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malignant rhabdoid tumor

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https://www.readbyqxmd.com/read/28731921/loss-of-cdkn1c-in-a-recurrent-atypical-teratoid-rhabdoid-tumor
#1
Dustin Tran, Sandra Camelo-Piragua, Avneesh Gupta, Kate Gowans, Patricia L Robertson, Rajen Mody, Carl Koschmann
Atypical teratoid/rhabdoid tumor (AT/RT) is a malignant tumor that is commonly associated with biallelic alterations of SMARCB1. Recurrent or refractory AT/RT has not been molecularly characterized as well. We present the case of a child with recurrent AT/RT who underwent clinically integrated molecular profiling (germline DNA and tumor DNA/RNA sequencing). This demonstrated a somatic lesion in CDKN1C alongside hallmark loss of SMARCB1. This data allowed us to explore potential personalized therapies for this patient and expose a molecular driver that may be involved in similar cases...
July 20, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28731919/a-case-presentation-rare-occurrence-of-an-adolescent-male-presenting-with-an-atrt-and-simultaneous-low-grade-glioneuronal-tumor
#2
Matthew Cascio, Marie Rivera-Zengotita, John Fort
Atypical rhabdoid/teratoid tumor (ATRT) is an uncommon and highly malignant tumor of the central nervous system. The majority of ATRT tumors occur in infancy and young children located in the posterior fossa. The ideal treatment for cure remains controversial and prognosis is typically unfavorable. We present a case of an atypical presentation of ATRT, presenting in adolescence with an additional low-grade glioneuronal tumor discovered at diagnosis.
July 20, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28714904/brd9-inhibition-alone-or-in-combination-with-cytostatic-compounds-as-a-therapeutic-approach-in-rhabdoid-tumors
#3
Katja F Krämer, Natalia Moreno, Michael C Frühwald, Kornelius Kerl
Rhabdoid tumors (RT) are malignant neoplasms of early childhood. Despite intensive therapy, survival is poor and new treatment approaches are required. The only recurrent mutations in these tumors affect SMARCB1 and less commonly SMARCA4, both subunits of the chromatin remodeling complex SWItch/Sucrose Non-Fermentable (SWI/SNF). Loss of these two core subunits alters the function of the SWI/SNF complex, resulting in tumor development. We hypothesized that inhibition of aberrant SWI/SNF function by selective blockade of the BRD9 subunit of the SWI/SNF complex would reduce tumor cell proliferation...
July 16, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28683521/expression-of-cd34-and-%C3%AE-catenin-in-malignant-rhabdoid-tumor-of-the-liver-mimicking-proximal-type-epithelioid-sarcoma
#4
Woo Cheal Cho, Fabiola Balarezo
No abstract text is available yet for this article.
July 7, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28677107/a-french-retrospective-study-on-clinical-outcome-in-102-choroid-plexus-tumors-in-children
#5
A Siegfried, S Morin, C Munzer, M B Delisle, M Gambart, S Puget, C A Maurage, C Miquel, C Dufour, P Leblond, N André, D Figarella Branger, J Kanold, J-L Kemeny, C Icher, A Vital, E Uro Coste, A I Bertozzi
The aim of this study was to review and describe therapeutic approaches in children with choroid plexus tumor (CPT) based on a nationwide series. The World Health Organization classification subdivides these rare tumors into three histological subtypes corresponding to three grades of malignancy: low grade (grade I) choroid plexus papilloma (CPP), intermediate grade (grade II) atypical choroid plexus papilloma (aCPP) and high grade (grade III) choroid plexus carcinoma (CPC). This retrospective study included 102 French children younger than 18 years, treated from 2000 to 2012: 54 CPP, 26 aCPP and 22 CPC...
July 4, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28650886/elevated-fdg-activity-in-the-nonpneumatized-sphenoid-bone-in-an-infant
#6
Yin Jie Chen, Nelleke Tolboom, Lisa J States, Hongming Zhuang
A 7-month-old boy with malignant rhabdoid tumor of the right lateral neck, status post resection and chemotherapy, underwent FDG PET/CT for restaging. The images showed diffuse increased activity in the spleen and in the bone marrow of the appendicular bones and the spine, which is related to hematopoietin administered after chemotherapy. The images also revealed intense activity in the region of sphenoid bone, which is not a common region to have elevated FDG activity. The subsequent MRI scan showed that this activity was due to not-yet-converted red marrow in the sphenoid bone in this pediatric patient...
June 24, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28648942/dedifferentiated-chondrosarcoma-of-bone-with-prominent-rhabdoid-component
#7
Matthew Stemm, Chad Beck, Rajeev Mannem, John Neilson, Michael J Klein
Dedifferentiated chondrosarcomas (DDCS) are rare lesions, defined as tumors having a low-grade chondrosarcomatous component with an abrupt transition to a high-grade sarcoma. Although "malignant fibrous histiocytoma" (undifferentiated pleomorphic sarcoma) is the most common high grade saromatous component, many different types of sarcoma have been described. We present a case of dedifferentiated chondrosarcoma with rhabdomyosarcomatous differentiation harboring a prominent rhabdoid tumor component. To our knowledge, rhabdoid morphology in dedifferentiated chondrosarcoma has not been described in the English-language literature...
