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Li-Fraumeni Syndrome Imaging Study

Not Applicable
Conditions
Li-Fraumeni Syndrome
Registration Number
NCT03176836
Lead Sponsor
The Hospital for Sick Children
Brief Summary

Li-Fraumeni Syndrome (LFS) is a cancer predisposition disorder in which most affected individuals develop cancer during their lifetime. The majority of LFS patients carry a mutation in a gene called TP53, whose normal function is to control cell growth and prevent cells with damaged DNA from becoming cancerous. There is currently no way to determine when, where or what type of tumour will develop. This project will use novel techniques utilizing magnetic resonance imaging (MRI) to determine how sensitive they are at detecting very small tumors and how specific they are in terms of distinguishing malignant tumors from benign tumors.

Detailed Description

Not available

Recruitment & Eligibility

Status
ENROLLING_BY_INVITATION
Sex
All
Target Recruitment
30
Inclusion Criteria
  • LFS kindreds who either carry a known TP53 mutation or are obligate mutation carriers
Exclusion Criteria
  • General contraindications for an MRI scan (ferromagnetic prostheses, pacemaker, or other implants incompatible with the magnetic field of the MR scanner), claustrophobia.

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Primary Outcome Measures
NameTimeMethod
Evaluation of imaging traits on suspected tumorsThrough study completion, an average of 2 years

Imaging traits include: signal heterogeneity, mass effect, and neurovascular bundle involvement (recorded on STIR MRI); necrosis, and signal/necrosis ratio (recorded on STIR and DW MRI): FDG metabolic activity and uptake (PET-MRI) or other additional imaging findings. Results will be combined and analyzed for "extraction" of imaging-gene expression phenotypes.

Secondary Outcome Measures
NameTimeMethod

Trial Locations

Locations (1)

The Hospital for Sick Children

🇨🇦

Toronto, Ontario, Canada

The Hospital for Sick Children
🇨🇦Toronto, Ontario, Canada

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