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https://www.readbyqxmd.com/read/28521298/insulin-like-growth-factor-2-axis-supports-the-serum-independent-growth-of-malignant-rhabdoid-tumor-and-is-activated-by-microenvironment-stress
#1
Ting Li, Jin Wang, Pengfei Liu, Jiadong Chi, Han Yan, Lei Lei, Zexing Li, Bing Yang, Xi Wang
Malignant rhabdoid tumors (MRTs) are rare, lethal, pediatric tumors predominantly found in the kidney, brain and soft tissues. MRTs are driven by loss of tumor suppressor SNF5/INI1/SMARCB1/BAF47. The prognosis of MRT is poor using currently available treatments, so new treatment targets need to be identified to expand treatment options for patients experiencing chemotherapy resistance. The growth hormone insulin-like growth factor 2 (IGF2) signaling pathway is a promising target to overcome drug resistance in many cancers...
May 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28521039/rhabdoid-meningioma-grading-and-prognostic-significance-of-this-uncommon-variant
#2
Valeria Barresi, Maria Caffo
No abstract text is available yet for this article.
May 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28511398/undifferentiated-uterine-sarcoma-an-uncommon-case-report
#3
Madhumita Mukhopadhyay, Chhanda Das, Tamanna Parvin, Keya Basu
Endometrial Stromal Sarcomas (ESS) are rare uterine malignancy of mesodermal origin. A 65-year-old female presented with postmenopausal bleeding in the Department of Gynaecology in our hospital. Computed Topography (CT) revealed an enlarged uterus with areas of low attenuation. On gross appearance endometrial cavity was distorted with an irregular friable necrotic mass. Histopathologically, it was diagnosed as undifferentiated uterine sarcoma. Rhabdoid, osteoid and cartilaginous differentiation were found along with osteoclast like giant cells...
March 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28508928/embryonal-tumors-in-canadian-children-less-than-36-months-of-age-results-from-the-canadian-pediatric-brain-tumor-consortium-cpbtc
#4
E Story, D L Johnston, U Bartels, A S Carret, B Crooks, D D Eisenstat, C Fryer, L Lafay-Cousin, V Larouche, B Wilson, S Zelcer, M Silva, J Brossard, E Bouffet, D L Keene
Embryonal tumors are a heterogeneous group of central nervous system (CNS) tumors whose subgroups have varying incidence and outcome. Despite these differences, they are often grouped as a single entity for study purposes. To date, there are no Canadian multi-institutional studies examining the incidence and outcome of all embryonal subtypes. The current study is an observational study reviewing embryonal tumors in all patients less than 36 months of age diagnosed with a CNS tumor in Canada from 1990 to 2005...
May 16, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28504702/pgbd5-promotes-site-specific-oncogenic-mutations-in-human-tumors
#5
Anton G Henssen, Richard Koche, Jiali Zhuang, Eileen Jiang, Casie Reed, Amy Eisenberg, Eric Still, Ian C MacArthur, Elias Rodríguez-Fos, Santiago Gonzalez, Montserrat Puiggròs, Andrew N Blackford, Christopher E Mason, Elisa de Stanchina, Mithat Gönen, Anne-Katrin Emde, Minita Shah, Kanika Arora, Catherine Reeves, Nicholas D Socci, Elizabeth Perlman, Cristina R Antonescu, Charles W M Roberts, Hanno Steen, Elizabeth Mullen, Stephen P Jackson, David Torrents, Zhiping Weng, Scott A Armstrong, Alex Kentsis
Genomic rearrangements are a hallmark of human cancers. Here, we identify the piggyBac transposable element derived 5 (PGBD5) gene as encoding an active DNA transposase expressed in the majority of childhood solid tumors, including lethal rhabdoid tumors. Using assembly-based whole-genome DNA sequencing, we found previously undefined genomic rearrangements in human rhabdoid tumors. These rearrangements involved PGBD5-specific signal (PSS) sequences at their breakpoints and recurrently inactivated tumor-suppressor genes...
