Rhabdoid Tumor Information

Websites 

READ ME FIRST: Please take care when accessing these sites. Most do contain survival rates. I caution parents of children diagnosed with MRT to remember that statistics are not truths. No one can tell you how your child will respond to treatment. 

Information: Children's Hospital of Philidelphia: Rhabdoid Predisposition Syndrome Dana-Farber Cancer Institute: Childhhood Rhabdoid Tumors Dana-Farber Cancer Instutute: Malignant Rhabdoid Tumor Cancer.gov: Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment (PDQ®) Patient Version The Hospital for Sick Children: Rhabdoid Tumors St. Baldrick's Foundation: Types of Cancer: Atypical Teratoid Rhabdoid Tumor St Jude's Children's Research Hospital: Rhabdoid Predisposition Syndrome St Jude's Children's Research Hospital:Atypical Teratoid Rhabdoid Tumor Wikipedia: Malignant Rhabdoid Tumor Wikipedia: Atypical Teratoid Rhabdoid Tumor Children's Cancer Web: Malignant Rhabdoid Tumor Orphanet: Rhabdoid Tumor

Organizations: Grace Kelly Ladybird Trust (UK) The Buttercup Foundation in Memory of Carter Mandley The Zoë Faye Foundation (Boston, MA, USA) Sean Duggan Foundation (Chicago, IL, USA) Amazing Gracie's Gift (New York, USA) Children's Brain Tumor Foundation (USA) Hawaii Children's Cancer Foundation (Hawaii, USA)

News Articles 


P53 in Relation to SMARB1- Tumors

New Therapeutic Target Found for Aggressive Pediatric Cancers with Few Treatment Options

Researchers at The University of Texas MD Anderson Cancer Center have discovered that malignant rhabdoid tumors (MRT), a rare pediatric cancer without effective treatments, may be sensitive to drugs that block the cancer cell's ability to dispose of misfolded proteins. The findings provide a much-needed therapeutic target for these and other cancers caused by mutations in the SMARCB1 gene.

Study provides insight into link between two rare tumor syndromes


PGBD5

Memorial Sloan Kettering Researchers Suggest a Mechanism for the Triggering of Solid Tumors in Children and Young Adults

A new discovery published in the journal Nature Genetics identifies a mechanism for the triggering of solid tumors — including most types of cancers that affect children and young adults. The enzyme made by the gene PGBD5 cuts DNA segments and then rearranges them. This DNA remodeling can drastically alter normal gene function and cause cancer. Rather than normal cells turning cancerous as a result of random errors or mutations, the molecule encoded by the PGBD5 gene itself can induce specific mutations and turn cells malignant. Jumping Genes and the Dark Genome: MSK Researchers Gain New Insight into Childhood Cancers

Medical Papers

Warning: These academic papers often detail poor outcomes. Do not lose hope!! Statistics cannot tell you what your child's outcome will be.  Hopefully this research will lead to treatments that can make this disease manageable, if not provide a cure. This is by no means a comprehensive list of papers, simply what I have come by in my own research of my son's MRT.

Liver, AT/RT and Extrarenal Tumors

SMARCB1-deficient Tumors of Childhood: A Practical Guide

Epidemiology of Rhabdoid Tumors in Early Childhood Malignant rhabdoid tumor of the liver: a case report and literature review * Malignant Rhabdoid Tumour of the Liver in the Young Adult: Report of First Two Cases Malignant rhabdoid tumour of the liver in a seven-month-old female infant: A case report and literature review Surgery and Actinomycin Improve Survival in Malignant Rhabdoid Tumor * European Rhabdoid Registry V4 2015* * gives some treatment advice

Genetics of Rhabdoid Tumors

SMARCB1/INI1 Alterations and Hepatoblastoma: Another Extrarenal Rhabdoid Tumor Revealed? Spectrum of SMARCB1/INI1 Mutations in Familial and Sporadic Rhabdoid Tumors Germ-Line and Acquired Mutations of INI1 in Atypical Teratoid and Rhabdoid Tumors 1 RHABDOID TUMOR PREDISPOSITION SYNDROME 1; RTPS1 A remarkably simple genome underlies highly malignant pediatric rhabdoid cancers Rhabdoid Tumor Predisposition Syndrome The role of SMARCB1/INI1 in development of rhabdoid tumor Treatment Research: Rhabdoid Specific Papers:

p53 Is a Master Regulator of Proteostasis in SMARCB1-Deficient Malignant Rhabdoid Tumors

--Timing of Smarcb1 and Nf2 inactivation determines schwannoma versus rhabdoid tumor development (Predicessor to p53 Is a Master...)

Rhabdoid Tumor Growth is Inhibited by Flavopiridol Malignant Rhabdoid Tumor A Study with Two Established Cell Lines  Durable tumor regression in genetically altered malignant rhabdoid tumors by inhibition of methyltransferase EZH2  General: Development of EZH2 Inhibitors for the Treatment of Genetically Defined Cancers Cancer epigenetics drug discovery and development: the challenge of hitting the mark The emerging science of epigenomics Immunotherapy of Childhood Cancer: From Biologic Understanding to Clinical Application Monoclonal antibody therapy of cancer The safety and side effects of monoclonal antibodies

Liver Tumors: 

Towards an international pediatric liver tumor consensus classification: proceedings of the Los Angeles COG liver tumors symposium


General Tumor Information:

Mechanisms and Functions of ATP-Dependent Chromatin-Remodeling Enzymes

Abstracts:

p53 Is a Master Regulator of Proteostasis in SMARCB1-Deficient Malignant Rhabdoid Tumors

Renal and extrarenal rhabdoid tumors in children: a clinicopathologic study of 14 patient Long-term survival after spontaneous rupture of a malignant rhabdoid tumor of the liver Malignant rhabdoid tumor of the liver. Evidence for epithelial differentiation. Malignant rhabdoid tumor of the liver. A distinct clinicopathologic entity. The clinicopathologic spectrum of putative extrarenal rhabdoid tumors. An analysis of 42 cases studied with immunohistochemistry or electron microscopy. Malignant rhabdoid tumor mimicking hepatoblastoma: a case report and literature review.Extrarenal rhabdoid tumors of soft tissue: A clinicopathologic and immunohistochemical study of 18 cases New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs

Outcome of extracranial malignant rhabdoid tumours in children registered in the European Paediatric Soft Tissue Sarcoma Study Group Non-Rhabdomyosarcoma Soft Tissue Sarcoma 2005 Study—EpSSG NRSTS 2005

Treatment of Central Nervous System Malignant Rhabdhoid Tumors

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