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https://www.readbyqxmd.com/read/27923222/pediatric-primary-diffuse-leptomeningeal-primitive-neuroectodermal-tumor-a-case-report-and-literature-review
#1
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...
December 7, 2016: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27920700/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-stomach
#2
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/27867771/prospect-and-progress-of-oncolytic-viruses-for-treating-peripheral-nerve-sheath-tumors
#3
Slawomir Antoszczyk, Samuel D Rabkin
INTRODUCTION: Peripheral nerve sheath tumors (PNSTs) are an assorted group of neoplasms originating from neuroectoderm and growing in peripheral nerves. Malignant transformation leads to a poor prognosis and is often lethal. Current treatment of PNSTs is predominantly surgical, which is often incomplete or accompanied by significant loss of function, in conjunction with radiotherapy and/or chemotherapy, for which the benefits are inconclusive. Oncolytic viruses (OVs) efficiently kill tumor cells while remaining safe for normal tissues, and are a novel antitumor therapy for patients with PNSTs...
2016: Expert Opinion on Orphan Drugs
https://www.readbyqxmd.com/read/27822490/primitive-neuroectodermal-tumor-of-the-uterus
#4
C R Elizalde, A Yagüe, J Fernandez, P Dieste, M J Puente, J Hernandez
•PNET of the uterus is rare and requires early diagnoses and treatment.•Molecular analysis is important to distinguish it from other tumors.•Different combinations of adjuvant chemotherapy have been report.
November 2016: Gynecologic Oncology Reports
https://www.readbyqxmd.com/read/27784193/successful-management-of-a-patient-with-malignant-thyroid-teratoma
#5
Guilherme Rabinowits, Justine Barletta, Lynette M Sholl, Encarnacion Reche, Jochen Lorch, Laura Goguen
BACKGROUND: Malignant thyroid teratomas are rare tumors with a poor prognosis. Little is known about their pathogenesis or treatment. Here, the case is reported of an adult woman with an aggressive thyroid teratoma with primitive neuroectodermal tumor (PNET) malignant transformation, successfully managed with neoadjuvant chemotherapy and surgery. PATIENT FINDINGS: Sequencing of paired tumor and normal tissues revealed a DICER1 c.5438A>G (p.E1813G) somatic mutation in 56% of sequencing reads consistent with a driver event...
November 28, 2016: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/27783941/mek-inhibitors-reverse-growth-of-embryonal-brain-tumors-derived-from-oligoneural-precursor-cells
#6
Katarzyna Modzelewska, Elena F Boer, Timothy L Mosbruger, Daniel Picard, Daniela Anderson, Rodney R Miles, Mitchell Kroll, William Oslund, Theodore J Pysher, Joshua D Schiffman, Randy Jensen, Cicely A Jette, Annie Huang, Rodney A Stewart
Malignant brain tumors are the leading cause of cancer-related deaths in children. Primitive neuroectodermal tumors of the CNS (CNS-PNETs) are particularly aggressive embryonal tumors of unknown cellular origin. Recent genomic studies have classified CNS-PNETs into molecularly distinct subgroups that promise to improve diagnosis and treatment; however, the lack of cell- or animal-based models for these subgroups prevents testing of rationally designed therapies. Here, we show that a subset of CNS-PNETs co-express oligoneural precursor cell (OPC) markers OLIG2 and SOX10 with coincident activation of the RAS/MAPK (mitogen-activated protein kinase) pathway...
October 25, 2016: Cell Reports
https://www.readbyqxmd.com/read/27776324/ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-kidney-a-report-of-three-cases
#7
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
https://www.readbyqxmd.com/read/27770281/long-term-safety-of-growth-hormone-replacement-therapy-after-childhood-medulloblastoma-and-pnet-it-is-time-to-set-aside-old-concerns
#8
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
https://www.readbyqxmd.com/read/27721314/gamma-knife-surgery-for-recurrent-or-residual-supratentorial-primitive-neuroectodermal-tumors
#9
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...
2016: Stereotactic and Functional Neurosurgery
https://www.readbyqxmd.com/read/27715363/orbital-medulloepithelioma-in-an-adult-patient-radiation-induced-second-neoplasia
#10
Antony George Francis Thottian, Rony Benson, Seema Kashyap, K P Haresh, Subhash Gupta, Dayanand Sharma, Goura Kishor Rath
Second cancers in survivors of hereditary retinoblastoma occur much more commonly than in the general population. This can be attributed both to the germline mutation of the RB gene and chemoradiation used for treatment of this paediatric cancer. Medulloepithelioma is an uncommon tumor of neuroectodermal origin, seen largely in the paediatric population and rarely reported in adults. Though the incidence of second malignancies is common in retinoblastoma, medulloepithelioma as a second malignancy in retinoblastoma survivors is rare, with only one case reported so far...