May 15, 2017: Nature Genetics
https://www.readbyqxmd.com/read/28503314/atypical-teratoid-rhabdoid-tumor-of-lumbar-spine-in-a-toddler-child
#6
Min Fang Chao, Yu-Feng Su, Twei-Shiun Jaw, Shyh-Shin Chiou, Chih-Hung Lin
BACKGROUND: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare pediatric central nervous system malignancy with poor outcome. AT/RT is infrequently located in the spinal cord. CLINICAL PRESENTATION: A 16-month-old boy presented with progressive urinary retention and weakness of the lower extremities. Magnetic resonance imaging of the spine revealed an intradural extramedullary mass occupying the spinal canal at the level of T10-L3. The tumor was successfully resected by using neuroendoscopy...
2017: Spinal Cord Series and Cases
https://www.readbyqxmd.com/read/28502320/atypical-teratoid-rhabdoid-tumor-arising-in-a-pleomorphic-xanthoastrocytoma-a-rare-entity
#7
Meral Uner, Arzu Saglam, Bilge Can Meydan, Kerim Aslan, Figen Soylemezoglu
Atypical teratoid rhabdoid tumor (AT/RT) is a rare and aggressive tumor usually occurring at younger ages. Pleomorphic xanthoastrocytomas (PXA) on the other hand are quiescent tumors with benign behavior. AT/RTs arising in the setting of PXA are exceptional. We present the case of a 23-year-old female patient, the fourth in the literature, speculated as having AT/RT arising within a PXA, as demonstrated by the presence of INI1 mutation. The patient presented with a short history of headache, which increased over time, and emerging seizures...
May 15, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28498283/synovial-sarcoma-of-peripheral-nerves-analysis-of-15-cases
#8
John S A Chrisinger, Usama I Salem, Lars-Gunnar Kindblom, Behrang Amini, Magnus Hansson, Jeanne M Meis
Synovial sarcoma of peripheral nerve (SSPN) is rare with only 26 cases reported in English. SSPN is often mistaken for a benign or malignant peripheral nerve sheath tumor (PNST) by clinicians and pathologists. Fifteen cases of SSPN were retrieved from the pathology files of 3 institutions. All tumors arose in a nerve based on imaging and/or operative findings and the diagnoses were histologically confirmed. Neuropathic symptoms predominated in 11 women and 4 men, 19- to 62-year-old (median, 39 years) with tumors involving the ulnar (5), median (3), peroneal (3) or sciatic (2) nerve, or the L4 or T1 nerve root (2)...
May 11, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28482042/bap1-mutations-in-high-grade-meningioma-implications-for-patient-care
#9
Ganesh M Shankar, Sandro Santagata
We have recently shown that the BAP1 (BRCA1-associated protein-1) tumor suppressor gene is inactivated in a subset of clinically-aggressive meningiomas that display rhabdoid histomorphology. Immunohistochemistry for BAP1 protein provides a rapid and inexpensive method for screening suspected cases. Notably, some patients with BAP1-mutant meningiomas have germline BAP1 mutations and the BAP1 tumor predisposition syndrome (BAP1-TPDS). It appears that nearly all patients with germline BAP1 mutations develop malignancies by age 55, most frequently uveal melanoma, cutaneous melanoma, pleural or peritoneal malignant mesothelioma or renal cell carcinoma, although other cancers have also been associated with the BAP1-TPDS...
May 8, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/28474974/primary-spinal-epidural-cic-dux4-undifferentiated-sarcoma-in-a-child
#10
John E Donahue, Evgeny Yakirevich, Shan Zhong, Diana O Treaba, Nelli S Lakis, Siraj M Ali, Suzanne M de la Monte, Shamlal Mangray
Primitive round- or spindle-cell EWSR1-negative undifferentiated sarcomas harboring CIC-DUX4 gene fusion are the most common form of Ewing-like sarcomas. These tumors primarily occur in peripheral soft tissues, but examples have been described within viscera and the brain. As far as we are aware, CIC-DUX4 positive primary epidural spinal sarcoma has not been reported. Herein, we describe a T5-T6 epidural tumor in a 15-year-old girl in which many neoplastic cells had moderate and focally abundant cytoplasm, including plasmacytoid or rhabdoid cells, rather than the more common Ewing-like morphology described in the majority of such tumors...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28469334/clinical-profile-of-pediatric-oncology-patients-treated-by-external-beam-radiotherapy-an-institutional-experience
#11
Virender Suhag, B S Sunita, Pankaj Vats, Arti Sarin, A K Singh, Mayuri Jain
INTRODUCTION: Pediatric tumors are a heterogeneous group of malignant conditions requiring multimodal treatment, and management of such cases is at time challenging. We present the clinical profile of pediatric cancer patients who received radiation, either alone or as adjuvant to surgery and chemotherapy; in prophylactic, radical or palliative clinical setting. AIM: This study was envisaged to review our experience of pediatric oncology cases, their clinical and morphological profile, dosage schedule of radiotherapy, and the therapy induced complications...
