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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/29334442/a-rare-initial-presentation-of-primary-diffuse-leptomeningeal-pnet-in-a-10-year-old-male
#1
Peter Paul Lim, Jagadeesh Ramdas, Michal Ann Miller, Conrad Schuerch, George Wu
Primary leptomeningeal primitive neuroectodermal tumors (PNETs) are extremely rare childhood central nervous system malignancies harboring a very poor prognosis. There is no consensus treatment for these tumors to date. We report a case of a 10-year-old male who presented with mental status change, hydrocephalus, intracranial and spinal diffuse leptomeningeal enhancement without a primary mass upon cranial imaging and a negative initial biopsy until five months into his presentation. He responded significantly well to initial chemotherapy and radiotherapy...
December 2017: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/29333015/a-profile-of-pediatric-solid-tumors-a-single-institution-experience-in-kashmir
#2
Namita Sharma, Ayesha Ahmad, Gull M Bhat, Sheikh A Aziz, Mohammad Maqbool Lone, Nisar A Bhat
Aims: The purpose of this retroprospective study was to study the epidemiological characteristics and outcomes of children with solid tumors at our institution. Subjects and Methods: Three hundred and three pediatrics patients registered at Regional Cancer Centre (RCC), Sher-i-Kashmir Institute of Medical Sciences (SKIMS), Srinagar, Kashmir, between January 2008 and June 2014, were analyzed with regard to demographic status, presenting complaints, investigations, treatment, morbidity, and outcomes...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29315108/review-of-molecular-classification-and-treatment-implications-of-pediatric-brain-tumors
#3
Ana S Guerreiro Stucklin, Vijay Ramaswamy, Craig Daniels, Michael D Taylor
PURPOSE OF REVIEW: Brain tumors are the most common solid tumors and leading cause of cancer-related death in children. The advent of large-scale genomics has resulted in a plethora of profiling studies that have mapped the genetic and epigenetic landscapes of pediatric brain tumors, ringing in a new era of precision diagnostics and targeted therapies. In this review, we highlight the most recent findings, focusing on studies published after 2015, and discuss how new evidence is changing the care of children with brain tumors...
February 2018: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/29299687/the-incidence-of-brainstem-primitive-neuroectodermal-tumors-of-childhood-based-on-seer-data
#4
Omar Chamdine, Ghada Ahmad Saad Elhawary, Ahmad Samir Alfaar, Ibrahim Qaddoumi
PURPOSE: Incidence of BS primitive neuroectodermal tumors (BS-PNET) in children is not reported to date. Our main objectives were to estimate the incidence and report the outcome of BS-PNET in children. METHODS: Data were collected using the Surveillance Epidemiology and End Results cancer registry. RESULTS: From 1973 to 2013, we identified 83 pediatric patients (aged 0-21 years). Patients were divided into two age groups (0-3 years and 4-21 years)...
January 3, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29285586/intracranial-ewing-sarcoma-four-pediatric-examples
#5
Michael J Yang, Ros Whelan, Jennifer Madden, Jean M Mulcahy Levy, B K Kleinschmidt-DeMasters, Todd C Hankinson, Nicholas K Foreman, Michael H Handler
BACKGROUND: Ewing sarcoma typically arises in bone and is unrelated to intraparenchymal small blue cell embryonal central nervous system (CNS) tumors previously designated primitive neuroectodermal tumors (PNETs). When the CNS is impacted, it is usually secondary to local extension from either the epidural space, skull, or intracranial or spinal metastases. Primary examples within the cranial vault are rare, usually dural-based, and are largely case reports in the literature. We detail four pediatric patients with solitary, primary intracranial Ewing sarcoma, all manifesting the archetypal EWRS1 gene rearrangement that confirms diagnosis...
December 28, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29280457/reirradiation-for-recurrent-pediatric-central-nervous-system-malignancies-a-multi-institutional-review
#6
Avani D Rao, Arif S Rashid, Qinyu Chen, Rosangela C Villar, Daria Kobyzeva, Kristina Nilsson, Karin Dieckmann, Alexey Nechesnyuk, Ralph Ermoian, Sara Alcorn, Shannon M MacDonald, Matthew M Ladra, Eric C Ford, Brian A Winey, Maria Luisa S Figueiredo, Michael J Chen, Stephanie A Terezakis
PURPOSE: Reirradiation has been proposed as an effective modality for recurrent central nervous system (CNS) malignancies in adults. We evaluated the toxicity and outcomes of CNS reirradiation in pediatric patients. METHODS AND MATERIALS: The data from pediatric patients <21 years of age at the initial diagnosis who developed a recurrent CNS malignancy that received repeat radiation therapy (RT) across 5 facilities in an international pediatric research consortium were retrospectively reviewed...