August 12, 2016: Orbit
https://www.readbyqxmd.com/read/27695363/atypical-teratoid-rhabdoid-tumors-challenges-and-search-for-solutions
#11
Ahitagni Biswas, Lakhan Kashyap, Aanchal Kakkar, Chitra Sarkar, Pramod Kumar Julka
Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal central nervous system tumor commonly affecting children <3 years of age. It roughly constitutes 1%-2% of all pediatric central nervous system tumors. Recent data show that it is the most common malignant central nervous system tumor in children <6 months of age. Management of this aggressive tumor is associated with a myriad of diagnostic and therapeutic challenges. On the basis of radiology and histopathology alone, distinction of AT/RT from medulloblastoma or primitive neuroectodermal tumor is difficult, and hence this tumor has been commonly misdiagnosed as primitive neuroectodermal tumor for decades...
2016: Cancer Management and Research
https://www.readbyqxmd.com/read/27682250/complete-and-sustained-response-of-adult-medulloblastoma-to-first-line-sonic-hedgehog-inhibition-with-vismodegib
#12
Emil Lou, Matthew Schomaker, Jon D Wilson, Mary Ahrens, Michelle Dolan, Andrew C Nelson
Medulloblastoma is an aggressive primitive neuroectodermal tumor of the cerebellum that is rare in adults. Medulloblastomas fall into 4 prognostically significant molecular subgroups that are best defined by experimental gene expression profiles: the WNT pathway, sonic hedgehog (SHH) pathway, and subgroups 3 and 4 (non-SHH/WNT). Medulloblastoma of adults belong primarily to the SHH category. Vismodegib, an SHH-pathway inhibitor FDA-approved in 2012 for treatment of basal cell carcinoma, has been used successfully in the setting of chemorefractory medulloblastoma, but not as a first-line therapy...
August 12, 2016: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/27601837/a-rare-case-of-extraskeletal-ewing-s-sarcoma-primitive-neuroectodermal-tumor-developing-in-maxillary-sinus-of-an-old-patient
#13
Maithili Mandar Kulkarni, Siddhi Gaurish Sinai Khandeparkar, Avinash R Joshi, Chitrangi Barpande
Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) family of tumors is an uncommon group of malignant neoplasms that may present in both skeletal and extraskeletal sites. PNET outside the central nervous system is called peripheral PNET (pPNET) developing from migrating embryonal cells of the neural crest. Very few cases of pPNET of the maxilla are reported in English literature. These tumors may be difficult to diagnose due to their primitive morphology. These tumors occur predominantly in infancy or early childhood...
May 2016: Journal of Oral and Maxillofacial Pathology: JOMFP
https://www.readbyqxmd.com/read/27586176/neurenteric-cyst-or-neuroendodermal-cyst-immunohistochemical-study-and-pathogenesis
#14
Chun-Ting Chen, Hung-Yi Lai, Shih-Ming Jung, Ching-Yi Lee, Chieh-Tsai Wu, Shih-Tseng Lee
BACKGROUND: Neurenteric cysts are rare central nervous system lesions derived from an endodermal origin. There is no consensus concerning pathogenesis due to the paucity of occurrences. The authors report their immunohistochemical study of ten cases with neurenteric cysts and postulate its pathogenesis. METHOD: Ten patients underwent surgical treatment for neurenteric cysts from 1995 to 2015. We retrospectively reviewed clinical, radiological, operative, and pathological findings for these patients...
August 29, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27567676/brain-stereotactic-biopsy-flow-cytometry-for-central-nervous-system-lymphoma-characterization-advantages-and-pitfalls
#15
Iole Cordone, Serena Masi, Mariantonia Carosi, Antonello Vidiri, Francesco Marchesi, Mirella Marino, Stefano Telera, Alessia Pasquale, Andrea Mengarelli, Laura Conti, Edoardo Pescarmona, Andrea Pace, Carmine M Carapella
BACKGROUND: Brain stereotactic biopsy (SB) followed by conventional histopathology and immunohistochemistry (IHC) is the gold standard approach for primary central nervous system lymphoma (PCNSL) diagnosis. Flow cytometry (FCM) characterization of fine-needle aspiration cytology and core needle biopsies are increasingly utilized to diagnose lymphomas however, no biological data have been published on FCM characterization of fresh single cell suspension from PCNSL SB. The aim of this study was to establish the feasibility and utility of FCM for the diagnosis and characterization of brain lymphomas from a tissue samples obtained by a single SB disaggregation...
