Ignite Creation Date:
2024-05-06 @ 9:55 AM
Last Modification Date:
2024-10-26 @ 12:22 PM
Study NCT ID:
NCT03115164
Status:
UNKNOWN
Last Update Posted:
2017-04-14
First Post:
2017-04-04
Brief Title:
Astrocytoma Desmoplastic Gamliogliomes DIA DIG - Study of the French Cohort of the Last 20 Years Clinical Anatomopathological Molecular and Radiological Charactersics
Sponsor:
Centre Hospitalier Universitaire Amiens
Organization:
Centre Hospitalier Universitaire Amiens
Study Overview
Official Title:
Astrocytoma Desmoplastic Gamliogliomes DIA DIG - Study of the French Cohort of the Last 20 Years Clinical Anatomopathological Molecular and Radiological Charactersics
Status:
UNKNOWN
Status Verified Date:
2017-04
Last Known Status:
RECRUITING
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
DIADIG
Brief Summary:
Astrocytomas infantile desmoplastic gangliogliomas DIA DIG are rare brain tumors usually affecting infants They represent about 05 of all pediatric brain tumors DIA DIG occurs mainly in the first 2 years of life with a sex ratio M F of 17 to 1 From a histological point of view DIA DIG are neuroepithelial tumors These tumors may have a purely astrocytic differentiation DIA or be composed of tumor cells with astrocytic and neuronal differentiation DIG The desmoplastic component is usually adjacent to the meninges and is defined by the increase or modification of connective tissues related to the presence of neoplastic cells with the formation of a collagen-rich extracellular matrix
Due to their benign biological behavior and favorable clinical course they are classified in benign tumors ie grade I according to the WHO classification However all tumors called DIA DIG do not behave in a benign manner Cases of metastatic cerebrospinal and malignant disorders have been described It appears that about 40 of DIG cases require additional medical treatment such as chemotherapy radiotherapy and or new surgery and 15 of infants and children with GIDD die from the disease It is possible that what is grouped within the DIA DIG is a heterogeneous group of tumors evolution and prognosis very variable
The cytogenetic knowledge of DIA DIG is very limited and is only available on small numbers of cases Cytogenetic analyzes of several cases of DIG showed normal karyotypes More recently a CGH-Array study of 3 cases of DIA DIG did not find any significant chromosomal gains or losses
It has been shown however that a mutation involving BRAF BRAF rearrangement or BRAF V600E mutations was a recurrent element in low grade gliomas particularly in pediatric patients
It is also suggested that deregulation of BRAF activity in some DIA DIG may indicate the importance of the MAPK mitogen-activated protein kinase pathway in signaling pathways for DIA DIG development However data on the link between the BRAF gene and DIA DIG remains very limited Thus further studies are needed to study the other members of the MAPK pathway in DIA DIG eg PI3K AKT mTOR This could provide new therapeutic possibilities involving targeted therapies specific to the MAPK signaling pathway
It appears that DIA DIG does not all behave in a benign manner and some would undergo a malignant transformation that could be due to chromosomal alterations such as for example TP53 PI3K In addition because of the limited number of cases it would be interesting to study the characteristics of patients with DIA DIG in order to study their characteristics and whether there are clinical pathological cytogenetic and Molecular forms between benign and malignant forms
Due to their benign biological behavior and favorable clinical course they are classified in benign tumors ie grade I according to the WHO classification However all tumors called DIA DIG do not behave in a benign manner Cases of metastatic cerebrospinal and malignant disorders have been described It appears that about 40 of DIG cases require additional medical treatment such as chemotherapy radiotherapy and or new surgery and 15 of infants and children with GIDD die from the disease It is possible that what is grouped within the DIA DIG is a heterogeneous group of tumors evolution and prognosis very variable
The cytogenetic knowledge of DIA DIG is very limited and is only available on small numbers of cases Cytogenetic analyzes of several cases of DIG showed normal karyotypes More recently a CGH-Array study of 3 cases of DIA DIG did not find any significant chromosomal gains or losses
It has been shown however that a mutation involving BRAF BRAF rearrangement or BRAF V600E mutations was a recurrent element in low grade gliomas particularly in pediatric patients
It is also suggested that deregulation of BRAF activity in some DIA DIG may indicate the importance of the MAPK mitogen-activated protein kinase pathway in signaling pathways for DIA DIG development However data on the link between the BRAF gene and DIA DIG remains very limited Thus further studies are needed to study the other members of the MAPK pathway in DIA DIG eg PI3K AKT mTOR This could provide new therapeutic possibilities involving targeted therapies specific to the MAPK signaling pathway
It appears that DIA DIG does not all behave in a benign manner and some would undergo a malignant transformation that could be due to chromosomal alterations such as for example TP53 PI3K In addition because of the limited number of cases it would be interesting to study the characteristics of patients with DIA DIG in order to study their characteristics and whether there are clinical pathological cytogenetic and Molecular forms between benign and malignant forms
Detailed Description:
None
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None