2,322 results match your criteria Malignant Rhabdoid Tumor


MDM2 and MDM4 are Therapeutic Vulnerabilities in Malignant Rhabdoid Tumors.

Cancer Res 2019 Feb 12. Epub 2019 Feb 12.

Comprehensive Cancer Center, St. Jude Children's Research Hospital

Malignant rhabdoid tumors (MRT) are highly aggressive pediatric cancers that respond poorly to current therapies. In this study, we screened several MRT cell lines with large-scale RNAi, CRISPR-Cas9, and small-molecule libraries to identify potential drug targets specific for these cancers. We discovered MDM2 and MDM4, the canonical negative regulators of p53, as significant vulnerabilities. Read More

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http://dx.doi.org/10.1158/0008-5472.CAN-18-3066DOI Listing
February 2019
1 Read

Expression of cyclin D1 in clear cell sarcoma of kidney. Is it useful in differentiating it from its histological mimics?

Diagn Pathol 2019 Feb 8;14(1):13. Epub 2019 Feb 8.

Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan.

Background: Clear cell sarcoma of the kidney (CCSK) is a rare malignant pediatric renal neoplasm with a heterogeneous histological appearance which often results in misdiagnosis. There are no specific immunohistochemical markers which can help in differentiating CCSK from other pediatric renal neoplasms. Recently Cyclin D1 has been investigated as a possible marker in this regard. Read More

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http://dx.doi.org/10.1186/s13000-019-0790-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6368701PMC
February 2019
1 Read

Freiburg Neuropathology Case Conference : Posterior Fossa Mass in an Infant.

Clin Neuroradiol 2019 Feb 7. Epub 2019 Feb 7.

Department of Neuropathology, Medical Centre-University of Freiburg, University of Freiburg, Freiburg, Germany.

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http://dx.doi.org/10.1007/s00062-019-00759-3DOI Listing
February 2019
1 Read

Advances in Diagnostic Immunohistochemistry for Primary Tumors of the Central Nervous System.

Authors:
David M Meredith

Adv Anat Pathol 2019 Feb 1. Epub 2019 Feb 1.

Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.

As genomic characterization becomes increasingly necessary for accurate diagnosis of tumors of the central nervous system, identification of rapidly assessible biomarkers is equally important to avoid excessive cost and delay in initiation of therapy. This article reviews novel immunohistochemical markers that may be used to determine mutation status, activation of signaling pathways, druggable targets, and cell lineage in many diverse tumor types. In particular, recently added entities to the 2016 WHO classification of central nervous system tumors will be addressed, including IDH-mutant gliomas, diffuse midline glioma, epithelioid glioblastoma, angiocentric glioma, RELA-rearranged ependymoma, embryonal tumors (medulloblastoma, atypical teratoid/rhabdoid tumor, pineoblastoma, embryonal tumor with multilayered rosettes, and other genetically defined high-grade neuroepithelial tumors), and meningiomas associated with germline alterations. Read More

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http://dx.doi.org/10.1097/PAP.0000000000000225DOI Listing
February 2019
1 Read

SWI/SNF Complex-Deficient Soft Tissue Neoplasms: A Pattern-Based Approach to Diagnosis and Differential Diagnosis.

Authors:
Abbas Agaimy

Surg Pathol Clin 2019 Mar;12(1):149-163

Institute of Pathology, Friedrich-Alexander University Erlangen-Nürnberg (FAU), University Hospital, Krankenhausstrasse 8-10, 91054 Erlangen, Germany. Electronic address:

Loss of different components of the Switch/sucrose nonfermentable (SWI/SNF) chromatin remodeling complex has been increasingly recognized as a central molecular event driving the initiation and/or dedifferentiation of mostly lethal but histogenetically diverse neoplasms in different body organs. This review summarizes and discusses the morphologic and phenotypic diversity of primary soft tissue neoplasms characterized by SWI/SNF complex deficiency with an emphasis on convergent and divergent cytoarchitectural patterns. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18759181183006
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http://dx.doi.org/10.1016/j.path.2018.10.006DOI Listing
March 2019
5 Reads

Atypical teratoid rhabdoid tumor mimicking type II neurofibromatosis: A case report.

Medicine (Baltimore) 2019 Feb;98(5):e14308

Department of Neurological Surgery, Children's Hospital, Zhejiang University School of Medicine, China.

Rationale: Brain magnetic resonance imaging (MRI) images of atypical teratoid rhabdoid tumor (ATRT) often present heterogeneous signals of various cells without remarkable features of the disease. We describe a unique case of atypical brain MRI images presenting as an type II neurofibromatosis and explore some diagnostic hints.

Patient Concerns: A 1-year-and-7-month-old boy admitted to our department with a 7-day history of drowsiness and 2-day history of emesis, and his presenting complaint was repeated vomit. Read More

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http://dx.doi.org/10.1097/MD.0000000000014308DOI Listing
February 2019
1 Read

[Clinicopathological characteristics of fumarate hydratase-deficient renal cell carcinoma].

