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malignant rhabdoid tumor

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https://www.readbyqxmd.com/read/28877071/uncommon-localization-of-extrarenal-xp11-2-translocation-associated-renal-cell-carcinoma-rcc-case-report
#1
Jaudah Ahmed Al-Maghrabi, Mohamad Nidal Khabaz
The World Health Organization has recognized Xp11.2 translocation-associated renal cell carcinoma (RCC) as a distinct neoplasm that arises within the kidney. Although many reports of extrarenal carcinoma may be found in the literature, to the best of our knowledge, Xp11 translocation-associated RCC with intact kidneys has not been documented. This report describes a multilobulated right retroperitoneal soft tissue mass (7.9×5.3×12.6 cm) of a 37-year-old man complaining of abdominal pain in the right side...
September 4, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28843054/high-dose-treatment-for-malignant-rhabdoid-tumor-of-the-kidney-no-evidence-for-improved-survival-the-gesellschaft-f%C3%A3-r-p%C3%A3-diatrische-onkologie-und-h%C3%A3-matologie-gpoh-experience
#2
Rhoikos Furtwängler, Leo Kager, Patrick Melchior, Christian Rübe, Martin Ebinger, Nasenien Nourkami-Tutdibi, Felix Niggli, Steven Warmann, Jochen Hubertus, Gabriele Amman, Ivo Leuschner, Christian Vokuhl, Norbert Graf, Michael C Frühwald
BACKGROUND: Malignant rhabdoid tumor of the kidney (MRTK) is the most aggressive childhood renal tumor with overall survival (OS) rates ranging from 22% to 42%. Whether high-dose chemotherapy with autologous stem-cell transplantation (HDSCT) in an intensive first-line treatment offers additional benefit is an ongoing discussion. METHODS: A retrospective analysis of all 58 patients with MRTK from Austria, Switzerland, and Germany treated in the framework of consecutive, prospective renal/rhabdoid tumor studies SIOP9/GPO, SIOP93-01/GPOH (where SIOP is International Society of Pediatric Oncology and GPOH is German Society of Pediatric Oncology and Hematology), SIOP2001/GPOH, and European Rhabdoid Tumor Registry from 1991 to 2014...
August 26, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28820306/prognostic-value-of-estrogen-receptor-in-who-grade-iii-meningioma-a-long-term-follow-up-study-from-a-single-institution
#3
Lingyang Hua, Hongda Zhu, Jingrun Li, Hailiang Tang, Dapeng Kuang, Yin Wang, Feng Tang, Xiancheng Chen, Liangfu Zhou, Qing Xie, Ye Gong
OBJECTIVE Malignant meningioma is rare and classified as Grade III in the WHO classification of CNS tumors. However, the presence of estrogen receptor (ER) in WHO Grade III meningiomas and its correlation with patients' outcomes are still unclear. In this single-center cohort study, the authors analyzed clinical features, treatment, and prognosis of these malignant tumors in patients with long-term follow-up. METHODS A total of 87 patients who were pathologically diagnosed with WHO Grade III meningiomas between 2003 and 2008 were enrolled in this study and followed for at least 7 years...
August 18, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28815340/intradural-lumbosacral-malignant-extrarenal-rhabdoid-tumor-a-case-report
#4
Richard Justin Garling, Rasanjeet Singh, Carolyn Harris, Abilash Haridas
BACKGROUND: Rhabdoid tumors are highly malignant tumors predominantly affecting the pediatric population. When these tumors occur outside of the kidneys, they are referred to as malignant extrarenal rhabdoid tumors (MERT), a rare highly aggressive subtype. Less commonly, these tumors involve the neuro-axis. OBJECTIVE: Here we present a case of a 15-year-old girl with intradural MERT of the lumbosacral spine who presented with back pain, sudden worsening of lower extremity strength, and complete loss of bowel and bladder control...
August 16, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28797981/rhabdoid-meningioma-arising-concurrent-in-pulmonary-and-intracranial-with-a-rare-malignant-clinical-progression-case-report-and-literature-review
#5
Peng Zhao, Ning Li, Jinfeng Cao, Xiangtao Lin, Changhu Liang
BACKGROUND: Rhabdoid meningioma (RM) is an unusual variant of meningioma, classified as WHO grade III. Although its recurrence is common, extracranial metastasis is rare and usually misdiagnosed. The transfer mechanism and pathway are ambiguous; once it develops, there is a poor prognosis and no effective management. The present case is the first report on concurrent intracranial and pulmonary RM with rapid and widespread metastasis. We hope this report can be a helpful reference for clinicians and radiologists...
August 7, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28783147/marrow-ablative-chemotherapy-followed-by-tandem-autologous-hematopoietic-cell-transplantation-in-pediatric-patients-with-malignant-brain-tumors
#6
J A Guerra, G Dhall, A Marachelian, E Castillo, J Malvar, K Wong, R Sposto, J L Finlay
To improve survival in young children with malignant brain tumors, irradiation-avoiding or -minimizing marrow-ablative chemotherapy (HDCx) with autologous hematopoietic cell transplantation (AuHCT) has been investigated. We evaluated the outcome of 44 children with malignant brain tumors treated with HDCx and tandem AuHCT at Children's Hospital Los Angeles between June 1999 and July 2012. Forty-four children with malignant brain tumors were studied. Twenty-one had medulloblastoma/primitive neuro-ectodermal tumor, eight atypical teratoid/rhabdoid tumor (ATRT), five high-grade glioma, four malignant germ cell tumor, three ependymoma and three choroid plexus carcinoma...
August 7, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28744687/lin28a-a-sensitive-immunohistochemical-marker-for-embryonal-tumor-with-multilayered-rosettes-etmr-is-also-positive-in-a-subset-of-atypical-teratoid-rhabdoid-tumor-at-rt
#7
Shilpa Rao, R T Rajeswarie, T Chickabasaviah Yasha, Bevinahalli N Nandeesh, Arimappamagan Arivazhagan, Vani Santosh
INTRODUCTION: CNS embryonal tumors comprise a group of highly malignant neoplasms with a wide spectrum of histomorphological entities that includes Medulloblastoma (MB), Atypical Teratoid/Rhabdoid Tumor (AT/RT), Neuroblastoma (NB), Ganglioneuroblastoma (GNB), Embryonal Tumor with Multilayered Rosettes (ETMR), and the embryonal tumor-Not Otherwise Specified (NOS). The entity ETMR includes previously described histopathologic patterns-Embryonal Tumor with Abundant Neuropil and True Rosettes (ETANTR), Ependymoblastoma (EBL), and Medulloepithelioma (MEPL)...
July 25, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28731921/loss-of-cdkn1c-in-a-recurrent-atypical-teratoid-rhabdoid-tumor
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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...
October 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28648942/dedifferentiated-chondrosarcoma-of-bone-with-prominent-rhabdoid-component
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
(no author information available yet)
PGBD5 binds to PSS sequences to promote somatic genomic rearrangements in rhabdoid tumor cells.
May 26, 2017: Cancer Discovery
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