2026 Hereditary Cancer Awareness Week: What's New in NCCN Version 1.2026? Key Updates in Hereditary GI Cancer

Jennifer Fijor, ARNP, CGA-IGC Communications Committee member

The NCCN Guidelines for Genetic/Familial High-Risk Assessment were updated in June 2026, with real changes across colorectal, endometrial, gastric, and now esophageal cancer for the first time.

Here's what changed, and where the field still doesn't agree:

1. Universal Lynch syndrome assessment expands, but it wasn't an easy vote

Version 1.2026 now recommends universal Lynch syndrome assessment for all patients with colorectal, endometrial, or gastric cancer, with germline multigene panel testing specified for colorectal cancer. For endometrial cancer, panel testing is a full recommendation regardless of age. For colorectal and gastric cancer diagnosed at 50 or older, it's a new Category 2B recommendation. Category 2B means the panel reached consensus, but not strong consensus. Somewhere between 50 and 85% support, not the near-unanimous agreement you'd see with a Category 1 or 2A.

That 2B designation got pushback. There's real concern about whether we can actually deliver this volume of testing, and some panel members want better yield data before expanding further. How often are we actually finding something like FAP at this age? The age-50 cutoff exists because yield drops off in older patients with colorectal and gastric cancer, so this is an attempt to target testing where it's more likely to find something.

Universal tumor testing is still part of the picture, but the guideline is upfront that it's a stepwise process and it's cumbersome. Germline testing may catch patients who'd otherwise get lost, especially those with limited access to their own family history. 

2. New risk tables for EPCAM deletions

EPCAM 3' deletion risk is now split by whether MSH2 is also involved.

With MSH2 involvement, risk is on par with standard MSH2-associated Lynch syndrome. Without it, colorectal cancer risk runs about 75% by age 70, and endometrial cancer risk drops to 12–25%. Gastric cancer data is minimal but appears increased.

Worth saying plainly: the panel itself flags these estimates as resting on a small number of older studies with likely ascertainment bias. This split is real and now formally recognized, but the numbers behind it are still thin. Hoping to see more research here in the future.

3. Salpingectomy gets its own place

The language around risk-reducing surgery for Lynch syndrome now explicitly allows opportunistic salpingectomy, removing the fallopian tubes without the ovaries, as an option for premenopausal patients who aren't ready for a full oophorectomy, especially in the setting of risk-reducing hysterectomy. Worth being clear with patients that the data supporting salpingectomy alone to prevent Lynch-associated ovarian cancer is limited. It's a bridge option, not an equivalent one.

4. Separately, PMS2 is still absent from the minimum gastric cancer testing panel. 

No definitive evidence of increased gastric cancer risk with PMS2 variants. If there's a family history suggestive of Lynch syndrome plus a personal or family history of gastric cancer, those patients will still get tested regardless.

5. Polyposis terminology shifts, and the evaluation threshold gets formalized

What used to be called colonic adenomatous polyposis of unknown etiology, or CPUE, is now idiopathic adenomatous polyposis, IAP.

The prior guideline already flagged 10 or more adenomas as worth a look. What's new is a formal, named category: 10 to 19 cumulative adenomatous colorectal polyps is now its own indication for genetic evaluation, distinct from the 20-or-more threshold that triggers genetic testing. That's a deliberate two-step distinction. Evaluation means stepping back and looking for other signs, extraintestinal manifestations suggestive of FAP, for example, before deciding whether testing is warranted. Testing means the evaluation already pointed that way.

Not everyone in the 10 to 19 range needs testing. Think about a 75-year-old who just crossed 10 polyps with no other family history versus someone presenting at 45 with 10 advanced adenomas on their first colonoscopy. Very different yield. The new category widens who gets a closer look, not who gets tested.

6. A new section: hereditary esophageal cancer

For the first time, the guideline gives hereditary esophageal squamous cell carcinoma its own section, built around two syndromes: tylosis, tied to RHBDF2, and Fanconi anemia. There's now a dedicated screening table with ages and intervals for each. Adenocarcinoma is excluded here. The literature search didn't find enough evidence for a familial or inherited pattern in esophageal adenocarcinoma.

7. New gastric polyp pathology tables for FAP

There's added detail on what different gastric polyp types actually mean for cancer risk and follow-up in FAP patients. A polyp in the antrum should come out, as it's more likely to be adenomatous. And fundic gland polyps with low-grade dysplasia don't, on their own, mean a patient needs a total gastrectomy, though other polyp types in these patients can mean something different.

The bigger picture

Version 1.2026 is a field still working out where the lines should be. How much testing volume the system can actually absorb, how much yield justifies widening criteria, and how to formalize distinctions, like EPCAM with versus without MSH2, or evaluation versus testing in polyposis, that used to get handled case by case. Keep an eye on that Category 2B vote next year.

For the full discussion, including the panel debate behind some of this, listen to the accompanying CGA-IGC podcast HERE.

Reference: National Comprehensive Cancer Network. Genetic/Familial High-Risk Assessment: Colorectal, Endometrial, Esophageal, and Gastric. Version 1.2026. Updated June 16, 2026.

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