Linear array EUS procedures in patients with MEN1 between 2002 and 2015 were identified...
January 2017: Endoscopy
https://www.readbyqxmd.com/read/27974690/benefit-of-sunitinib-in-the-treatment-of-pulmonary-primitive-neuroectodermal-tumors-a-case-report-and-literature-review
#11
Chunhui Zhang, Jingchun Zhang, Guangyu Wang, Jiajia Xu, Yanlin Li, Qing Guo, Tongsen Zheng, Yanqiao Zhang
Primitive neuroectodermal tumor (PNET) is a highly aggressive small round celltumor but is extremely rare in the lung. Next-generation sequencing (NGS) has led to breakthroughs for genetic analyses and personalizedmedicine approaches for cancer treatment.We report the case of a 30-year-old woman with an advanced pulmonary PNET treated with multiple chemotherapeutic regimens, and achieved a partial response (PR) as a best response. However, there was a disease progression after these treatment regimens.The NGS revealed the presence of a copy number loss (CNL) of Von Hippel-Lindau (VHL), CDKN2A/B and TP53 genes...
December 10, 2016: Oncotarget
https://www.readbyqxmd.com/read/27965134/national-rise-of-primary-pancreatic-carcinoid-tumors-comparison-to-functional-and-non-functional-pancreatic-neuroendocrine-tumors
#12
Gyulnara G Kasumova, Omidreza Tabatabaie, Mariam F Eskander, Abhishek Tadikonda, Sing Chau Ng, Jennifer F Tseng
BACKGROUND: New guidelines recommend differentiating between carcinoid and pancreatic neuroendocrine tumors (PNETs) during clinical trials. However, little is known about the behavior and incidence of primary pancreatic carcinoid tumors. STUDY DESIGN: Retrospective cohort study utilizing the National Cancer Data Base (NCDB) to identify adults with primary PNETs diagnosed between 2004 and 2013. The Kaplan-Meier method was used to evaluate overall survival and multivariate Cox proportional hazards model was used to assess the hazard of death in resected patients...
December 10, 2016: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/27942928/efficacy-and-safety-of-everolimus-and-sunitinib-in-patients-with-gastroenteropancreatic-neuroendocrine-tumor
#13
Changhoon Yoo, Hyungwoo Cho, Min Jeong Song, Seung-Mo Hong, Kyu-Pyo Kim, Heung-Moon Chang, Heejung Chae, Tae Won Kim, Yong Sang Hong, Min-Hee Ryu, Yoon-Koo Kang, Song Cheol Kim, Baek-Yeol Ryoo
PURPOSE: Efficacy of targeted agents, such as everolimus and sunitinib, has been demonstrated in prospective trials on patients with gastroenteropancreatic neuroendocrine tumors (GEP-NETs). Considering the heterogeneous clinicopathological characteristics of neuroendocrine tumors (NETs), evaluation of treatment outcomes in a real-world setting is necessary. METHODS: Clinical records of 44 patients with GEP-NET who were treated with everolimus or sunitinib between March 2007 and October 2014 were retrospectively reviewed...
December 10, 2016: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/27928440/a-rare-case-of-metastasized-non-functional-pancreatic-neuroendocrine-tumor-with-a-good-long-term-survival
#14
Mirică A, Bădărău Ia, Mirică R, Păun S, Păun Dl
Background: Non-functional neuroendocrine tumors of the pancreas (NF-pNETs) are a varied group of extremely rare malignancies. The majority of patients already have liver metastases at the diagnosis moment, thus, treatment options are restricted, and the survival rate is reserved. Case report: We presented the case of 59-year-old patient, diagnosed with non-functional well-differentiated pancreatic neuroendocrine tumor grade II (NET G2) with the presence of chromogranin A, synaptophysin and somatostatin receptor 2, together with liver and bone metastases...