Zhonghua Bing Li Xue Za Zhi 2019 Feb;48(2):120-126

Department of Pathology, the Affiliated Hospital of Qingdao University, Qingdao 266003, China.

To investigate the clinicopathologic characteristics, molecular and genetic features, differential diagnoses and prognosis of fumarate hydratase-deficient renal cell carcinoma (FH-RCC). The immunohistochemical (IHC) expression of FH in 391 renal neoplasms in tissue chips collected from the Affiliated Hospital of Qingdao University and 971 Hospital of PLA Navy from January 2011 to December 2017 was evaluated. The clinicopathologic data of eight FH negative cases were collected. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0529-5807.2019.02.009DOI Listing
February 2019
6 Reads

Renal Medullary Carcinoma: a Report of the Current Literature.

Curr Urol Rep 2019 Jan 17;20(1). Epub 2019 Jan 17.

FINAER (Foundation for Research and Assistance in Kidney Disease), Palestina 525, 1182, Buenos Aires, Argentina.

Purpose Of The Review: We present an updated report of renal medullary carcinoma (RMC), a rare and aggressive condition.

Recent Findings: There is a majority of male patients, of African descent, in the second or third decade of life. In differential diagnosis, other tumors, such as malignant rhabdoid tumor (MRT), vinculin-anaplastic lymphoma kinase (VCL-ALK) translocation renal cell carcinoma, and collecting duct carcinoma, may present difficulties. Read More

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http://dx.doi.org/10.1007/s11934-019-0865-9DOI Listing
January 2019
3 Reads

SMARCB1- and vimentin-positive esophageal carcinoma with undifferentiated components, rhabdoid features, and a good prognosis: a case report.

Surg Case Rep 2019 Jan 16;5(1). Epub 2019 Jan 16.

1st Department of Surgery, Faculty of Medicine, University of Fukui, 23-3 Matsuokashimoaizuki, Yoshida-gun, Eiheiji-cho, Fukui, 910-1193, Japan.

Background: Undifferentiated carcinoma of the esophagus with rhabdoid features is a very rare histologic finding that is occasionally associated with the loss of SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1); however, until now, few survey reports of this type of tumor have been published. In this study, we describe a case of esophageal carcinoma with undifferentiated components and rhabdoid features that was exclusively positive for vimentin and SMARCB1 in a patient with prolonged survival.

Case Presentation: A 67-year-old man complained of a stomachache and loss of appetite persisting for 1 month. Read More

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https://surgicalcasereports.springeropen.com/articles/10.118
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http://dx.doi.org/10.1186/s40792-019-0562-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6335233PMC
January 2019
3 Reads

Multimodal Management of Congenital Orbital Malignant Rhabdoid Tumor: Review of Literature and Report of a Rare Case.

J Pediatr Hematol Oncol 2019 Jan 4. Epub 2019 Jan 4.

Pathology, All India Institute of Medical Sciences, New Delhi, India.

Background: Malignant rhabdoid tumor (MRT) is a rare and aggressive tumor with a dismal prognosis. It commonly arises in the brain (65%), soft tissues (26%), and the kidney (9%). Primary orbital involvement is extremely rare. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001402DOI Listing
January 2019
4 Reads

Comprehensive Analysis of Chromatin States in Atypical Teratoid/Rhabdoid Tumor Identifies Diverging Roles for SWI/SNF and Polycomb in Gene Regulation.

Cancer Cell 2019 Jan 27;35(1):95-110.e8. Epub 2018 Dec 27.

Hopp-Children's Cancer Center at the NCT Heidelberg (KiTZ), 69120 Heidelberg, Germany; Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), Im Neuenheimer Feld 280, 69120 Heidelberg, Germany. Electronic address:

Biallelic inactivation of SMARCB1, encoding a member of the SWI/SNF chromatin remodeling complex, is the hallmark genetic aberration of atypical teratoid rhabdoid tumors (ATRT). Here, we report how loss of SMARCB1 affects the epigenome in these tumors. Using chromatin immunoprecipitation sequencing (ChIP-seq) on primary tumors for a series of active and repressive histone marks, we identified the chromatin states differentially represented in ATRTs compared with other brain tumors and non-neoplastic brain. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15356108183053
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http://dx.doi.org/10.1016/j.ccell.2018.11.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6341227PMC
January 2019
4 Reads

Imaging features of spinal atypical teratoid rhabdoid tumors in children.

Medicine (Baltimore) 2018 Dec;97(52):e13808

Department of Pathology, Guangzhou Women and Children's Medical Center, Guangzhou, Guangdong, China.

