• Title/Summary/Keyword: PNET

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Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumor in the Cerebellopontine Angle : Diagnosis and Treatment

  • Choi, Ho-Yong;Kim, Yong-Hwy;Kim, Jee-Hyun;Kim, In-Ah;Choe, Ghee-Young;Kim, Chae-Yong
    • Journal of Korean Neurosurgical Society
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    • v.49 no.6
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    • pp.359-362
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    • 2011
  • Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an unusual malignancy with aggressive behavior. ES/PNET in the cerebellopontine angle (CPA) is extremely uncommon, and we report on a rare case here. A 31-year-old man presented with one month history of left facial palsy, hearing loss, swallowing difficulty, and hoarseness. Magnetic resonance images showed a large mass in the left CPA and a small one in the right cerebellar hemisphere. The patient underwent a surgery for the CPA mass lesion, and the pathology was compatible with ES/PNET. Radiation therapy and chemotherapy were administered. In contrast to the initial radiologic findings resembling vestibular schwannoma or meningioma, ES/PNET had several distinct clinical features. A patient with a CPA mass and presenting unusual clinical features should be suspected of having a rare malignancy.

Ewing's Sarcoma/PNET of the Talus - Report of a Case - (거골에서 발생한 유윙 육종/원시신경 외배엽종양 - 1예 보고 -)

  • Kim, Sun-Young;Kwon, Hyuck-Po;Roh, Jae-Su;Cho, Hyoun-Oh
    • The Korean Journal of Cytopathology
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    • v.15 no.2
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    • pp.120-125
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    • 2004
  • Ewing's sarcoma (ES)/PNET is common in both axial and appendicular skeletons, but is extremely rare in the talus. Here, we report a case of ES/PNET of the left talus in a 29-year-old male patient diagnosed by fine needle aspiration cytology, and review the literature on similar cases. The cytological smears were composed of individually dispersed small round cells and occasional clusters of loosely cohesive cells. The tumor cells were fragile, frequently exhibiting naked nuclei. Two distinct types of cells were observed. The light (chief) cells displayed round or slightly oval nuclei with frequent indentations, generally inconspicuous nucleoli, and a thin rim of cytoplasm, which sometimes harbored small vacuoles. The dark cells were smaller, displaying scanty cytoplasm with dense hyperchromatic nuclei, intermixed with chief cells, and often manifesting as small molded groups. However, no significant nuclear pleomorphisms or mitoses were noted. Tumor cells in the ceil block revealed positive cytoplasmic glycogen, as determined by a PAS stain with diastase control, and also exhibited positive immunoreactivity for CD99.

Reliability Analysis of Multiple Failure Modes of Rubble-Mound Breakwaters (경사제의 다중 파괴모드에 대한 신뢰성 해석)

  • Lee, Cheol-Eung
    • Journal of Korean Society of Coastal and Ocean Engineers
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    • v.20 no.2
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    • pp.137-147
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    • 2008
  • A reliability analysis has been performed to investigate the systematic stability of multi-failure modes of rubble-mound breakwaters. The reliability functions of four different failure modes are established straightforwardly. AFDA(Approximate Full Distribution Approcah) reliability models for each failure modes are directly developed and satisfactorily calibrated through the comparison with CIAD's results. In the reliability analysis of single failure mode, the probabilities of failure are calculated and the influence coefficients of random variables in the failure modes are properly evaluated. Meanwhile, three different models such as uni-modal bounds, bimodal bounds, and PNET are applied to evaluate the probabilities of failure of multi-failure modes for rubble-mound breakwaters. It may be found that uni-modal bounds tend to overestimate the probability of failure of multi-failure modes. Therefore, for the systematic reliability analysis of multi-failure modes, it is recommended to use bi-modal bounds or PNET which consider the correlation between the failure modes for rubble-mound breakwaters. By introducing the reliability analysis of multi-failure modes, it could be possible to find out the additional probabilities of failure occurred by the multi-failure modes of a multi-component system such as rubble-mound breakwaters.

Receiver Operating Characteristic Curve Analysis of SEER Medulloblastoma and Primitive Neuroectodermal Tumor (PNET) Outcome Data: Identification and Optimization of Predictive Models