October 2016: Journal of Medicine and Life
https://www.readbyqxmd.com/read/27926987/impact-of-everolimus-on-japanese-patients-with-advanced-pancreatic-neuroendocrine-neoplasms
#15
Lingaku Lee, Tetsuhide Ito, Hisato Igarashi, Keijiro Ueda, Takashi Fujiyama, Ken Kawabe, Yoshihiro Ogawa
BACKGROUND: Although everolimus has become a key therapeutic agent in patients with advanced pancreatic neuroendocrine neoplasms (PNEN), its efficacy and safety in clinical practice remains unclear. METHODS: Forty-seven patients with advanced PNEN treated with everolimus were reviewed retrospectively. To evaluate the safety of everolimus as a long-term treatment, the patients were divided into 2 groups according to treatment duration: group A, ≤1 year (n = 21); group B, >1 year (n = 26)...
December 7, 2016: Journal of Hepato-biliary-pancreatic Sciences
https://www.readbyqxmd.com/read/27923222/pediatric-primary-diffuse-leptomeningeal-primitive-neuroectodermal-tumor-a-case-report-and-literature-review
#16
Jesna Mathew Sublett, Caitlin Davenport, Howard Eisenbrock, Shamsher Dalal, Syed A Jaffar Kazmi, Amir Kershenovich
BACKGROUND: Primary diffuse leptomeningeal primitive neuroectodermal tumor (PDL PNET) is extremely rare, with only 19 cases reported in the literature to date. We present a case of a child with rapidly progressive PDL PNET and a literature review. A 10-year-old boy presented with mood lability, hallucinations, generalized pain, enuresis, and headaches. Initial investigation failed to produce a diagnosis. The symptoms progressed to seizure, back pain, and papilledema. Imaging showed acute hydrocephalus and mild diffuse leptomeningeal enhancement without an identifiable primary lesion...
7, 2016: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27920700/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-stomach
#17
Safi Khuri, Hayim Gilshtein, Sa'd Sayidaa, Bishara Bishara, Yoram Kluger
Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation). These findings were compatible with gastric ES/PNET...
September 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27919713/primary-primitive-neuroectodermal-tumor-arising-from-an-ovarian-mature-cystic-teratoma-in-a-12-year-old-girl-a-case-report
#18
Ashley Jaramillo-Huff, Rania Bakkar, Jason Q McKee, Nancy Sokkary
BACKGROUND: Ovarian mature cystic teratomas (MCTs) rarely transform to primary primitive neuroectodermal tumors. This case report offers evidence that MCTs might have undetected microfoci of malignant neural tumors. CASE: We describe the case of a 12-year-old girl who presented with right-sided abdominal pain and distention. Intraoperative findings revealed a right ovarian MCT. However, pathology showed a 0.5-cm focus of malignant neural tumor within the 11-cm MCT...
December 2, 2016: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/27919009/successful-percutaneous-ct-guided-microwave-ablation-of-adrenal-gland-for-ectopic-cushing-syndrome
#19
Rebecca Zener, Andrew Zaleski, Stan H Van Uum, Daryl K Gray, Amol Mujoomdar
Adrenocorticotropic hormone production by pancreatic neuroendocrine tumor (PNET) is rare and results in hyperstimulation of the adrenal gland to produce ectopic Cushing syndrome. Our case showcases the safety and effectiveness of percutaneous CT-guided microwave ablation of the adrenal gland in a 49-year-old female with PNET and hepatic metastases who presented with ectopic Cushing syndrome despite surgical resection of the primary pancreatic tumor and left adrenal gland. Prior to ablation, the right adrenal gland measured 4...
November 29, 2016: Clinical Imaging
https://www.readbyqxmd.com/read/27916439/adult-supratentorial-primitive-neuroectodermal-tumour-presenting-as-intracranial-haemorrhage-case-report
#20
Sean P Black-Tiong, Simon J I Sandler, Sophia Otto, Adam J Wells
Primitive neuroectodermal tumours (PNET) are highly malignant tumours with an aggressive clinical behaviour. Commonly seen in children, they are uncommon in the adult population, and rare in the supratentorial location. Adult supratentorial PNETs (ST-PNET) typically present with symptoms relating to raised intracranial pressure, seizures, or focal neurological deficits. Presentation with intracranial haemorrhage has been reported only twice before in the literature, one of which was fatal. We report the case of intracranial haemorrhage secondary to ST-PNET in a young adult and her immediate management...
December 1, 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
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