More on germline mutations in patients with NETs 453 patients with NETs, 11.5% with a germline pathogenic or likely pathogenic variant, most commonly in MEN1 (3.5%). Other alterations noted were PALB2, SDHB, BRIP1 and MUTYH. A nice addition to the lit. onlinelibrary.wiley.com/doi/full/10....

Rage, rage against the dying of the light… False spring #6 this year in Rochester. How was your commute home from work…?

Are you an oncologist with interest in GI cancer? If so, are you interested in joining a growing academic GI Oncology practice in Rochester, MN? We have 13 GI oncs with additional 3 joining soon and still growing 😀 We are hiring! PM me if interested. jobs.mayoclinic.org/job/rocheste...

Adjuvant immunotherapy (camrelizumab) and CRT following resection of gall bladder cancer and extrahepatic cholangiocarcinoma. Small randomized trial, interesting findings in need of further validation. jamanetwork.com/journals/jam...

Want a quick update in medical oncology on July 19 (with some heme updates in the mix)...? If so, we have the course for you! I can promise excellent content/speakers and there is a livestream (virtual) option too for those who cannot attend in person. ce.mayo.edu/hematology-a...

The next frontier in radioligand therapy (RLT), radioligand-antibody drug conjugates...? Interesting data targeting TROP2 and HER2 in xenograft mouse models with orthotopic breast tumors. I am certainly keeping an eye on how this develops... aacrjournals.org/mct/article/...

PRRT is a very effective for well-differentiated NETs but associated with a low risk of treatment related myeloid neoplasms ( t-MN ). If you are interested in learning more, this is the review for you... erc.bioscientifica.com/view/journal...

Lot to unpack from the excellent NORDIC NEC2. - ORR: 31% for NEC (mostly plat/eto) - this is NOT SCLC - ORR: 27% for G3 NETs (mostly TMZ-based) - Immediate progression: 41% of NECs, 21% G3 NETs - PFS: 3.4 m NEC; 7.4 G3 NET NECs are really bad cancers! www.nature.com/articles/s41...

Did you just wake up thinking how great it wold be to have a new review on extrahepatic cholangiocarcinoma...? If so, you are in luck. Here it is, courtesy of EASL www.sciencedirect.com/science/arti...

Are you looking for a short update course in oncology this summer. Like a day-long course that covers recent data presented at ASCO and other recent meetings and publications. If so, we sure have the course for you. Online option also. @mayocliniccancer.bsky.social ce.mayo.edu/hematology-a...

It is not all about tumor shrinkage after PRRT. In this NETTER-1 analysis, PFS and OS were not different in patients where tumor shrinkage was seen. Not a new observation but a topic I discuss every week with patients. Benefits can be seen w/o high ORR... onlinelibrary.wiley.com/doi/10.1002/...

Wishing you had access to new and updated guidelines on MEN1...? Worry not, this team has you covered. www.sciencedirect.com/science/arti...

Time to rethink our approach to NET grading and use whole slide analysis (WSA) in addition to hotspot analysis (HSA) when assigning tumor grade using Ki-67? It certainly seems so.... @IHC_guy www.modernpathology.org/article/S089...

Trying to summarize nuanced treatment decisions in one slide... What to do with small NF-pNETs? Who to observe? When to intervene...? What am I missing/getting wrong...?

Looking for a review on genomics in cancers of unknown primary...? Here you have it! As I have said before, every CUP patient seen in the Mayo CUP Clinic will have genomic testing. It is a somewhat surprisingly target-rich malignancy. www.sciencedirect.com/science/arti...

DLL3 expression in high-grade NENs? Not rare at all! Check out this large study. 64% of NECs pos, 42% lung NETs, but only 5% GEPNETs are pos with the exception of G3 NETs which are 20% mod or strongly pos. DLL3-directed trials coming but not for G3 NETs. link.springer.com/article/10.1...

What's happened in imaging for CUPs in the last decades, where are we now and where might we be going...? Very good overview with detailed tables of detection rates for different modalities. The stuff you need for your next talk or paper on CUP... academic.oup.com/bjr/advance-...

Are you interested in learning more about the management of small panc NETs and how molecular analysis may predict outcomes? If so, I have just the right paper for you... 😀 Thanks to @PancPathologist, Anna Vera Verschuur, C. Heaphy, L. Brosens et al. erc.bioscientifica.com/view/journal...

In case you needed a reminder of the not-so-predictable somatostatin receptor expression of lung NETs, here you have it... Keep in mind that only 44% had uniformly positive SSTR expression and some have none at all like this patient. jnm.snmjournals.org/content/64/1...

CUP oncology is increasingly precision oncology. Patient in 60s, extensive BRG-1 deficient undifferentiated poorly diff carcinoma of unknown primary. Near complete but not durable response to docetaxel/cisplatin/pembro followed by durable response to adagrasib for KRAS G12C mut.

Should we routinely add ICIs to chemo (platinum/etoposide) in extrapulmonary NECs? I think no. Yes, I realize the outcomes in advanced NEC are poor but we simply do not have the data yet. No benefit in this retrospective study. Enroll on SWOG S 2012!!! academic.oup.com/oncolo/artic...

Why are you answering emails and working on a book chapter on a Saturday…? We could be out playing.

If you were thinking of how nice it would be to have a recent review on functional NETs, you got one here courtesy of Francesca Spada and team. Excellent introduction to the fascinating world of functional NETs. www.sciencedirect.com/science/arti...

Still a little confused on the difference between grade and differentiation in panc NETs...? Need a quick primer on the pathology of pNETs including the genetic/molecular alterations. If so, Dr's. Rindi and Inzani have you covered. meridian.allenpress.com/aplm/article...