  • Cheung, Min Rex
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.16
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    • pp.6781-6785
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    • 2014
  • Purpose: This study used receiver operating characteristic curves to analyze Surveillance, Epidemiology and End Results (SEER) medulloblastoma (MB) and primitive neuroectodermal tumor (PNET) outcome data. The aim of this study was to identify and optimize predictive outcome models. Materials and Methods: Patients diagnosed from 1973 to 2009 were selected for analysis of socio-economic, staging and treatment factors available in the SEER database for MB and PNET. For the risk modeling, each factor was fitted by a generalized linear model to predict the outcome (brain cancer specific death, yes/no). The area under the receiver operating characteristic curve (ROC) was computed. Similar strata were combined to construct the most parsimonious models. A Monte Carlo algorithm was used to estimate the modeling errors. Results: There were 3,702 patients included in this study. The mean follow up time (S.D.) was 73.7 (86.2) months. Some 40% of the patients were female and the mean (S.D.) age was 16.5 (16.6) years. There were more adult MB/PNET patients listed from SEER data than pediatric and young adult patients. Only 12% of patients were staged. The SEER staging has the highest ROC (S.D.) area of 0.55 (0.05) among the factors tested. We simplified the 3-layered risk levels (local, regional, distant) to a simpler non-metastatic (I and II) versus metastatic (III) model. The ROC area (S.D.) of the 2-tiered model was 0.57 (0.04). Conclusions: ROC analysis optimized the most predictive SEER staging model. The high under staging rate may have prevented patients from selecting definitive radiotherapy after surgery.

Formation of Brain Tumor and Lymphoma by Deregulation of Apoptosis Related Gene Expression in VP-SV40 T Ag Transgenic Mice

  • Lee, Jeong-Woong;Lee, Eun-Ju;Lee, Hoon-Taek;Chung, Kil-Saeng;Ryoo, Zae-Yoong
    • Proceedings of the KSAR Conference
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    • 2001.03a
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    • pp.47-47
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    • 2001
  • The neuropeptide vasopressin (VP) is a nine- amino acid hormone synthesized as preprohormone in the cell bodies of hypothalamic magnocellular neurons. The tumor in magnocellular neurons of the hypothalamus is associated with disfunctions of the cell bodies, leading to the diabetes insipidus. In order to study with the diabetes insipidus caused by a defect in VP synthesis and its secretion, we have produced the transgenic mice regulated by vasopressin promoter inserted to SV40 T antigen coding sequence (pVPSV.IGR2.1). One transgenic line expressing high levels of SV40 T antigen was propagated. The founder and all transgene positive adult animals have appeared with shorten mortality or apparent phenotypic abnormalities, including immune complex disease, and eventually die between 4 and 8 months of age. The mRNA and protein of SV40T antigen transgene were detected in brain of fetus as well as in brain, spleen, lung and lymph node in moribund at the age of 20 weeks. Histological analysis of transgenic mice showed that tumor developed in brain similar to primitive neuroectodermal tumors (PNET) in man. We also detected lymphomas in spleen and lymph node, and consequent tumor formation in various tissues of the transgenic mice. In pVPSV.IGR2.1, 21% mice showed brain tumor (PNET) at 5 weeks and 100% mice showed brain tumor after 15 weeks. In addition, Expression of apoptosis related genes (Bcl-28 & Bax) was increased over their age in mice with PNET as compared to control mice. Apoptosis related gene expression might be deregulated in mice with brain tumor. However, transgenic mice were not developed with the diabetes insipidus. These mice represent the first disease model to exhibit primitive neuroectodermal tumor in brain, as well as a unique model system for exploring the cellular pathogenesis of lymphomas.

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Primitive Neuroectodermal Tumor of the Liver in a 13-year-old Boy: A Case Report (염증성 거짓 종양으로 오인된 간 내 발생한 원시신경외배엽종양)

  • Lee, Sang-Goo;Kim, Ji-Eun;Lee, Ji-Hyuk;Lee, Hye-Jin;Lee, Jong-Seung;Lee, Jee-Hyun;Choe, Yon-Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.2
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    • pp.214-218
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    • 2008
  • Primary primitive neuroectodermal tumor (PNET) of the liver is a rare disease with aggressive behavior and poor prognosis. We report a case of a PNET of the liver in a 13-year-old boy. The patient was admitted to the hospital with fever and abdominal pain. Abdominal CT and MRI revealed a 5.5 cm sized, septated, non-enhancing mass in the hepatic hilum. The patient was initially diagnosed with an inflammatory pseudotumor. Despite 9 days of antibiotic therapy, the patient's clinical symptoms did not improve. A liver biopsy was performed in the interest of formulating a differential diagnosis. This procedure revealed tumor cells positive for CD99 on immunohistochemistry. The patient was diagnosed with a PNET.