This study aims to analyze and summarize the imaging features of spinal atypical teratoid/rhabdoid tumors (AT/RT) in children.Imaging features in 8 children with spinal AT/RT confirmed by surgical pathology were retrospectively analyzed. All patients had underwent total spine 3. Read More

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http://dx.doi.org/10.1097/MD.0000000000013808DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314652PMC
December 2018
7 Reads

Broad Spectrum Activity of the Checkpoint Kinase 1 Inhibitor Prexasertib as a Single Agent or Chemopotentiator Across a Range of Preclinical Pediatric Tumor Models.

Clin Cancer Res 2018 Dec 18. Epub 2018 Dec 18.

Eli Lilly and Company, Lilly Corporate Center, Indianapolis, Indiana.

Checkpoint kinase 1 (CHK1) inhibitors potentiate the DNA-damaging effects of cytotoxic therapies and/or promote elevated levels of replication stress, leading to tumor cell death. Prexasertib (LY2606368) is a CHK1 small-molecule inhibitor under clinical evaluation in multiple adult and pediatric cancers. In this study, prexasertib was tested in a large panel of preclinical models of pediatric solid malignancies alone or in combination with chemotherapy. Read More

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http://dx.doi.org/10.1158/1078-0432.CCR-18-2728DOI Listing
December 2018
3 Reads

Primary Pulmonary Meningioma With Rhabdoid Features.

Int J Surg Pathol 2018 Dec 18:1066896918819257. Epub 2018 Dec 18.

1 Vilnius University, Vilnius, Lithuania.

Only 1% to 2% of meningiomas have primary extrameningeal location, which is mostly head and neck region. Primary pulmonary meningiomas (PPMs) are even more uncommon with up to 50 cases reported in the literature. Only 5 cases of PPM with confirmed or possible malignancy have been previously described. Read More

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http://dx.doi.org/10.1177/1066896918819257DOI Listing
December 2018
0.961 Impact Factor

A Systematic Review of Atypical Teratoid Rhabdoid Tumor in Adults.

Front Oncol 2018 28;8:567. Epub 2018 Nov 28.

Division of Neuropathology, University of Alberta Hospital, Edmonton, AB, Canada.

Atypical teratoid/rhabdoid tumor in adults is a relatively rare malignant neoplasm. It is characterized by the presence of rhabdoid cells in combination with loss of either the INI1 or BRG1protein from the tumor cells. A systematic review was conducted using MEDLINE using the terms "atypical teratoid rhabdoid tumor" AND "adult. Read More

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https://www.frontiersin.org/article/10.3389/fonc.2018.00567/
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http://dx.doi.org/10.3389/fonc.2018.00567DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6279935PMC
November 2018
12 Reads

Immunohistochemical detection of ALK protein identifies APC mutated medulloblastoma and differentiates the WNT-activated medulloblastoma from other types of posterior fossa childhood tumors.

Brain Tumor Pathol 2019 Jan 6;36(1):1-6. Epub 2018 Dec 6.

Department of Experimental and Clinical Pathology, Mossakowski Medical Research Centre, Polish Academy of Sciences, A. Pawińskiego 5 Street, 02-106, Warsaw, Poland.

Expression of the ALK gene strongly correlates with the WNT-activated medulloblastomas, which are routinely identified by detection of CTNNB1 mutation. However, some tumors have mutations in other than CTNNB1 genes. Therefore, we investigated if ALK expression may identify WNT-activated tumors without CTNNB1 mutation. Read More

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http://dx.doi.org/10.1007/s10014-018-0331-2DOI Listing
January 2019
1 Read

Desmoplastic Small Round Cell Tumors With Atypical Presentations: A Report of 34 Cases.

Int J Surg Pathol 2018 Dec 6:1066896918817140. Epub 2018 Dec 6.

2 Mayo Clinic, Rochester, MN, USA.

Objectives: Desmoplastic small round cell tumor (DSRCT) is an aggressive round cell sarcoma that arises in the abdominal cavity/pelvis of young males. We sought to expand its clinicopathologic spectrum.

Methods: Cases of DSRCT presenting in patients >30 years of age or tumors arising outside of the abdominal cavity/pelvis were retrieved. Read More

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http://dx.doi.org/10.1177/1066896918817140DOI Listing
December 2018
8 Reads

Overall Survival of Primary Intracranial Atypical Teratoid Rhabdoid Tumor Following Multimodal Treatment: A Pooled Analysis of Individual Patient Data.

Neurosurg Rev 2018 Dec 8. Epub 2018 Dec 8.

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Tiantan Xili 6, Dongcheng District, Beijing, People's Republic of China.

No standard treatment protocol to guide the management of the primary central nervous system atypical teratoid rhabdoid tumors (ATRTs). To evaluate the efficacy of GTR (gross total resection), RT (radiotherapy), CCMT (conventional chemotherapy), or intensified chemotherapy (ICMT) and verify the optimal treatment strategy. A total of 501 cases (18 cases from our center and 483 cases from published literature) were eligible for analysis. Read More

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http://dx.doi.org/10.1007/s10143-018-1055-9DOI Listing
December 2018
1 Read

Spontaneous Regression of Atypical Teratoid Rhabdoid Tumor Without Therapy in a Patient With Uncommon Regional Inactivation of SMARCB1 ( hSNF5/INI1).

