Showing posts with label Genetics and Cancer Risk. Show all posts
Showing posts with label Genetics and Cancer Risk. Show all posts

Friday, August 4, 2017

Von Hippel-Lindau Syndrome

Gene VHL
Kidney cancer
Multiple non-cancerous tumors such as pheochromocytoma

Multiple Endocrine Neoplasia

Type 1 - Wermer Syndrome
Gene MEN1
pancreatic endocrine tumors.
Also (usually) benign parathyroid and pituitary gland tumors

Type 2
Gene RET
Medullary thyroid cancer
Also benign adrenal gland tumor

Retinoblastoma

Gene: RB1

eye cancer, pinealoma (cancer of pineal gland), osteosarcoma, melanoma, soft tissue sarcoma

Familial Adenomatous Polyposis

Gene: APC
increased risk of colorectal cancer as well as non-malignant colon polyps.  Also cancer of small intestine, brain, stomach, bone and skin

Cowden Syndrome

PTEN hamartoma tumor syndrome

Increased risk of breast, thyroid, endometrial

Li-Fraumeni Syndrome

Mutation in TP53 - a gene that normally acts to suppress tumors.
Less common than BRCA, but can cause early onset of cancers such as brain tumors and soft tissue sarcoma.

BRCA

1 in 400 to 800 men and women in the general populations carry BRCA1 or BRCA2 mutations.
Risk is higher in men and women of Ashkenazi Jewish descent (1 in 40 chance)

BRCA1 - breast, ovarian, melanoma, colon

PARP inhibitors - poly ADP ribose polymerace - block an enzyme used by cancer cells to repair damage to their DNA.  Shows promise in patients with inherited BRCA mutations and BRCA abnormalities.

  • Lynparza (olaparib)

Lynch Syndrome

A hereditary disorder caused by a genetic mutation that predisposes a person to several cancers, including colon, stomach, pancreas, kidneys, brain, uterus and skin.

One out of every 279 people has it.
Lynch syndrome accounts for 2-4% of colon cancer cases in US and 2-5% endometrial cancers in women.
If a person tests positive for Lynch syndrome, their children have a 50% chance of inheriting the mutation


  • MLH1
  • MSH2
    • MLH1 and MSH2 face a lifetime risk of developing colon cancer between 52-82% (compared with 4.8 for general population)
  • MSH6 - women have 25-60% lifetime risk of developing endometrial cancer
  • PMS2
  • EOCAM
All genes involved in the process of DNA repair.