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Reduction of Outage Probability due to Handover by Mitigating Inter-cell Interference in Long-Term Evolution Networks

  • Hussein, Yaseein Soubhi;Ali, Borhanuddin Mohd;Rasid, Mohd Fadlee A.;Sali, Aduwati
    • ETRI Journal
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    • v.36 no.4
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    • pp.554-563
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    • 2014
  • The burgeoning growth of real-time applications, such as interactive video and VoIP, places a heavy demand for a high data rate and guarantee of QoS from a network. This is being addressed by fourth generation networks such as Long-Term Evolution (LTE). But, the mobility of user equipment that needs to be handed over to a new evolved node base-station (eNB) while maintaining connectivity with high data rates poses a significant challenge that needs to be addressed. Handover (HO) normally takes place at cell borders, which normally suffers high interference. This inter-cell interference (ICI) can affect HO procedures, as well as reduce throughput. In this paper, soft frequency reuse (SFR) and multiple preparations (MP), so-called SFRAMP, are proposed to provide a seamless and fast handover with high throughput by keeping the ICI low. Simulation results using LTE-Sim show that the outage probability and delay are reduced by 24.4% and 11.9%, respectively, over the hard handover method - quite a significant result.

A Case of Long-term Survival in a Patient with Primary Primitive Neuroectodermal Tumor of the Lung

  • Kang, Lae Hyung;Kim, Hyeong Jin;Jang, Jin Ho;Kim, Jun Hyun;Choi, Kyoung Un;Jeon, Doosoo
    • Kosin Medical Journal
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    • v.33 no.2
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    • pp.263-270
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    • 2018
  • Primitive neuroectodermal tumor (PNET) arising primarily in the lung is an extremely rare and aggressive malignancy with poor chances of patient survival. We present a case of long-term survival by a 29-year-old woman with PNET diagnosed after a hertological and immunohertochemical examination of a biopsy specimen obtained by performing video-assisted thoracic surgery. The patient underwent a left lower lung lobe lobectomy and 6 cycles of adjuvant chemotherapy. The patient has been free of any symptoms of the recurrence of the disease for 6 years after treatment completion.

Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

  • Tao, Hai-Tao;Hu, Yi;Wang, Jin-Liang;Cheng, Yao;Zhang, Xin;Wang, Huan;Zhang, Su-Jie
    • Asian Pacific Journal of Cancer Prevention
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    • v.14 no.5
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    • pp.2967-2971
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    • 2013
  • Background: Extraskeletal Ewing sarcoma (EES)/primitive neuroectodermal tumours (PNET) are rare soft tissue sarcomas. Prognostic factors and optimal therapy are still unconfirmed. Materials and Methods: We performed a retrospective analysis on patients to explore the clinic characteristics and prognostic factors of this rare disease. A total of 37 patients older than 15 years referred to our institute from Jan., 2002 to Jan., 2012 were reviewed. The characteristics, treatment and outcome were collected and analyzed. Results: The median age was 28 years (range 15-65); the median size of primary tumours was 8.2 cm (range 2-19). Sixteen patients (43%) had metastatic disease at the initial presentation. Wide surgical margins were achieved in 14 cases (38%). Anthracycline or platinum-based chemotherapy was performed on 29 patients (74%). Radiotherapy was delivered in 13 (35%). At a median follow-up visit of 24 months (range 2-81), the media event-free survival (EFS) and overall survival (OS) were 15.8 and 30.2 months, respectively. The 3-year EFS and OS rates were 24% and 43%, respectively. Metastases at presentation and wide surgical margins were significantly associated with OS and EFS. Tumour size was significantly associated with OS but not EFS. There were no significant differences between anthracycline and platinum based chemotherapy regarding EFS and OS. Conclusions: EES/PNET is a malignant tumour with high recurrence and frequent distant metastasis. Multimodality therapy featuring wide surgical margins, aggressive chemotherapy and adjuvant local radiotherapy is necessary for this rare disease. Platinum-based chemotherapy can be used as an adjuvant therapy.

Primitive neuroectodermal tumor of the maxillary sinus in an elderly male: A case report and literature review

  • Shah, Saiquat;Huh, Kyung-Hoe;Yi, Won-Jin;Heo, Min-Suk;Lee, Sam-Sun;Choi, Soon-Chul
    • Imaging Science in Dentistry
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    • v.44 no.4
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    • pp.307-314
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    • 2014
  • Primitive neuroectodermal tumor (PNET), which belongs to the Ewing's sarcoma (ES) family of tumors, is mainly seen in children and young adults. PNETs are extremely rare in the maxilla. Here, we report a case of PNET of the left maxillary sinus in an elderly male. Magnetic resonance imaging (MRI) revealed a slightly enhanced solid mass occupying the left maxillary sinus and infiltrating into the retroantral space. A partial maxillectomy was performed. Despite postoperative chemotherapy, follow-up computed tomography (CT) and MRI revealed a nodal metastasis in the submandibular space. Neck dissection was performed. However, the patient died 10 months after the second surgery because of distant metastasis to the liver. MRI and CT were particularly useful in detecting the extent of the tumor, recurrence, and metastasis. Further, a literature review of the previously reported PNET cases of the maxilla was carried out. In this paper, we also discuss the current approach for the diagnosis and management of these tumors.