It may still be dull, gray and brown but spring is in the air in Minnesota. Unseasonably warm day, the fields are freshly tilled and I am off today. What better than a good dose of gravel riding. Just what the doctor ordered…

Not sure about you but I find this depressing.. Of 5059 respondents, 47.7% reported fewer than 15 days of vacation in the last year, with 9.8% taking 5 or fewer days. Personally, I take 5-6 weeks off every year and rarely log into the EMR or do work. www.mayoclinicproceedings.org/article/S002...

Look, I am posting a non-NET, non-CUP paper! Weekend warrior exercise does seem to have similar protective benefits as more regular exercise throughout the week. Very large study from the UK Biobank. What about us who do both...? 😀 www.mayoclinicproceedings.org/article/S002...

PRRT + PARP inhibitors in advanced NETs? To date, no trials have convincingly shown that PRRT + drug X (whatever that may be) is better than PRRT alone. The addition of PARPi seems to add little early toxicity but larger trials needed to show efficacy. jnm.snmjournals.org/content/earl...

Who doesn't like good clinical reviews...? As my rectal cancer practice shrinks (and my NET/CUP/PDAC practice grows), I find this a great and practical, even as a seasoned GI oncologist. www.sciencedirect.com/science/arti...

Preparing for the ENETS annual conference next month. Here is a simplified vision of what might be the future in the world of radioligand therapy for NETs (RLT which I now prefer over PRRT as a term...). Need better predictors of efficacy and tox I have never been good at predicting...

Do you want to know what is happening in radioligand therapy for patients with neuroendocrine tumors? What is hot and what is not...? if so this team (which has not yet migrated over to BlueSky...) has you covered... 😀 onlinelibrary.wiley.com/doi/10.1111/...

If you needed a reminder of the poor outcomes of patients with NECs, here you go. The current approach of platinum/etoposide yields the same disappointing results as in prior studies and outcomes of 2nd line therapy are poor. Better therapy sorely needed.

This is a valuable study on the changing epidemiology of colorectal NENs. Not surprisingly, there is increase in the incidence of well diff NETs but the incidence of NECs remains the same. Also not surprisingly, outcomes of colorectal NECs are poor. #oncsky

Liver resection in patients with high-grade neuroendocrine neoplasms, should that be considered...? Pts with G3 NET & NEC certainly have inferior outcomes but some may benefit from surgical debulking. Who are these patients? How can we better select? #oncsky www.sciencedirect.com/science/arti...

Performance of FDG PET in patients with CUP. Sensitivity and PPV were 58% and 92% and FDG PET therefore reasonable upfront imaging study. Others have reported similar findings but in our CUP clinic, the utility seems less. I would guess <30% sensitivity... www.thieme-connect.com/products/ejo...

Response assessment following PRRT in patients with metastatic NETs. Not as simple as one might have thought... Response heterogeneity in all patients in this small study. jnm.snmjournals.org/content/earl...

What to do with carcinoid crisis? 🦓 A frequent concern for patients with #NETs having surgery We put together an international 🌎🌍🌏 multidisciplinary expert consensus statement 📢 Now open for feedback! Sign-up here 🔗 surveymonkey.com/r/periopcarc... 📆 Open until March 3rd!

Is the other ALT, alternative lengthening of telomeres, ready for routine use in pts w/ resected panc NETs...? I think so. In this study of large pNETs (≥3cm), ALT+ provided added prognostic info on recurrence but was not associated with OS. #oncsky onlinelibrary.wiley.com/doi/10.1111/...

Type 2 DM (DM2) and new-onset DM2 are associated with higher risk of pan NETs but longstanding DM2 was not. Nonsignificant association with family hx of PDAC and inverse association with alcohol use. No association with smoking, obesity and aspirin use. erc.bioscientifica.com/view/journal...

Can the therapy of grade 3 neuroendocrine neoplasms be distilled down to a single slide? Not sure, but here is my attempt for the upcoming NANETS Regional Conference in Mexico City next weekend... Lots of low-ish quality data and I left out the NABNEC data on nab-pacli + carbo.

Functional imaging in NECs and much more... Check out this excellent paper. Lots of goodies in here such as >90% of NECs and G3 NETs FDG PET pos. Also, 66% of NECs pos on SSTR PET. What does that mean? The SSTR is not (yet) a good NEC target. erc.bioscientifica.com/view/journal...

Here is an excellent review on cervical NECs, a deadly disease often affecting young women. A paper dedicated to the late Dr. Michael Frumovitz, a true expert on this rare entity. www.sciencedirect.com/science/arti...

It is not surprising to see that not all G2 panc NETs are created equal. Why would pNET w/ Ki67 of 4% behave the same as 20%. Indeed, they don't. Time to split up the G2 to G2a and G2b w/ 12% cutoff? G2b clearly more aggressive, more mets etc. link.springer.com/article/10.1...

Unusual NET imaging this week: Tiny small bowel NET with metastasis to the right masticator space... Also a reminder that tiny sbNET primaries can have substantial mesenteric nodal mets. Stable without therapy but lanreotide was discontinued to to recurrent hypoglycemic episodes.

It’s a beautiful day in the Mayo neighborhood…

Just a reminder that NETs are radiosensitive tumors. Locally advanced, nonmetastatic, unresectable (extensive vascular involvement, cavernous portal vein transformation) G2 pancreatic NET (Ki67 9%). CAPTEM x12 with good partial response, followed by CRT with capecitabine. 23 mos post CRT #OncSky

Take good history and do a physical exam before ordering tests…

Another stressful morning commute… Soundtrack provided by Sturgill Simpson… 🤠

New epidemiology data on gastroenteropancreatic NETs (GEP-NETs) in the US. As I have said before and will continue saying, the incidence keeps rising (probably mostly from better diagnostic methods). jamanetwork.com/journals/jam...