Pediatr Dev Pathol 2018 Nov 23:1093526618814696. Epub 2018 Nov 23.

3 Department of Pathology, Baylor College of Medicine, Texas Children's Hospital, Houston, Texas.

Atypical teratoid/rhabdoid tumor (ATRT) is a high-grade central nervous system tumor, with poor prognosis despite intensive multimodal therapy. Loss of nuclear immunostaining for INI1 due to inactivation of the hSNF5/INI1 tumor suppressor gene is pathognomonic of ATRT. We present a patient with congenital ATRT, who had spontaneous tumor regression without therapy, and is disease-free 4 years later. Read More

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http://journals.sagepub.com/doi/10.1177/1093526618814696
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http://dx.doi.org/10.1177/1093526618814696DOI Listing
November 2018
3 Reads

Functional relevance of genes predicted to be affected by epigenetic alterations in atypical teratoid/rhabdoid tumors.

J Neurooncol 2019 Jan 16;141(1):43-55. Epub 2018 Nov 16.

Institute of Neuropathology, University Hospital Münster, Pottkamp 2, 48149, Münster, Germany.

Purpose: Atypical teratoid/rhabdoid tumor (ATRT) is a highly malignant brain tumor predominantly arising in infants. Mutations of SWI/SNF chromatin remodeling complex members SMARCB1/INI1 or (rarely) SMARCA4/Brg1 are the sole recurrent genetic lesions. Epigenetic studies revealed a large number of genes predicted to be affected by differential histone modifications in ATRT, but the role of these genes in the biology of ATRT remains uncertain. Read More

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http://link.springer.com/10.1007/s11060-018-03018-6
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http://dx.doi.org/10.1007/s11060-018-03018-6DOI Listing
January 2019
8 Reads

Less known aspects of central hypothyroidism: Part 1 - Acquired etiologies.

J Clin Transl Endocrinol 2018 Dec 26;14:25-33. Epub 2018 Sep 26.

Department of Medical Endocrinology and Metabolism, Rigshospitalet, National University Hospital, Copenhagen University, Copenhagen, Denmark.

Central hypothyroidism (CH) is a rare cause of hypothyroidism. CH is frequently overlooked, as its clinical picture is subtle and includes non-specific symptoms; furthermore, if measurement of TSH alone is used to screen for thyroid function, TSH concentrations can be normal or even above the upper normal reference limit. Indeed, certain patients are at risk of developing CH, such as those with a pituitary adenoma or hypophysitis, those who have been treated for a childhood malignancy, have suffered a head trauma, sub-arachnoid hemorrhage or meningitis, and those who are on drugs capable to reduce TSH secretion. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22146237183006
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http://dx.doi.org/10.1016/j.jcte.2018.09.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6205405PMC
December 2018
8 Reads

Sellar Atypical Teratoid/Rhabdoid Tumor Presenting with Subarachnoid and Intraventricular Hemorrhage.

World Neurosurg 2018 Nov 4. Epub 2018 Nov 4.

Department of Neurosurgery, Henry Ford Health System, Detroit, Michigan, USA.

Background: Atypical teratoid/rhabdoid tumors (ATRT) are uncommon malignancies of the central nervous system and are often difficult to distinguish radiographically and pathologically from other common tumors. We present the first case of sellar ATRT presenting with subarachnoid hemorrhage (SAH) and intraventricular hemorrhage (IVH).

Case Description: A 62-year-old woman, who had presented with symptoms of headache, diabetes insipidus, hypothyroidism, and seizures, was found to have a sellar tumor with hemorrhagic transformation. Read More

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http://dx.doi.org/10.1016/j.wneu.2018.10.198DOI Listing
November 2018
7 Reads

Polo-Like Kinase 4 (PLK4) Is Overexpressed in Central Nervous System Neuroblastoma (CNS-NB).

Bioengineering (Basel) 2018 Nov 4;5(4). Epub 2018 Nov 4.

Division of Pediatric Neurosurgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL 60611, USA.

Neuroblastoma (NB) is the most common extracranial solid tumor in pediatrics, with rare occurrences of primary and metastatic tumors in the central nervous system (CNS). We previously reported the overexpression of the polo-like kinase 4 (PLK4) in embryonal brain tumors. PLK4 has also been found to be overexpressed in a variety of peripheral adult tumors and recently in peripheral NB. Read More

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http://www.mdpi.com/2306-5354/5/4/96
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http://dx.doi.org/10.3390/bioengineering5040096DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6315664PMC
November 2018
10 Reads

Second rhabdoid tumor 8 years after treatment of atypical teratoid/rhabdoid tumor in a child with germline SMARCB1 mutation.

