CNS and SCT Tumors in Families

[Jody]

Hey all,

I know we've covered this before, but I've started doing some research on family history of sct and cns tumors. I found this article www.medscape.com/viewarticle/522326 which deals mainly with intracranial tumors, and want to know if anyone knows of any research about cns tumors in families.

The reason I ask is because I'm an sct survivor, my dad was just diagnosed with an sct, and my uncle has a brain stem/sct. That's too many people, too closely related for my taste, and there's got to be something out there that might shed some light on this.

Thanks!

Jody

[bettygillian]

Here's an NIH project researching very, very rare familial chordoma.

www.cancer.gov/clinicaltrials/ft-NCI-78-C-0039

[Chordoma is a rare cns tumor, about one per million people.]

[stargirl65]

Most of these are spontaneous but there is some suggestions that some may have a genetic component. This was taken from and eMedicine article on astrocytoma.

"Evidence exists for genetic susceptibility to glioma development. For example, familial clustering of astrocytomas is well described in inherited neoplastic syndromes, such as Turcot syndrome, neurofibromatosis type 1 (NF1) syndrome, and p53 germ line mutations (eg, Li-Fraumeni syndrome).
Biological investigation has implicated that mutations in specific molecular pathways, such as the p53-MDM2-p21 and p16-p15-CDK4-CDK6-RB pathways, are associated with astrocytoma development and progression. In addition, inherited elements of the immune response known as human leukocyte antigens (HLA) have been both positively and negatively associated with an increased risk for the development of glioblastoma multiforme.
Recently, attempts have been made to determine prognosis and response to various treatment modalities based on the individual pattern of genetic changes in a particular patient. For example, patients with oligodendrogliomas that exhibit chromosomal changes at band 1p19q are known to have improved responses to the procarbazine, CCNU, vincristine (PCV) regimen of chemotherapy. Efforts are underway to identify similar unique susceptibilities associated with other commonly altered genes and proteins in astrocytomas. Other groups are working on developing models that will allow for a more accurate assessment of prognosis based on a combination of molecular profiling of the tumors and clinical characteristics of the patient."