Reviewed By: Pramod Kerkar, MD, FFARCSI

What Is The Prognosis For Lynch Syndrome?

Lynch syndrome or hereditary non polyposis colorectal cancers (HNPCC) prognosis depends on whether the patient will get a cancer during their lifetime & if they get a cancer then whether the cancer can be cured or not. Lynch syndrome patients have 20 -80% risk of getting colorectal cancers, 1-13% risk of getting stomach cancers, women are at risk of getting 15 – 60% endometrial cancer & 1-38% ovarian cancer. A study done on HNPCC patients & sporadic colorectal cancer patients show overall survival of colorectal cancer (CRC) in patients with HNPCC is better than sporadic colorectal cancer patients. The different outcome probably relates to the specific tumorigenesis involving DNA mismatch repair dysfunction. Studies done on lynch syndrome patients with endometrial cancer & sporadic cases show no difference in the outcome.

What Is The Prognosis For Lynch Syndrome?

Lynch syndrome or hereditary non polyposis colorectal cancers (HNPCC) prognosis depends on whether the patient will get a cancer during their lifetime. If they get a cancer whether the cancer can be cured or not. Lynch syndrome patients are more prone to get certain cancers. Following is a list of cancers lynch syndrome patients are at increased risk of getting with the percentages.

  • Colorectal cancer - 20 – 80 percent
  • Stomach cancer - 1 - 13 percent
  • Hepatobiliary tract cancer (bile duct /liver) - 1 - 4 percent
  • Urinary tract cancer - 1 - 18 percent
  • Central nervous system or brain tumor - 1 - 3 percent
  • Pancreatic cancer - 1 - 6 percent
  • Small bowel cancer (intestines) - 1 - 6 percent
  • Cancer risk of females with Lynch syndrome
  • Ovarian cancer - 1 - 38 percent
  • Endometrial cancer - 15 - 60 percent`

Survival Rates Of Lynch Syndrome Patients With Cancer

The following data are retrieved from research studies published about the survival rates of lynch syndrome patients with cancer.

Colorectal Cancer

40 individuals with Lynch syndrome (25 men & 15 women) & 573 individuals with sporadic colorectal cancer (312 men & 261 women) participated in the study. The study results revealed the median age at diagnosis of the primary colorectal cancer (CRC) was 46.8 years in the Lynch syndrome series & 61 years in sporadic colorectal cancer club. In lynch syndrome group 85% had a right cancer location, whereas sporadic cancer class 57% has right cancer location. 70% of Lynch syndrome patients were in early cancer stages (Dukes’ A & B) & 61.6% were in early cancer stages in the sporadic group. The crude 5-years cumulative survival after the primary colorectal cancer was 94.2% in Lynch syndrome patients & it was 75.3% in sporadic cancer patients. The results show that overall survival of colorectal cancer in individuals with Lynch syndrome is better than sporadic colorectal cancer individuals.

Therefore, survival of Lynch syndrome individuals not always depends on an early diagnosis, but probably also on distinct biologic features. This study also highlight that, microsatellite instability (MSI) mutation pattern plays an important prognostic role since colon cancer with microsatellite instability has a better prognosis than tumors without microsatellite instability.

Endometrial & Ovarian Cancer

Results of a study done in 2009 shows that the lifetime cumulative risk of endometrial cancer for females with Lynch syndrome is 40% to 60% which equals or exceeds their risk of colorectal cancer. No current evidence to suggest a better or a worst survival with endometrial cancer in individuals with lynch syndrome compared with the sporadic cases. Study also suggest family history, medical history & tumor testing provides an efficient diagnosis of lynch syndrome in individuals with endometrial cancer. Gynecologic cancer screening for females with lynch syndrome includes annual endometrial sampling & transvaginal ultrasonography should begin at the age of 30-35 years.

There hasn’t been many studies about the survival of lynch syndrome individuals with ovarian cancers. However, studies suggest females with lynch syndrome with additional risk factors, who have completed their family & who are at the age of 40-45 can undergo the prophylactic hysterectomy & bilateral salpingo-oophorectomy to prevent endometrial & ovarian cancers.

Also Read:

Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: November 17, 2018

This article does not provide medical advice. See disclaimer

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