2016: Journal of Experimental & Clinical Cancer Research: CR
https://www.readbyqxmd.com/read/27519263/development-of-mediastinal-lymphoma-after-radiotherapy-for-concurrent-medulloblastoma-and-pnet-in-a-patient-with-gorlin-syndrome
#16
Tao Jiang, Junmei Wang, Ying Wang, Chunde Li
BACKGROUND: Very young children with Gorlin syndrome are at risk for developing medulloblastoma. Patients with Gorlin syndrome may have multiple system abnormalities, including basal cell carcinomas, jaw cysts, desmoplastic medulloblastoma, palmar/plantar pits, rib abnormalities, and intracranial falx calcification. The early diagnosis of Gorlin syndrome in desmoplastic medulloblastoma patients is very important because these patients should receive chemotherapy as a first-line treatment and should avoid radiotherapy as much as possible...
August 12, 2016: World Journal of Surgical Oncology
https://www.readbyqxmd.com/read/27440405/clinical-behavior-of-intraocular-teratoid-medulloepithelioma-in-two-related-quarter-horses
#17
Caroline S Monk, William F Craft, Jeffrey R Abbott, Lisa L Farina, Sarah M Reuss, Sarah L Czerwinski, Dennis E Brooks, Caryn E Plummer
The objective of this paper is to describe clinical behavior, histopathologic features, and immunohistochemical staining of two-related horses with intraocular teratoid medulloepithelioma. Two-related Quarter Horses with similar intraocular masses presented to the UF-CVM Comparative Ophthalmology Service for evaluation and treatment. The first horse, a 3-year-old gelding, had glaucoma and a cyst-like mass in the anterior chamber. Enucleation was performed. Histopathology revealed a teratoid medulloepithelioma...
July 20, 2016: Veterinary Ophthalmology
https://www.readbyqxmd.com/read/27428490/primary-brachial-plexus-tumors-clinical-experiences-of-143-cases
#18
Xiaotian Jia, Jianyun Yang, Lin Chen, Cong Yu, Tadashi Kondo
OBJECTIVE: Primary brachial plexus tumors are extremely rare and the treatment is challengeable. Our aim is to share the experiences in the treatment of primary brachial plexus tumors. METHODS: A retrospective analysis of 143 patients with primary brachial plexus tumors was made in our department from January 2001 to December 2012. The clinical presentation of the patients, the characteristics and pathological results of the tumors and the prognosis were described...
September 2016: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/27413360/multimodality-treatment-in-ewing-s-sarcoma-family-tumors-of-the-maxilla-and-maxillary-sinus-review-of-the-literature
#19
REVIEW
David Thorn, Christoph Mamot, Fatime Krasniqi, Frank Metternich, Sven Prestin
The Ewing sarcoma family of tumors (ESFT) encompasses a group of highly aggressive, morphologically similar, malignant neoplasms sharing a common spontaneous genetic translocation that affect mostly children and young adults. These predominantly characteristic, small round-cell tumors include Ewing's sarcoma of the bone and soft tissue, as well as primitive neuroectodermal tumors (PNETs) involving the bone, soft tissue, and thoracopulmonary region (Askin's tumor). Extraosseous ESFTs are extremely rare, especially in the head and neck region, where literature to date consists of sporadic case reports and very small series...
2016: Sarcoma
https://www.readbyqxmd.com/read/27393580/metastatic-melanotic-neuroectodermal-tumor-of-infancy-report-of-a-case-and-review-of-the-literature
#20
Amir Azarisamani, Daniel Petrisor, John Wright, Ghali E Ghali
Melanotic neuroectodermal tumors of infancy (MNTI) are rapidly growing pigmented tumors that occur predominantly within bony head and neck structures. There are fewer than 400 cases reported in the literature with the majority affecting the maxilla. Locations in other intraosseous and extraosseous structures have been characterized, including the mandible (6% of MNTIs). Infants in the first year of life are primarily affected. Surgical resection is the primary treatment modality with and without adjuvant chemotherapy for malignant tumors, which comprise less than 25 cases in the literature, and of metatstatic mandibular tumors, which has only been documented in one other case...
December 2016: Journal of Oral and Maxillofacial Surgery
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