Pediatr Blood Cancer 2019 Mar 4;66(3):e27546. Epub 2018 Nov 4.

Pediatric Brain Tumour Program, The Hospital for Sick Children, University of Toronto, Ontario, Canada.

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http://doi.wiley.com/10.1002/pbc.27546
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http://dx.doi.org/10.1002/pbc.27546DOI Listing
March 2019
8 Reads

Implications of Programmed Death Ligand-1 Positivity in Non-Clear Cell Renal Cell Carcinoma.

J Kidney Cancer VHL 2018 13;5(4):6-13. Epub 2018 Oct 13.

Department of Genitourinary Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA.

The purpose of this study was to assess the prognostic value of programmed death ligand-1 (PD-L1) positivity in a non-clear cell renal cell carcinoma (non-ccRCC) cohort. PD-L1 expression was evaluated by immunohistochemistry (IHC) using formalin-fixed paraffin-embedded (FFPE) specimens from 45 non-ccRCC patients with available tissue. PD-L1 positivity was defined as ≥1% of staining. Read More

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http://jkcvhl.com/index.php/jkcvhl/article/view/107
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http://dx.doi.org/10.15586/jkcvhl.2018.107DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186848PMC
October 2018
8 Reads

New molecular targets in meningiomas: the present and the future.

Curr Opin Neurol 2018 Dec;31(6):740-746

Divisions of Neuro-Oncology and Hematology/Oncology, Departments of Neurology and Medicine, Massachusetts General Hospital/Harvard Medical School.

Purpose Of Review: Meningiomas, the most common primary brain tumor, have historically been managed with surgery and radiation. Traditional chemotherapy has not been effective. Fortunately, recent advances in genetic sequencing have led to an improved understanding of the molecular drivers in meningioma. Read More

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http://Insights.ovid.com/crossref?an=00019052-201812000-0001
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http://dx.doi.org/10.1097/WCO.0000000000000615DOI Listing
December 2018
9 Reads

An Antenatally Detected Pure Malignant Rhabdoid Tumor of the Bladder.

Urology 2019 Jan 23;123:221-223. Epub 2018 Oct 23.

University of Alberta, Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Edmonton, Alberta, Canada.

Pediatric malignant rhabdoid tumors are a rare entity with poor prognosis. The extrarenal variants of this disease are sparsely presented within the literature. Our case represents, to our knowledge, the first antenatally detected bladder-variant. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00904295183110
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http://dx.doi.org/10.1016/j.urology.2018.10.019DOI Listing
January 2019
7 Reads

Extensive Molecular and Clinical Heterogeneity in Patients With Histologically Diagnosed CNS-PNET Treated as a Single Entity: A Report From the Children's Oncology Group Randomized ACNS0332 Trial.

J Clin Oncol 2018 Oct 17:JCO2017764720. Epub 2018 Oct 17.

Eugene I. Hwang and Roger J. Packer, Children's National Medical Center, Washington, DC; Marcel Kool, Lukas Chavez, Sebastian Brabetz, David T.W. Jones, and Stefan M. Pfister, German Cancer Research Center; David Capper, Andreas von Deimling, and Stefan M. Pfister, University Hospital Heidelberg; Stefan M. Pfister, National Center for Tumor Diseases, Heidelberg; David Capper, Charité Medical University, Berlin, Germany; Peter C. Burger, Johns Hopkins University, Baltimore, MD; Chris Williams-Hughes, Children's Oncology Group, Littleton, CO; Catherine Billups, Yimei Li, and Amar Gajjar, St Jude Children's Research Hospital, Memphis, TN; Linda Heier, Weill Cornell Medical College, New York, NY; Alok Jaju, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL; Jeff Michalski, Washington University School of Medicine, St. Louis, MO; Sarah Leary and James M. Olson, Seattle Children's Hospital and Research Institute; Sarah Leary and James M. Olson, Fred Hutchinson Cancer Research Center, Seattle, WA; Tianni Zhou, California State University, Long Beach, Long Beach, CA; Maryam Fouladi, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; and Ian F. Pollack, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA.

Purpose: Children with histologically diagnosed high-risk medulloblastoma, supratentorial primitive neuroectodermal tumor of the CNS (CNS-PNET), and pineoblastoma (PBL) have had poor survival despite intensive treatment. We included these patients in this Children's Oncology Group trial. Molecular profiling later revealed tumor heterogeneity that was not detectable at protocol inception. Read More

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http://dx.doi.org/10.1200/JCO.2017.76.4720DOI Listing
October 2018
14 Reads

Clinical Benefit to an Aurora A Kinase Inhibitor in a Patient with Metastatic Integrase Interactor 1-Deficient Carcinoma.

Oncologist 2019 Feb 8;24(2):146-150. Epub 2018 Oct 8.