January 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28461278/does-stereotactic-radiosurgery-positively-impact-the-local-control-of-atypical-teratoid-rhabdoid-tumors
#12
REVIEW
Alfio Spina, Filippo Gagliardi, Nicola Boari, Michele Bailo, Pietro Mortini
Atypical Teratoid Rhabdoid Tumors (ATRT) are rare and aggressive tumours, usually affecting patients under 3 years of age and being characterized by poor prognosis. Nowadays a multimodal management, including surgery, chemotherapy (CT) and radiation therapy (RT), is advocated depending on patients' age and tumour stage, even if there is no consensus about the best treatment modality. Local RT seems to be the most effective treatment in prolonging progression-free and overall survival rates, although RT might not be used on younger children because the risk of neurocognitive and endocrine sequelae...
April 28, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28459167/development-of-an-atypical-teratoid-rhabdoid-tumor-in-a-meningioma
#13
Boleslaw Lach, Michelle Kameda-Smith, Sheila Singh, Olufemi Ajani
We describe an atypical teratoid rhabdoid tumor (AT/RT) with a component of low-grade and anaplastic rhabdoid meningioma in a 7-year-old child. The AT/RT was uniformly negative for INI1 and displayed immunoreactivity for vimentin, P53, CD99, cytokeratins with AE1/AE3 antibodies, epithelial membrane antigen, β-catenin, smooth muscle actin, E-cadherin, and S-100 protein. AT/RT was continuous, with small foci of recognizable low-grade and anaplastic meningioma. The low-grade meningioma was INI1 positive with scattered INI1-negative nuclei, whereas the remaining tumor components were INI1 negative...
April 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28458948/primary-atypical-teratoid-rhabdoid-tumor-in-the-adult-spine
#14
Michael McGinity, Huma Siddiqui, Gulpreet Singh, Fermin Tio, Ahmed Shakir
BACKGROUND: Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive tumor of the central nervous system (WHO grade IV), which is most frequently found intracranially in young children and infants. Only three prior cases of primary ATRT involving the adult spine were found following a literature review, and the average survival for these patients was only 20 postoperative months. CASE DESCRIPTION: A 43 year-old female presented with an acute exacerbation of chronic neck pain...
2017: Surgical Neurology International
https://www.readbyqxmd.com/read/28452074/clinicopathologic-features-and-immunohistochemical-spectrum-of-11-cases-of-epithelioid-malignant-peripheral-nerve-sheath-tumors-including-ini1-smarcb1-results-and-braf-v600e-analysis
#15
Bharat Rekhi, Kemal Kosemehmetoglu, Gaye Guler Tezel, Sergulen Dervisoglu
Epithelioid malignant peripheral nerve sheath tumor (MPNST) is a rare, relatively less chemosensitive sarcoma. We report clinicopathologic features of 11 epithelioid MPNSTs, including rare forms, along with INI1 immunostaining and BRAF V600E mutation results. BRAF V600E mutation was tested by Real-time polymerase chain reaction (PCR) technique. Eleven tumors occurred in six men and five women (M:F ratio = 0.85:1) within an age range of 5-73 years (average = 44), mostly in lower limbs (five), followed by upper limbs (four)...
April 27, 2017: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/28445970/acid-ceramidase-is-a-novel-drug-target-for-pediatric-brain-tumors
#16
Ninh B Doan, Ha S Nguyen, Andrew Montoure, Mona M Al-Gizawiy, Wade M Mueller, Shekar Kurpad, Scott D Rand, Jennifer M Connelly, Christopher R Chitambar, Kathleen M Schmainda, Shama P Mirza
Pediatric brain tumors are the most common solid tumors in children and are also a leading culprit of cancer-related fatalities in children. Pediatric brain tumors remain hard to treat. In this study, we demonstrated that medulloblastoma, pediatric glioblastoma, and atypical teratoid rhabdoid tumors express significant levels of acid ceramidase, where levels are highest in the radioresistant tumors, suggesting that acid ceramidase may confer radioresistance. More importantly, we also showed that acid ceramidase inhibitors are highly effective at targeting these pediatric brain tumors with low IC50 values (4...
