| Title |
Medulloblastoma, WNT-activated/SHH-activated: clinical impact of molecular analysis and histogenetic evaluation
|
|---|---|
| Published in |
Child's Nervous System, March 2018
|
| DOI | 10.1007/s00381-018-3765-2 |
| Pubmed ID | |
| Authors |
Eduardo Cambruzzi |
| Abstract |
Medulloblastoma (MDB) is a small cell poorly differentiated embryonal tumor of the cerebellum, which more frequently compromises children. Overall prognosis is favorable, but dependent of stage, histopathological pattern and molecular group. Approximately 30% of the affected patients will die from the disease. WHO 2016 Classification of Tumors of the Central Nervous System (CNS) has been classified MDB into four principal groups: WNT-activated MDB, SHH-activated MDB, group 3 MDB, and group 4 MDB. WNT-activated MDB is associated to monosomy 6, CTNNB1, DDX3X and TP53 mutations, beta-catenin nuclear immunoexpression, and a better prognosis than SHH-activated MDB. WNT-activated tumors account approximately for 10% of cases of MDBs, and are thought to arise from cells in the dorsal brain stem/lower rhombic lip progenitor cells. SHH-activated MDB more frequently arises in the lateral hemispheres of the cerebellum, and clinical outcome in this group is variable. TP53-mutant SHHactivated MDB usually shows the large cell/anaplastic pattern, and can be related to MYCN amplification, GLI2 amplification and 17p loss. TP53-wildtype SHH-activated MDB is more commonly of desmoplastic/nodular morphology, and can be related to PTCH1 deletion and 10q loss. Gene expression and methylation profiling is the gold standard for defining molecular groups of MDB. In immunohistochemistry assays, anti-GAB1 antibody expression is positive in tumors showing SHH pathway activation or PTCH mutation, while positive immunoexpression for YAP1 antibody can be only found in WNT-activated and SHH-activated MDB. |
X Demographics
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Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United States | 1 | 25% |
| Portugal | 1 | 25% |
| Unknown | 2 | 50% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 3 | 75% |
| Science communicators (journalists, bloggers, editors) | 1 | 25% |
Mendeley readers
The data shown below were compiled from readership statistics for 43 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 43 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Bachelor | 7 | 16% |
| Researcher | 5 | 12% |
| Other | 4 | 9% |
| Student > Postgraduate | 4 | 9% |
| Student > Master | 3 | 7% |
| Other | 6 | 14% |
| Unknown | 14 | 33% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 10 | 23% |
| Biochemistry, Genetics and Molecular Biology | 8 | 19% |
| Agricultural and Biological Sciences | 3 | 7% |
| Nursing and Health Professions | 2 | 5% |
| Neuroscience | 2 | 5% |
| Other | 2 | 5% |
| Unknown | 16 | 37% |
Attention Score in Context
This research output has an Altmetric Attention Score of 2. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 13 April 2018.
All research outputs
#14,096,200
of 23,031,582 outputs
Outputs from Child's Nervous System
#742
of 2,802 outputs
Outputs of similar age
#180,812
of 330,380 outputs
Outputs of similar age from Child's Nervous System
#12
of 75 outputs
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So far Altmetric has tracked 2,802 research outputs from this source. They receive a mean Attention Score of 1.9. This one has gotten more attention than average, scoring higher than 72% of its peers.
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We're also able to compare this research output to 75 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 82% of its contemporaries.