Department of Oncology and Radiological Sciences, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

Integrase interactor 1 (INI-1)-deficient carcinoma is a rare cancer characterized by the loss of the SWItch/Sucrose Non-Fermentable-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 gene () and tends to follow an aggressive clinical course. There is no currently available standard therapy option, although a few promising treatment strategies, including enhancer of zeste homolog 2 (EZH2) inhibition, are under active investigation. This report describes a 30-year-old woman with INI-1-deficient carcinoma who progressed on combination chemotherapy and an EZH2 inhibitor. Read More

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http://dx.doi.org/10.1634/theoncologist.2018-0279DOI Listing
February 2019
16 Reads

FNA of epithelioid sarcoma: Curie Institute experience and critical review of the literature.

Cancer Cytopathol 2018 Nov 6;126(11):934-941. Epub 2018 Oct 6.

Department of Pathology, Curie Institute, Paris, France.

Background: Epithelioid sarcoma (ES) is a rare mesenchymal tumor that is divided into 2 types: classic and proximal. To the authors' knowledge, ES has been poorly studied in cytology, with fewer than 50 cases reported to date. The objective of the current study was to analyze the cytological and immunohistochemical information regarding 5 cases of ES. Read More

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http://doi.wiley.com/10.1002/cncy.22052
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http://dx.doi.org/10.1002/cncy.22052DOI Listing
November 2018
14 Reads
3.354 Impact Factor

Cytologic Features of SMARCA4-Deficient Thoracic Sarcoma: A Case Report and Comparison with Other SWI/SNF Complex-Deficient Tumors.

Acta Cytol 2018 4;62(5-6):456-462. Epub 2018 Oct 4.

Division of Molecular Pathology, Faculty of Medicine, Tottori University, Yonago,

Background: SMARCA4-deficient thoracic sarcoma is a recently proposed entity of soft tissue tumors associated with an extremely poor prognosis. Its cytologic features have not been well described in the literature yet.

Case: A woman in her early 30s who presented with chest pain was found to have a tumor in the right chest wall. Read More

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http://dx.doi.org/10.1159/000493335DOI Listing
January 2019
1 Read

Ovarian small cell carcinoma in one of a pair of monozygous twins.

Fam Cancer 2018 Oct 4. Epub 2018 Oct 4.

Cancer Research Program, Research Institute of the McGill University Health Centre, Montreal, QC, Canada.

One of a pair of monozygous twins was diagnosed and died of small cell carcinoma of the ovary of hypercalcemic type (SCCOHT) at the age of 30 years. Her sister remained unaffected and was very concerned about her risk for developing SCCOHT. By performing comprehensive molecular analysis using whole exome sequencing (WES) approach, we showed that the deceased twin's tumour has bi-allelic somatic genetic defects (a pathogenic frameshift deletion in SMARCA4 and LOH on chr19p). Read More

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http://dx.doi.org/10.1007/s10689-018-0108-0DOI Listing
October 2018

Pediatric Suprasellar Atypical Teratoid Rhabdoid Tumor Arising from the Third Ventricle: A Rare Tumor at a Very Rare Location.

Asian J Neurosurg 2018 Jul-Sep;13(3):873-876

Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.

Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant tumor of the central nervous system, commonly affecting children below 3 years of age, with around 300 cases reported in the literature. Suprasellar area is a very rare location for such tumor in the pediatric population, with technical difficulties in complete excision. Third ventricular ATRT is very rare. Read More

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http://dx.doi.org/10.4103/ajns.AJNS_350_16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6159048PMC
October 2018
8 Reads

Advances in the classification of pediatric brain tumors through DNA methylation profiling: From research tool to frontline diagnostic.

Cancer 2018 Nov 26;124(21):4168-4180. Epub 2018 Sep 26.

Division of Neuro-Oncology, Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Despite significant improvements in pediatric brain tumor therapy and outcome, too many children still die of disease, and too many survivors experience significant sequelae as a result of conventional therapies. The molecular characterization of pediatric brain tumors has afforded tremendous insight into the basic biology and clinical management of these deadly childhood diseases. Genomic, epigenomic, and transcriptional profiling have facilitated the identification of significant heterogeneity among previously uniform disease entities. Read More

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http://doi.wiley.com/10.1002/cncr.31583
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http://dx.doi.org/10.1002/cncr.31583DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6263826PMC
November 2018
11 Reads

Embryonal Tumors of the Central Nervous System in Children: The Era of Targeted Therapeutics.

Bioengineering (Basel) 2018 Sep 23;5(4). Epub 2018 Sep 23.

Department of Pediatrics, MD Anderson Cancer Center, Houston, TX 77030, USA.