November 1, 2017: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/29245277/primary-intramedullary-primitive-neuroectodermal-tumor-a-case-report-and-review-of-the-literature
#7
Guoqing Wang, Fuyou Guo
RATIONALE: Primary spinal primitive neuroectodermal tumors (PNETs) are highly malignant tumors, which are extremely rare entities and primary intramedullary PNETs are extremely rare. Till now, only 24 cases of primary intramedullary PNET have been reported. PATIENT CONCERNS: A 26-year-old male presented with progressive low back and lower limb pain for 1 month. DIAGNOSES: Based on MRI and histopathological findings, he was diagnosed with primary intramedullary PNET...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29239847/metastatic-primitive-neuroectodermal-tumor-of-the-prostate-a-case-report-and-review-of-the-literature
#8
Ali Kord Valeshabad, Paul Choi, Nour Dababo, Ejaz Shamim, Alaa Alsadi, Karen L Xie
No abstract text is available yet for this article.
November 7, 2017: Clinical Genitourinary Cancer
https://www.readbyqxmd.com/read/29236237/outcome-of-children-and-adolescents-with-central-nervous-system-tumors-in-phase-i-trials
#9
Fernando Carceller, Francisco Bautista, Irene Jiménez, Raquel Hladun-Álvaro, Cécile Giraud, Luca Bergamaschi, Madhumita Dandapani, Isabelle Aerts, François Doz, Didier Frappaz, Michela Casanova, Bruce Morland, Darren R Hargrave, Gilles Vassal, Andrew D J Pearson, Birgit Geoerger, Lucas Moreno, Lynley V Marshall
Central nervous system (CNS) tumors are a leading cause of death in pediatric oncology. New drugs are desperately needed to improve survival. We evaluated the outcome of children and adolescents with CNS tumors participating in phase I trials within the Innovative Therapies for Children with Cancer (ITCC) consortium. Patients with solid tumors aged < 18 years at enrollment in their first dose-finding trial between 2000 and 2014 at eight ITCC centers were included retrospectively. Survival was evaluated using univariate/multivariate analyses...
December 13, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29226988/cns-high-grade-neuroepithelial-tumor-with-bcor-internal-tandem-duplication-a-comparison-with-its-counterparts-in-the-kidney-and-soft-tissue
#10
Yuka Yoshida, Sumihito Nobusawa, Satoshi Nakata, Mitsutoshi Nakada, Yoshiki Arakawa, Yohei Mineharu, Yasuo Sugita, Takako Yoshioka, Asuka Araki, Yuichiro Sato, Hideo Takeshima, Masahiko Okada, Akira Nishi, Tatsuya Yamazaki, Kenichi Kohashi, Yoshinao Oda, Junko Hirato, Hideaki Yokoo
Central nervous system high-grade neuroepithelial tumors with BCOR alteration (CNS HGNET-BCOR) are a recently reported rare entity, identified as a small fraction of tumors previously institutionally diagnosed as so-called CNS primitive neuroectodermal tumors. Their genetic characteristic is a somatic internal tandem duplication in the 3' end of BCOR (BCOR ITD), which has also been found in clear cell sarcomas of the kidney (CCSK) and soft tissue undifferentiated round cell sarcomas/primitive myxoid mesenchymal tumors of infancy (URCS/PMMTI), and these BCOR ITD-positive tumors have been reported to share similar pathological features...
December 11, 2017: Brain Pathology
https://www.readbyqxmd.com/read/29204198/profile-and-outcome-of-pediatric-brain-tumors-experience-from-a-tertiary-care-pediatric-oncology-unit-in-south-india
#11
Supriya Gujjar Suresh, Arathi Srinivasan, Julius Xavier Scott, Santosh Mohan Rao, Balasubramaniam Chidambaram, Sanjay Chandrasekar
Context: Tumors of the central nervous system (CNS) constitute the second most common pediatric cancers. Unlike leukemia, management of CNS tumors requires a good multidisciplinary team. Higher rates of treatment abandonment are documented in view of complexity of the treatment with long duration, involving neurosurgery, radiation, chemotherapy, and high cost of treatment. Morbidity associated with CNS tumors may be significant in terms of physical deficits as well as neuropsychological and neuroendocrine sequelae...
July 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29204027/a-simplified-overview-of-world-health-organization-classification-update-of-central-nervous-system-tumors-2016
#12
REVIEW
Anshu Gupta, Tanima Dwivedi
After 8 years, an update of central nervous system (CNS) tumors was published in 2016 after 2007. First time ever, molecular markers along with histology have been used in classification of any tumor. Major changes are seen in glioma and medulloblastoma groups. Few entities have been added such as diffuse midline glioma, H3 K27M-mutant, RELA fusion-positive ependymoma, embryonal tumor with multilayered rosettes, C19MC-altered, and hybrid nerve sheath tumors. Few variants and patterns that no longer have diagnostic and/or biological relevance and have been deleted such as glioblastoma cerebri, protoplasmic and fibrillary astrocytoma, and cellular ependymoma...
October 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/29200961/posterior-cerebral-fossa-medulloepithelioma-report-of-a-case
#13
Nezha Oumghar, Fatima Ezzahra Hazmiri, Abdelhamid El Omrani, Hanane Rais, Mouna Khouchani
Background: Medulloepithelioma is a rare primitive neuroectodermal tumor of the central nervous system, usually developing in childhood. Due to its rarity, the optimal management is still unknown. The prognosis is poor, especially when resection is incomplete. Adjuvant radiochemotherapy is often indicated. Case presentation: We report a rare case of infratentorial medulloepithelioma in a 3 year old girl. She presented symptoms of increased intracranial pressure...