April 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28442586/therapeutic-targeting-of-ptk7-is-cytotoxic-in-atypical-teratoid-rhabdoid-tumors
#17
Shanta M Messerli, Mariah M Hoffman, Etienne Z Gnimpieba, Ratan D Bhardwaj
Novel discoveries involving the evaluation of potential therapeutics are based on newly identified molecular targets for atypical teratoid rhabdoid tumors (ATRT), which are the most common form of infantile brain tumors. Central nervous system ATRTs are rare, aggressive, and fast growing tumors of the brain and spinal cord and carry a very poor prognosis. Currently, the standard of care for ATRT patients is based on surgical resection followed by systemic chemotherapy and radiation therapy, which result in severe side effects...
April 25, 2017: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/28436365/adverse-pathologic-characteristics-in-the-small-renal-mass-implications-for-active-surveillance
#18
Jamil S Syed, Cayce B Nawaf, James Rosoff, Campbell Bryson, Kevin A Nguyen, Alfredo Suarez-Sarmiento, Maria Serrano, Adam S Kibel, Peter Humphrey, Brian Shuch, Adebowale J Adeniran
INTRODUCTION: Evidence has demonstrated that tumor size is related to adverse oncologic outcomes in small renal tumors (≤ 4 cm). We evaluated the association of adverse pathologic features (APF) with tumor size and survival in patients with a small renal mass (SRM). MATERIALS AND METHODS: We retrospectively reviewed the pathologic characteristics of 380 surgically resected SRMs from a single institution. APFs included lymphovascular invasion, coagulative necrosis, sarcomatoid/rhabdoid features, papillary type II histology, and perinephric fat/renal sinus invasion...
April 2017: Canadian Journal of Urology
https://www.readbyqxmd.com/read/28434767/extrarenal-rhabdoid-tumor-presented-with-an-immobile-arm-in-a-one-year-old-boy
#19
Chi-Ting Chung, Yen-Lin Liu, Chien-Jui Cheng, Kevin Li-Chun Hsieh, Min-Lan Tsai, Tai-Tong Wong
Infants with an immobile arm may be easily overlooked in primary care settings. Differential diagnoses include injuries, infections, neuropathies, ischemia and rarely, neoplasms. We report the case of a one-year-old boy with weakness in his left arm after minor trauma with a diagnosis of brachial plexus palsy initially. After rehabilitation for 2months, his weakness progressed to unsteady gait and quadriparesis. MRI revealed a huge solid tumor in the left supraclavicular fossa, which also involved the left brachial plexus, upper thoracic cavity, and left paravertebral space with invasion into the spinal canal...
April 20, 2017: Brain & Development
https://www.readbyqxmd.com/read/28432176/a-phase-i-study-of-the-cdk4-6-inhibitor-ribociclib-lee011-in-pediatric-patients-with-malignant-rhabdoid-tumors-neuroblastoma-and-other-solid-tumors
#20
Birgit Geoerger, Franck Bourdeaut, Steven G DuBois, Matthias Fischer, James I Geller, Nicholas G Gottardo, Aurélien Marabelle, Andrew D J Pearson, Shakeel Modak, Thomas Cash, Giles W Robinson, Marlyane Motta, Alessandro Matano, Suraj G Bhansali, Jason R Dobson, Sudha Parasuraman, Susan N Chi
Purpose: The cyclin-dependent kinase (CDK) 4/6 inhibitor, ribociclib (LEE011), displayed preclinical activity in neuroblastoma and malignant rhabdoid tumor (MRT) models. In this phase I study, the maximum tolerated dose (MTD) and recommended phase II dose (RP2D), safety, pharmacokinetics (PK), and preliminary activity of single-agent ribociclib were investigated in pediatric patients with neuroblastoma, MRT, or other cyclin D-CDK4/6-INK4-retinoblastoma pathway-altered tumors.Experimental Design: Patients (aged 1-21 years) received escalating once-daily oral doses of ribociclib (3-weeks-on/1-week-off)...
April 21, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
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