June 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28642448/combined-brd4-and-cdk9-inhibition-as-a-new-therapeutic-approach-in-malignant-rhabdoid-tumors
#8
Natalia Moreno, Till Holsten, Julius Mertins, Annabelle Zhogbi, Pascal Johann, Marcel Kool, Michael Meisterernst, Kornelius Kerl
Rhabdoid tumors are caused by the deletion of SMARCB1, whose protein encodes the SMARCB1 subunit of the chromatin remodeling complex SWI/SNF that is involved in global chromatin organization and gene expression control. Simultaneously inhibiting the main players involved in the deregulated transcription machinery is a promising option for preventing exaggerated tumor cell proliferation and survival as it may bypass compensatory mechanisms. In support of this hypothesis, we report efficient impairment of cellular proliferation and strong induction of cell death elicited by inhibition of bromodomain protein BRD4 and transcription kinase CDK9 using small molecular compounds...
June 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28631714/-extrarenal-rhabdoid-tumor-a-review-of-literature-and-a-report-of-cases-with-atypical-morphology
#9
A M Mitrofanova, V Yu Roshchin, A N Kislyakov, D S Abramov, D V Rogozhin, D M Konovalov
Extrarenal extracranial rhabdoid tumor (EERT) is a rare malignant pediatric tumor characterized by extremely aggressive behavior, rapid metastatic spread, low survival rates, and no targeted therapy. The morphological diagnosis of classical cases of EERT is not difficult and it is based on its characteristic histologic pattern and immunohistochemical findings in a relatively short time, which allows one to immediately initiate therapy. The paper describes two cases of ERRT in the complete absence of classical rhabdoid morphology, as revealed by light microscopy when the material was primarily assessed...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28620006/cancer-surveillance-in-gorlin-syndrome-and-rhabdoid-tumor-predisposition-syndrome
#10
REVIEW
William D Foulkes, Junne Kamihara, D Gareth R Evans, Laurence Brugières, Franck Bourdeaut, Jan J Molenaar, Michael F Walsh, Garrett M Brodeur, Lisa Diller
Gorlin syndrome and rhabdoid tumor predisposition syndrome (RTPS) are autosomal dominant syndromes associated with an increased risk of childhood-onset brain tumors. Individuals with Gorlin syndrome can manifest a wide range of phenotypic abnormalities, with about 5% of family members developing medulloblastoma, usually occurring in the first 3 years of life. Gorlin syndrome is associated with germline mutations in components of the Sonic Hedgehog pathway, including Patched1 (PTCH1) and Suppressor of fused (SUFU)SUFU mutation carriers appear to have an especially high risk of early-onset medulloblastoma...
June 15, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28616426/cytologic-diagnosis-of-atypical-teratoid-rhabdoid-tumor-based-on-touch-imprint-study-report-of-a-case-with-review-of-literature
#11
Mansoureh Shokripour, Negar Azarpira, Navid Omidifar, Bita Pakniat
Atypical teratoid rhabdoid tumor (ATRT) is a rare malignant tumor with gloom destiny. Our case was a 4-year-old boy with a temporal lobe tumor that was then became evident of ATRT with recurrent happening. In a retrospective review of all cytologic slides, we found unique rhabdoid cells that are morphologically evident cells for ATRT in both times. Unfortunately, the cells were overlooked at the first time. We conclude if the pathologist is experienced to see rhabdoid cells noticing these cells is highly helpful for diagnosis ATRT, especially in frozen sectioning...
2017: Journal of Education and Health Promotion
https://www.readbyqxmd.com/read/28598565/atypical-teratoid-rhabdoid-tumors-in-children-treated-with-multimodal-therapies-the-necessity-of-upfront-radiotherapy-after-surgery
#12
Jeongshim Lee, Dong-Seok Kim, Jung Woo Han, Chang-Ok Suh
BACKGROUND: Atypical teratoid/rhabdoid tumor (ATRT) is a rare malignant pediatric brain tumor with a dismal prognosis. We evaluated the efficacy of multimodal therapy in children with ATRT. PROCEDURE: Nine children diagnosed with cranial ATRT, who received multimodal therapy between 2005 and 2014, including surgical resection followed by radiotherapy (RT), systemic chemotherapy (CT), and high-dose chemotherapy/stem cell transplantation (HDCT/SCT), were analyzed retrospectively...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28550054/pgbd5-is-a-dna-transposase-sufficient-for-malignant-transformation
#13
(no author information available yet)
PGBD5 binds to PSS sequences to promote somatic genomic rearrangements in rhabdoid tumor cells.
May 26, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/28541826/ini-expressing-epithelioid-sarcoma-with-osteoclastic-giant-cells-in-a-child-a-case-report-with-summary-of-prior-published-cases
#14
Riju Bhattacharyya, Ranajoy Ghosh, Koushik Saha, Uttara Chatterjee
BACKGROUND: Epithelioid sarcoma is a heterogeneous tumor with 2 subtypes, classic and proximal. The proximal variant is more aggressive and occurs in proximal location in young adults. CASE REPORT: We present a proximal epithelioid sarcoma in the leg of an 8 year old girl with rhabdoid morphology and scattered osteoclastic giant cells. Nuclear INI-1 was retained. Despite wide local excision, local recurrence occurred at 8 months. Following re-excision, she developed a chest wall metastasis after 9 months...
May 25, 2017: Fetal and Pediatric Pathology
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
#15
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/28503314/atypical-teratoid-rhabdoid-tumor-of-lumbar-spine-in-a-toddler-child
#16
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/28498283/synovial-sarcoma-of-peripheral-nerves-analysis-of-15-cases
#17
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)...
August 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28482042/bap1-mutations-in-high-grade-meningioma-implications-for-patient-care
#18
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/28469334/clinical-profile-of-pediatric-oncology-patients-treated-by-external-beam-radiotherapy-an-institutional-experience
#19
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/28452074/clinicopathologic-features-and-immunohistochemical-spectrum-of-11-cases-of-epithelioid-malignant-peripheral-nerve-sheath-tumors-including-ini1-smarcb1-results-and-braf-v600e-analysis
#20
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
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