2017: BMC Clinical Pathology
https://www.readbyqxmd.com/read/29200119/pediatric-brain-tumors
#14
Mai Dang, Peter C Phillips
PURPOSE OF REVIEW: This article describes the most common pediatric brain tumors and highlights recent developments in their diagnosis and treatment strategies. RECENT FINDINGS: We are in the midst of a molecular era for pediatric brain tumors. Genetic and epigenetic profiling of tumors has impacted their diagnosis, allowing for the subgrouping of heterogeneous tumor groups and leading to the complete renaming of some tumor types. These advances are reflected in the new 2016 World Health Organization classification...
December 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29194699/ultrasonographic-findings-of-1385-adrenal-masses-a-retrospective-study-of-1319-benign-and-66-malignant-masses
#15
Xia Gong, Yifei Yu, Weiwei Zhan
OBJECTIVES: To evaluate the features of adrenal masses on ultrasonography and correlate the findings with the pathologic diagnoses to help distinguish benign from malignant adrenal lesions. METHODS: Ultrasonography was performed in 1363 patients with adrenal lesions. The following ultrasonographic parameters were recorded: size, shape, margin, echogenicity, echo texture, cystic necrosis, calcifications, and blood supply. The sensitivity and specificity of aggressive features for predicting malignancy were calculated...
December 1, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/29180935/atypical-bilateral-cerebellopontine-angle-medulloblastoma-differential-diagnosis-immunohistochemical-features-and-radiological-presentation
#16
Kelvin M Piña Batista, Sayoa Alvarez de Eulate-Beramendi, María Rico, Victor Rodrigo, Yordy E Batista Batista, Kenia Y Alvarez Reyes
Medulloblastoma (MB) is a highly aggressive soft tissue neoplasm, classified as a primitive neuroectodermal tumor. It is the most common posterior fossa tumor in children, but occurs rarely in adults. MB accounts for approximately 20% of all primary central nervous system (CNS) tumors of childhood, while its incidence is around 1% of adult brain tumors. Most often it occurs in the cerebellum. We report a case of multicentric MB involving the bilateral cerebellopontine angle (CPA) and right cerebellar hemisphere...
2017: Contemporary Oncology Współczesna Onkologia
https://www.readbyqxmd.com/read/29176463/primary-ewing-sarcoma-of-the-mastoid-a-novel-case-mimicking-acute-mastoiditis
#17
Grace Egan, Joanna Pierro, Pillai Pallavi Madhusoodhan, Ghulam Ilyas, Benjamin Cohen, Teena Bhatla
Ewing sarcoma (EWS) is a primitive neuroectodermal tumor arising in bone or soft tissue. It is the second most common primary bone malignancy of children and adolescents, with a peak incidence in the second decade of life. It most often arises in the long bones of the extremities and pelvis. Here, we present a novel case of EWS arising from the mastoid bone in a 5-year-old African American male who presented with symptoms of acute mastoiditis. This unique presentation highlights the importance of considering EWS in a patient who presents with atypical mastoiditis or a rapidly growing mass in the postauricular region...
November 23, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29168356/single-institution-experience-on-cancer-among-adolescents-15-19-years-of-age
#18
Begül Yağcı-Küpeli, Canan Akyüz, Bilgehan Yalçın, Ali Varan, Tezer Kutluk, Münevver Büyükpamukçu
Yağcı-Küpeli B, Akyüz C, Yalçın B, Varan A, Kutluk T, Büyükpamukçu M. Single institution experience on cancer among adolescents 15-19 years of age. Turk J Pediatr 2017; 59: 1-5. Adolescent cancers differ from other age groups in terms of cancer types, treatment and outcome. We aimed to present our institutional data on survival of certain types of cancer in adolescents. Hospital files were retrospectively evaluated for distribution of tumor types, clinical features, and overall and event-free survival (OS and EFS) rates in adolescents with malignant tumors...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29163353/primary-ewing-sarcoma-of-the-thyroid-eight-cases-in-a-decade-a-case-report-and-literature-review
#19
Paweł Kabata, Sonia Kaniuka-Jakubowska, Wanda Kabata, Joanna Lakomy, Wojciech Biernat, Krzysztof Sworczak, Janusz Jaśkiewicz, Maciej Świerblewski
Sarcomas represent less than 1% of all malignant tumors found in the thyroid. Of these, primary extraosseoussarcoma has been reported only a few times in the past decade. We present the case of a 34-year-old male who had a fast-growing hard mass in the lower left neck. FNA was inconclusive. Core needle biopsy revealed the diagnosis of an Ewing sarcoma/primitive neuroectodermal tumor. Mutation of EWSR1 was confirmed using the FISH method. Following treatment by neoadjuvant chemotherapy, we observed clinical, radiological, and finally histopathological remission...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29151797/late-onset-of-primary-pulmonary-primitive-neuroectodermal-tumor-a-case-report
#20
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)
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