HNPCC and profolactic colectomy

[kristinkatelynn]

My 27 yr old sister was diagnosed with colon cancer in 4/06, and had a right hemi colectomy, 10 lymph nodes removed, and her appendix removed. The Cancer, Thank God, was only stage 2 with no lymph nodes affected, so she did not need chemo or radiation. She was encouraged to undergo genetic testing. Long and short 5 out of 6 of our family members have tested positive for HNPCC MSh2 exon2 deletion. It has been a shocker...The genetic counselors have talked to us about hysterectomys and all of the other prevenative tests that we need to keep up with annually. My sister just had an appointment with an oncologist at a premier cancer center. He asked her if her surgeon knew she had HNPCC when she was operated on. She said no, so then he went on to say that he thought she should have had a total colectomy.... Even though her surgeon said she is cancer free... He is going to a tumor convention next week where he is going to discuss her case further....Has anyonebeen encouraged to have a hystrectomy and/or a total colectomy this young after having cancer and HNPCC?

Thank you

[july181998]

Hi kristinkatelynn,

I'm sorry to hear about your sister. I hope she's doing well now. Although I don't have HNPCC, however; I do have (AFAP) Attenuated Familial Adenomatous Polyposis which is also a inherited gene that I received from my father, who died from colon cancer in his early 40's. In a couple of months, I am having my entire colon removed. I will be meeting with a couple of board certified surgeons that my genetic counselor has referred me to. Did your sister talk with a genetic counselor and was her surgeon board ceritfied? Did she receive second, and maybe third opinions before she had the surgery? Also, with her having HNPCC I'm surprise she didn't have to have chemo, or at least radition. By the way, have you been tested or been screened with a colonoscopy? I've heard depending on the type of inherited gene you have, that a hystrectomy would be necessary. I've already discussed this with my primary doc and genetic counselor, and I don't need to have one. However, I am at high risk for benign tumors of the foot, thyroid, and skin, which is called desmoid firobyosis (I think that's how you spell it). Well the best of luck to you and your sister, and keep the boards posted.

Good Luck,

July

[tpop]

Hi Kristinkatelynn,
Welcome. I have been diagnosed with HNPCC (Lynch Syndrome II). I had a right hemi colectomy in 2000 and had five months of chemo.7 months later I was diagnosed with a separate primary - clear cell uterine cancer. We have a long family history of colon and other cancers. I did have a total hysterectomy,6 months of chemo and 2 radiation treatments in 2001.
My daughter is 29, last year she had a colonoscopy and had severe dysplasia. She also had a right hemi colectomy. She has two sons and had her tubes tied after her youngest so she made the decision to have a total hysterectomy last December. Hopefully eliminating uterine, cervical, and ovarian cancer. (There is cervical, ovarian, and gall bladder cancersin our family). I believe this was the right choice she made and had I not been diagnosed with the uterine cancer, my choice to have a total hysterectomy would have been the same.
My father had colon cancer in 1999 and had the genetic testing last year. He tested positive for the MLH1 mutated gene. Neither my daughter or myself have been tested but assume we are positive because we have had the cancers.
I had a colonoscopy last month and my GI doc said if I were to have the same diagnosis now, they would recommend having my colon removed.
He did say that he feels that as long as I am compliant with the followups there is no reason to remove the colon unless I were to develop another cancer in the colon.
Hope this was of some help to you. If you have any questions, I'll try to help you out.
Does your sister have any children? I will keep her in my prayers.
God bless,
tpop

[Hannah]

HNPCC means "Hereditary Non-Polyposis Colorectal Cancer," but being diagnosed with it does put you at increased risk for other cancers as well - most commonly cancers of a woman's reproductive organs, and less commonly the GI and urinary tracts. Because of that, doctors often recommend complete colectomy for anyone with HNPCC, plus a hysterectomy for women.

However, these surgeries are certainly not required and there are other options, such as aggressive annual screening (including colonoscopy, pelvic exam with transvaginal ultrasound, annual CA-125 testing, and urine cytology on an annual basis, and endometrial biopsy less frequently).

Take the time to talk to a genetic counselor and educate yourself about your options. Please keep in mind that the only way to 100% prevent colorectal cancer is a complete colectomy - but that screening methods are constantly improving as well. I know people with HNPCC who have elected to have a hysterectomy and complete colectomy, and I also know those who decided to undergo the increased screening instead of having surgery. The important thing to all of them is that they found out about their options and chose the one that was best for them.

You may make a different decision than others in your family who have been diagnosed, but that is okay. Regardless of what you do, just remember that it is your body and the decision is YOURS.

Hannah