Embryonal tumors (ET) of the central nervous system (CNS) in children encompass a wide clinical spectrum of aggressive malignancies. Until recently, the overlapping morphological features of these lesions posed a diagnostic challenge and undermined discovery of optimal treatment strategies. However, with the advances in genomic technology and the outpouring of biological data over the last decade, clear insights into the molecular heterogeneity of these tumors are now well delineated. Read More

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http://dx.doi.org/10.3390/bioengineering5040078DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6315657PMC
September 2018
2 Reads

Radiation-induced glioblastoma with rhabdoid characteristics following treatment for medulloblastoma: A case report and review of the literature.

Mol Clin Oncol 2018 Oct 20;9(4):415-418. Epub 2018 Aug 20.

Department of Radiotherapy, 306th Hospital of PLA, Beijing 100101, P.R. China.

Glioblastoma multiforme (GBM) is a high-grade glioma that may develop from several other central nervous system tumors after radiation therapy. We herein report a case of GBM occurring 8 years after radiation therapy for medulloblastoma. The secondary tumor was histologically distinctly different from the primary tumor. Read More

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http://dx.doi.org/10.3892/mco.2018.1703DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6142298PMC
October 2018
2 Reads

Inhibition of MYC attenuates tumor cell self-renewal and promotes senescence in SMARCB1-deficient Group 2 atypical teratoid rhabdoid tumors to suppress tumor growth in vivo.

Int J Cancer 2019 Apr 10;144(8):1983-1995. Epub 2019 Jan 10.

Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora, CO, 80045, USA.

Loss of SMARCB1 is the hallmark genetic event that characterizes rhabdoid tumors in children. Rhabdoid tumors of the brain (ATRT) occur in young children and are particularly challenging with poor long-term survival. SMARCB1 is a member of the SWI/SNF chromatin remodeling complex that is responsible for determining cellular pluripotency and lineage commitment. Read More

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http://dx.doi.org/10.1002/ijc.31873DOI Listing
April 2019
1 Read

Volumetric voxelwise apparent diffusion coefficient histogram analysis for differentiation of the fourth ventricular tumors.

Neuroradiol J 2018 Dec 19;31(6):554-564. Epub 2018 Sep 19.

1 Department of Radiology and Biomedical Imaging, Yale School of Medicine, New Haven, USA.

Purpose: We applied voxelwise apparent diffusion coefficient (ADC) histogram analysis in addition to structural magnetic resonance imaging (MRI) findings and patients' age for differentiation of intraaxial posterior fossa tumors involving the fourth ventricle.

Participants And Methods: Pretreatment MRIs of 74 patients with intraaxial brain neoplasm involving the fourth ventricle, from January 1, 2004 to December 31, 2015, were reviewed. The tumor solid components were segmented and voxelwise ADC histogram variables were determined. Read More

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http://dx.doi.org/10.1177/1971400918800803DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6243467PMC
December 2018
8 Reads

Safety of high-dose intravenous mistletoe therapy in pediatric cancer patients: A case series.

Complement Ther Med 2018 Oct 11;40:198-202. Epub 2018 Jan 11.

Department of Integrative Pediatric and Adolescent Medicine, Gemeinschaftskrankenhaus Herdecke, Germany; Witten/Herdecke University, Faculty of Health, Professorship for Integrative Pediatrics, Centre for Integrative Medicine, Germany.

Background: Long-term survival of children with cancer has reached rates of up to 80%. Nevertheless, continued research devoted to further improvement of survival rates especially for patients with high-risk illnesses is necessary. Recent studies have shown direct positive effects on tumor reduction through Viscum album (mistletoe) extracts in adults, mainly as a result of higher dosage treatment and intravenous or intratumoral application. Read More

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http://dx.doi.org/10.1016/j.ctim.2018.01.002DOI Listing
October 2018
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Rhabdoid type intramedullary meningioma. A case report and review of the literature.

Neurocirugia (Astur) 2018 Sep 12. Epub 2018 Sep 12.

Servicio Anatomía Patológica, Hospital Universitario Puerta del Mar, Cádiz, España.

Meningiomas are the most frequent tumors located at the spinal level together with neurinomas and metastases. These tumors tend to be intradural and extramedullar. There are few cases described in the literature with a purely intramedullary location (less than 10 cases) and they are frequently observed in the union craneal-cervical. Read More

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http://dx.doi.org/10.1016/j.neucir.2018.08.001DOI Listing
September 2018

A Pediatric Intra-Axial Malignant SMARCB1-Deficient Desmoplastic Tumor Arising in Meningioangiomatosis.

J Neuropathol Exp Neurol 2018 Oct;77(10):883-889

Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota.

SMARCB1 inactivation is a well-established trigger event in atypical teratoid/rhabdoid tumor. Recently, a role for SMARCB1 inactivation has emerged as a mechanism of clonal evolution in other tumor types, including rare brain tumors. We describe an unusual malignant intra-axial SMARCB1-deficient spindle cell desmoplastic neoplasm, occurring in a 6-year-old child with meningioangiomatosis and a long history of seizures. Read More

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http://dx.doi.org/10.1093/jnen/nly075DOI Listing
October 2018
6 Reads

Specific computed tomography imaging characteristics of congenital mesoblastic nephroma and correlation with ultrasound and pathology.

