PMID- 25085603
OWN - NLM
STAT- MEDLINE
DCOM- 20150401
LR  - 20161125
IS  - 2210-7762 (Print)
VI  - 207
IP  - 9
DP  - 2014 Sep
TI  - Rhabdoid tumor: the Irish experience 1986-2013.
PG  - 398-402
LID - S2210-7762(14)00114-8 [pii]
LID - 10.1016/j.cancergen.2014.05.015 [doi]
AB  - Nomenclature for the three recognized forms of rhabdoid tumor reflect their 
      anatomic localization and include malignant rhabdoid tumor of the kidney (MRTK), 
      extrarenal extracranial rhabdoid tumor (EERT), and atypical teratoid rhabdoid 
      tumor (ATRT) involving the central nervous system. A strikingly simple karyotype 
      belies the fact that rhabdoid tumors are among the most lethal human cancers, and 
      now early strides are beginning to elucidate their molecular pathogenesis. 
      Rhabdoid tumors are largely confined to the pediatric population, where they 
      occur preferentially during infancy. Given the rarity of this tumor, 
      international consensus on best treatment has only recently been achieved in 
      conjunction with the establishment of the European Rhabdoid Tumor Registry. 
      Between 1986 and 2013, 25 pediatric patients were diagnosed with rhabdoid tumor 
      in the Republic of Ireland. Of these patients, 13 presented with ATRT, eight had 
      MRTK, and four had EERT. The mean age at diagnosis was 38.8 months, with an equal 
      sex incidence. Because of the lack of a standardized treatment strategy for 
      rhabdoid tumors, these patients have been treated largely according to anatomic 
      site, based on sarcoma, renal, or brain tumor protocols contemporary to their 
      diagnoses. Of the patients, 84% received chemotherapy, 80% underwent surgery, and 
      44% had radiation therapy. The outcome overall was poor, independent of anatomic 
      location. The overall survival rate was 24%, and mean time to death was just 
      under 9 months.
CI  - Copyright (c) 2014 Elsevier Inc. All rights reserved.
FAU - Uwineza, Alice
AU  - Uwineza A
AD  - National Children's Research Center, Our Lady's Children's Hospital, Crumlin, 
      Dublin, Ireland.
FAU - Gill, Harinder
AU  - Gill H
AD  - National Center for Medical Genetics, Our Lady's Children's Hospital, Crumlin, 
      Dublin, Ireland.
FAU - Buckley, Patrick
AU  - Buckley P
AD  - Molecular Pathology Laboratory, Clinical Directorate of Laboratory Medicine, 
      Beaumont Hospital, Dublin, Ireland.
FAU - Owens, Cormac
AU  - Owens C
AD  - National Pediatric Oncology Unit, Our Lady's Children's Hospital, Crumlin, 
      Dublin, Ireland.
FAU - Capra, Michael
AU  - Capra M
AD  - National Pediatric Oncology Unit, Our Lady's Children's Hospital, Crumlin, 
      Dublin, Ireland.
FAU - O'Sullivan, Catriona
AU  - O'Sullivan C
AD  - Radiation Oncology Unit, St. Luke's Hospital, Rathgar, Dublin, Ireland.
FAU - McDermott, Michael
AU  - McDermott M
AD  - Histology Laboratory, Pathology Department, Our Lady's Children's Hospital, 
      Crumlin, Dublin, Ireland.
FAU - Brett, Francesca
AU  - Brett F
AD  - Neuropathology Laboratory, Pathology Department, Beaumont Hospital, Dublin, 
      Ireland.
FAU - Farrell, Michael
AU  - Farrell M
AD  - Neuropathology Laboratory, Pathology Department, Beaumont Hospital, Dublin, 
      Ireland.
FAU - Pears, Jane
AU  - Pears J
AD  - National Pediatric Oncology Unit, Our Lady's Children's Hospital, Crumlin, 
      Dublin, Ireland.
FAU - O'Sullivan, Maureen J
AU  - O'Sullivan MJ
AD  - National Children's Research Center, Our Lady's Children's Hospital, Crumlin, 
      Dublin, Ireland; Histology Laboratory, Pathology Department, Our Lady's 
      Children's Hospital, Crumlin, Dublin, Ireland; Trinity College, Dublin, College 
      Green, Dublin, Ireland. Electronic address: maureen.osullivan@tcd.ie.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20140611
PL  - United States
TA  - Cancer Genet
JT  - Cancer genetics
JID - 101539150
RN  - 0 (Chromosomal Proteins, Non-Histone)
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (SMARCB1 Protein)
RN  - 0 (SMARCB1 protein, human)
RN  - 0 (Transcription Factors)
RN  - Teratoid Tumor, Atypical
SB  - IM
MH  - Adolescent
MH  - Base Sequence
MH  - Central Nervous System Neoplasms/*classification/genetics/pathology
MH  - Child
MH  - Child, Preschool
MH  - Chromosomal Proteins, Non-Histone/genetics
MH  - DNA-Binding Proteins/genetics
MH  - Female
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Ireland
MH  - Karyotype
MH  - Kidney Neoplasms/*classification/genetics/mortality
MH  - Male
MH  - Neoplasm Recurrence, Local/pathology
MH  - Rhabdoid Tumor/*classification/genetics/pathology
MH  - SMARCB1 Protein
MH  - Sequence Deletion
MH  - Survival Rate
MH  - Teratoma/*classification/genetics/pathology
MH  - Transcription Factors/genetics
OTO - NOTNLM
OT  - Irish
OT  - Rhabdoid tumor
OT  - SMARCB1
OT  - pediatric
EDAT- 2014/08/03 06:00
MHDA- 2015/04/02 06:00
CRDT- 2014/08/03 06:00
PHST- 2014/02/28 00:00 [received]
PHST- 2014/05/23 00:00 [revised]
PHST- 2014/05/31 00:00 [accepted]
PHST- 2014/08/03 06:00 [entrez]
PHST- 2014/08/03 06:00 [pubmed]
PHST- 2015/04/02 06:00 [medline]
AID - S2210-7762(14)00114-8 [pii]
AID - 10.1016/j.cancergen.2014.05.015 [doi]
PST - ppublish
SO  - Cancer Genet. 2014 Sep;207(9):398-402. doi: 10.1016/j.cancergen.2014.05.015. Epub 
      2014 Jun 11.