Intracranial tumours

2010 ◽  
pp. 4967-4972
Author(s):  
Jeremy Rees
Keyword(s):  
Risk Factors ◽  
Intracranial Tumours ◽  
Li Fraumeni Syndrome ◽  
Von Hippel Lindau ◽  
Li Fraumeni ◽  
Von Hippel Lindau Syndrome ◽  
Neuroepithelial Tumours

Intracranial tumours represent about 2% of all cancers. There are no known risk factors apart from prior irradiation to the skull and brain and a few rare neurogenetic syndromes, e.g. neurofibromatosis, von Hippel–Lindau syndrome, Li–Fraumeni syndrome. They may be intrinsic or extrinsic, which determines potential resectability. Neuroepithelial tumours (predominantly gliomas) account for 50 to 60% of all primary tumours. Recent discovery of ...

scholarly journals Li-Fraumeni Syndrome and p53 in 2015: Celebrating their Silver Anniversary

2016 ◽  
Vol 39 (1) ◽  
pp. 37 ◽  
Author(s):  
David Malkin
Keyword(s):  
Wilms Tumor ◽  
Cancer Genetics ◽  
Tp53 Gene ◽  
Pleuropulmonary Blastoma ◽  
Li Fraumeni Syndrome ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Li Fraumeni ◽  
Sick Children ◽  
Risk Of Cancer

In a typical morning in the Cancer Genetics Clinic at The Hospital for Sick Children in Toronto, the following array of patients and families might be seen: a family of three children, all harbouring a mutation of the succinyl dehydrogenase C gene inherited from their father who had had extensive surgery several years ago for a secreting paraganglioma; three families with Li-Fraumeni syndrome, each with at least one child harbouring a TP53 gene mutation conferring a lifetime risk of cancer approaching 100% and currently undergoing surveillance for early tumour detection; two children with Li-Fraumeni syndrome undergoing treatment for cancer – one having had three cancer diagnoses before 19 months of age and the other just completing therapy for metastatic adrenocortical carcinoma at age 3; two children with von Hippel-Lindau disease being monitored for persistent pancreatic neuroendocrine tumors and cerebellar hemangioblastomas, respectively; and one child with Beckwith-Wiedeman syndrome and Wilms tumor and another child completing therapy for a pleuropulmonary blastoma (PPB).

Intracranial tumours

2020 ◽  
pp. 6048-6054
Author(s):  
Jeremy Rees
Keyword(s):  
Histological Grade ◽  
Whole Brain Radiotherapy ◽  
Accurate Information ◽  
Cancer Treatments ◽  
Intracranial Tumours ◽  
Li Fraumeni Syndrome ◽  
Von Hippel Lindau ◽  
Brain Radiotherapy ◽  
Integrated Diagnosis ◽  
Anti Cancer

Intracranial tumours represent about 2% of all cancers. There are no known risk factors apart from prior irradiation to the skull and brain and a few rare neurogenetic syndromes (e.g. neurofibromatosis, von Hippel–Lindau syndrome, Li–Fraumeni syndrome). They may be intrinsic or extrinsic, which determines potential resectability. Neuroepithelial tumours (predominantly gliomas) account for 50–60% of all primary tumours. Molecular analysis has now been added to the histological grade and subtype to provide an integrated diagnosis which provides more accurate information for treatment and prognosis. As systemic anti-cancer treatments evolve and produce long-term control of visceral and bone disease, brain metastases are becoming increasingly common and are best treated with feither stereotactic radiosurgery or neurosurgery to avoid the cognitive decline associated with whole brain radiotherapy.

Von Hippel-Lindau syndrome: in vivo portrait with 68Ga-DOTANOC PET-CT

2017 ◽  
Author(s):  
Moreira Ana Paula ◽  
Gracinda Costa ◽  
de Lima Joao Pedroso
Keyword(s):  
Von Hippel Lindau ◽  
Pet Ct ◽  
Von Hippel Lindau Syndrome

Li-Fraumeni syndrome and adrenal tumours: case report

2018 ◽  
Author(s):  
Mariana Tome ◽  
Jessica Guarino ◽  
Marta Iturregui
Keyword(s):  
Case Report ◽  
Li Fraumeni Syndrome ◽  
Adrenal Tumours ◽  
Li Fraumeni

Neuroradiology Manifestations of Li-Fraumeni Syndrome: Epidemiology, Genetics, Imaging Findings, and Management

Neurographics ◽  
2020 ◽  
Vol 10 (4) ◽  
pp. 228-235
Author(s):  
S. Naganawa ◽  
T. Donohue ◽  
A. Capizzano ◽  
Y. Ota ◽  
J. Kim ◽  
...  
Keyword(s):  
Cell Cycle ◽  
Tumor Suppressor ◽  
Tumor Suppressor Gene ◽  
Soft Tissue Sarcomas ◽  
Suppressor Gene ◽  
Imaging Findings ◽  
Li Fraumeni Syndrome ◽  
Increased Risk ◽  
Li Fraumeni ◽  
Risk Of Cancer

Li-Fraumeni syndrome is a familial cancer predisposition syndrome associated with germline mutation of the tumor suppressor gene 53, which encodes the tumor suppressor p53 protein. Affected patients are predisposed to an increased risk of cancer development, including soft-tissue sarcomas, breast cancer, brain tumors, and adrenocortical carcinoma, among other malignancies. The tumor suppressor gene TP53 plays an important, complex role in regulating the cell cycle, collaborating with transcription factors and other proteins. The disruption of appropriate cell cycle regulation by mutated TP53 is considered to be the cause of tumorigenesis in Li-Fraumeni syndrome. Appropriate surveillance, predominantly by using MR imaging, is used for early malignancy screening in an effort to improve the survival rate among individuals who are affected. Patients with Li-Fraumeni syndrome are also at increased risk for neoplasm development after radiation exposure, and, therefore, avoiding unnecessary radiation in both the diagnostic and therapeutic settings is paramount. Here, we review the epidemiology, genetics, imaging findings, and the current standard surveillance protocol for Li-Fraumeni syndrome from the National Comprehensive Cancer Network as well as potential treatment options.Learning Objective: Describe the cause of second primary malignancy among patients with Li-Fraumeni syndrome.

Spitzoid Melanoma in a Child with Li-Fraumeni Syndrome

2014 ◽  
Vol 17 (1) ◽  
pp. 64-69 ◽  
Author(s):  
Ramya Kollipara ◽  
Linda D. Cooley ◽  
Kimberly A. Horii ◽  
Maxine L. Hetherington ◽  
Philip E. LeBoit ◽  
...  
Keyword(s):  
Li Fraumeni Syndrome ◽  
Li Fraumeni

Utility of interim blood tests for cancer screening in Li-Fraumeni syndrome

2021 ◽  
Author(s):  
Leatrisse Oba ◽  
Ana F. Best ◽  
Phuong L. Mai ◽  
Maria Isabel Achatz ◽  
Paul S. Albert ◽  
...  
Keyword(s):  
Cancer Screening ◽  
Li Fraumeni Syndrome ◽  
Blood Tests ◽  
Li Fraumeni

VON HIPPEL–LINDAU SYNDROME

2020 ◽  
pp. 1005-1020
Author(s):  
Samantha E. Greenberg ◽  
Luke D. Maese ◽  
Benjamin L. Maughan
Keyword(s):  
Von Hippel Lindau ◽  
Von Hippel Lindau Syndrome
Sign in / Sign up

Export Citation Format

Share Document