However, some reports exist of AT/RTs presenting in two members of the same family, or one family member with an AT/RT and another with a renal rhabdoid tumor or other CNS tumor. (wikipedia.org)
PURPOSE: Report relevance of molecular groups to clinicopathologic features, germline SMARCB1/SMARCA4 alterations (GLA), and survival of children with atypical teratoid rhabdoid tumor (ATRT) treated in two multi-institutional clinical trials. (bvsalud.org)
BACKGROUND: Malignant rhabdoid tumor of the kidney (RTK) is a rare and highly aggressive pediatric malignancy. (bvsalud.org)
Pediatric2
Around 17% of all pediatric cancers involve the CNS, making these cancers the most common childhood solid tumor. (wikipedia.org)
AT/RT is the first pediatric brain tumor for which a candidate tumor suppressor gene has been identified. (wikipedia.org)
Rare1
Atypical teratoid/rhabdoid tumors are very rare, and absolute risk to siblings is not reported in the literature. (wikipedia.org)
Cell2
There are also some emerging mouse models of the AT/RT cancer as well as experimental cell lines derived from tumors. (wikipedia.org)
Correlation analysis between the risk score of our model and tumor-infiltrating cell were also investigated. (bvsalud.org)
Rhabdoid tumors10
Genetic similarities have been found within rhabdoid tumors. (wikipedia.org)
Most rhabdoid tumors have INI1 deletions whether they occur in the CNS, kidney, or elsewhere. (wikipedia.org)
INI1/hSNF5, a component of the chromatin remodeling SWI/SNF complex, is a critical tumor suppressor biallelically inactivated in rhabdoid tumors. (wikipedia.org)
Identification of INI1 as a tumor suppressor has facilitated accurate diagnosis of rhabdoid tumors. (wikipedia.org)
Atypical teratoid/rhabdoid tumors are very rare, and absolute risk to siblings is not reported in the literature. (wikipedia.org)
Rorke LB, Packer R, Biegel J: Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. (karger.com)
1. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. (nih.gov)
3. [Atypical teratoid/rhabdoid tumors of central nervous system in childhood: a clinical and histopathologic study of 6 cases]. (nih.gov)
14. Immunohistochemical analysis of INI1 protein in malignant pediatric CNS tumors: Lack of INI1 in atypical teratoid/rhabdoid tumors and in a fraction of primitive neuroectodermal tumors without rhabdoid phenotype. (nih.gov)
18. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. (nih.gov)
INI12
The chromosome 22 area contains the hSNF5/INI1 gene that appears to function as a classic tumor suppressor gene. (wikipedia.org)
A mutation or deletion in the INI1/hSNF5 gene occurs in the majority of AT/RT tumors. (wikipedia.org)
Highly malignant2
Atypical teratoid/rhabdoid tumor of the central nervous system is a highly malignant neoplasm and that usually arises in the posterior fossa, survival from this is frequently poor. (karger.com)
7. Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. (nih.gov)
19. Atypical teratoid rhabdoid tumor in childhood, 15 cases of a single institute experience. (nih.gov)
19961
AT/RT was only recognized as an entity in 1996 and added to the World Health Organization Brain Tumor Classification in 2000 (Grade IV). (wikipedia.org)
Central nervou10
Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS), including the spinal cord. (wikipedia.org)
Caldemeyer KS, Smith RR, Azzarelli B, Boaz JC: Primary central nervous system malignant rhabdoid tumor: CT and MR appearance simulates a primitive neuroectodermal tumor. (karger.com)
Agranovich AL, Ang LC, Griebel RW, Kobrisky NL, Lowry N, Tchang SP: Malignant rhabdoid tumor of the central nervous system with subarachnoid dissemination. (karger.com)
Hilden JM, Meerbaum S, Burger P, Finlay J, Janss A, Scheithauer BW, Walter AW, Rorke LB, Biegel JA: Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. (karger.com)
6. Atypical teratoid/rhabdoid tumor of the central nervous system: a comparative study with primitive neuroectodermal tumor/medulloblastoma. (nih.gov)
8. [Clinicopathologic and immunohistochemical study of atypical teratoid/rhabdoid tumor of central nervous system]. (nih.gov)
11. Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases. (nih.gov)
16. Atypical teratoid/rhabdoid tumor of the central nervous system: clinico-pathological study. (nih.gov)
17. Atypical teratoid/rhabdoid tumor of the central nervous system in children: an atypical series and review. (nih.gov)
Cancer1
There are also some emerging mouse models of the AT/RT cancer as well as experimental cell lines derived from tumors. (wikipedia.org)
Study2
Beckwith JB, Palmer NF: Histopathology and prognosis of Wilm's tumor: results from the first National Wilm's tumor study. (karger.com)
Horizontal tumor extent (HZTE) has limited prognostic significance in 2018 FIGO stage I endocervical adenocarcinoma (ECA): a retrospective study of 416 cases. (patologia.ro)
Child2
A case of a seven-month-old child with a primarily spinal tumor that presented with progressive paraplegia and abnormal feeling in the legs was reported. (wikipedia.org)
Chung YN, Wang KC, Shin SH, Kim N, Chi JG, Min KS, Cho BK: Primary intracranial atypical teratoid/rhabdoid tumor in a child: A case report. (karger.com)
Case2
We present a unique case in a 21-month-old girl who had an atypical teratoid/rhabdoid tumor with cystic components located in the right fronto-parietal lobe. (karger.com)
Lu L, Wilkinson EJ, Yachnis AT: CSF Cytology of atypical teratoid/rhabdoid tumor of the brain in a two-year-old girl: a case report. (karger.com)
Years2
Two years later at the last follow-up visit, there was no evidence of a tumor relapse on MRI, and the examination was symptom free. (karger.com)
10. Cytomorphological features of atypical teratoid/rhabdoid tumor: an account of 12 years' experience. (nih.gov)
Expression2
In addition, the OPN gene has a higher expression in AT/RT tumors. (wikipedia.org)
The cytokeratin 17 expression in primary ovarian tumors has diagnostic but not prognostic significance. (patologia.ro)
Analysis1
Clear Cell Carcinoma (CCC) of the Cervix Is a Human Papillomavirus (HPV)-independent Tumor Associated With Poor Outcome: A Comprehensive Analysis of 58 Cases. (patologia.ro)