J Pediatr Urol 2018 Dec 1;14(6):571.e1-571.e6. Epub 2018 Aug 1.

Department of Radiology, The First Affiliated Hospital, Sun Yat-sen University, 58th, The Second Zhongshan Road, Guanzhou, China. Electronic address:

Introduction: Congenital mesoblastic nephroma (CMN) is a common solid renal tumor in the neonate. Congenital mesoblastic nephroma can be divided into classic, cellular, and mixed types. The prognosis of CMN is very optimistic. Read More

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http://dx.doi.org/10.1016/j.jpurol.2018.07.020DOI Listing
December 2018
1 Read

[Implantation of VPS in the early postoperative period following removal of posterior cranial fossa tumors in children].

Zh Vopr Neirokhir Im N N Burdenko 2018;82(4):81-86

Burdenko Neurosurgical Institute, Moscow, Russia.

One of the main manifestations of posterior cranial fossa (PCF) tumors is the development of hydrocephalus (HC) symptoms that in most cases are the reason for examination and diagnosis [1, 2]. According to the literature, the rate of shunt surgery after removal of PCF tumors in the children's population is 18-40% [3-9]. Hydrocephalus remaining after removal of PCF tumor and requiring further treatment is called persistent hydrocephalus. Read More

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http://dx.doi.org/10.17116/neiro201882481DOI Listing
January 2018
2 Reads

In Vivo Expansion of Cancer Stemness Affords Novel Cancer Stem Cell Targets: Malignant Rhabdoid Tumor as an Example.

Stem Cell Reports 2018 Sep 16;11(3):795-810. Epub 2018 Aug 16.

Pediatric Stem Cell Research Institute, Edmond and Lily Sara Children's Hospital, Sheba Medical Center, Tel Hashomer, Ramat-Gan 52621, Israel; Sheba Centers for Regenerative Medicine and Cancer Research, Sheba Medical Center, Ramat-Gan 52621, Israel; Division of Pediatric Nephrology, Safra Children's Hospital, Sheba Medical Center, Ramat-Gan 52621, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv 6997801, Israel. Electronic address:

Cancer stem cell (CSC) identification relies on transplantation assays of cell subpopulations sorted from fresh tumor samples. Here, we attempt to bypass limitations of abundant tumor source and predetermined immune selection by in vivo propagating patient-derived xenografts (PDX) from human malignant rhabdoid tumor (MRT), a rare and lethal pediatric neoplasm, to an advanced state in which most cells behave as CSCs. Stemness is then probed by comparative transcriptomics of serial PDXs generating a gene signature of epithelial to mesenchymal transition, invasion/motility, metastasis, and self-renewal, pinpointing putative MRT CSC markers. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22136711183031
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http://dx.doi.org/10.1016/j.stemcr.2018.07.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6135722PMC
September 2018
5 Reads

Factors Influencing Overall Survival of Children, Adolescents, and Young Adults With High-risk Renal Tumors.

Urology 2018 Oct 1;120:222-230. Epub 2018 Aug 1.

University of Colorado, Department of Surgery, Division of Urology; Children's Hospital Colorado, Aurora, CO. Electronic address:

Objective: To identify factors impacting overall survival (OS) in children, adolescents, and young adults with high-risk renal tumors (HRRTs).

Methods: The National Cancer Database was queried for patients ≤30 years old diagnosed with anaplastic Wilms tumors (AWT), clear cell sarcoma of the kidney (CCSK), or rhabdoid tumor of the kidney (RTK) between 2004 and 2013. Demographic, clinical, and OS data were abstracted. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00904295183079
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http://dx.doi.org/10.1016/j.urology.2018.07.032DOI Listing
October 2018
5 Reads

Pediatric hepatic rhabdoid tumor: A rare cause of abdominal mass in children.

Radiol Case Rep 2018 Jun 10;13(3):724-727. Epub 2018 May 10.

Division of Pediatric Radiology, Department of Radiology, University of Virginia Health System, University Hospital, First Floor,1215 Lee St., Charlottesville, VA, 22908.

Pediatric hepatic rhabdoid tumors are rare tumors of the liver, with few cases reported in the literature. These aggressive tumors can be difficult to differentiate from hepatoblastomas on imaging alone, and surgical biopsy combined with special immunohistochemical stains can assist in differentiating these 2 tumor types. We present a case of hepatic rhabdoid tumor in a 7-month-old female infant, which was originally thought to be a hepatoblastoma; however, using BAF47 staining for INI-1 we were able to diagnose a rhabdoid tumor and affect the patient's medical oncologic therapy. Read More

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http://dx.doi.org/10.1016/j.radcr.2018.03.017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6066605PMC
June 2018
6 Reads