Extramammary Paget's disease (EMPD) case series from the Mayo Rochester Cancer of Unknown Primary group. Excellent work by Drs. Fuentes-Bayne, Halfdanarson, Suleiman and the others co-authors. Well discussed by Thor.
My other hat in clinic than NET/GI is unknown primary cancer (CUP) and rare cancers that do not fit neatly in other tumor groups. One such cancer is extramammary Paget's disease (EMPD). It is almost as if I like to work in the data-free/data-limited zone... 😉
Metastatic EMPD is a rare malignancy, in fact, extremely rare. Therefore, very little is known about the management.
Thanks to @SuleimanRiham, @hfuentesbayne, @StephanThomeMD and others, we now have a little more information.
A few highlights:
-- Median overall survival (OS) 20.0 months
-- IHC showed universal CK7 and GATA3 positivity, with 80% androgen receptor (AR) expression.
-- HER2 was positive (3+) or amplified in approximately one-third of cases.
-- NGS revealed frequent mutations in TP53 (68.8%), ERBB2 (50%), and CDKN2A/B (50%). A “triple-hit” deletion at the 9p21 locus (CDKN2A/B/MTAP) was identified in 25% of cases.
-- Genomic profiling showed strong similarity to HER2+ breast and urothelial carcinomas.
-- HER2-directed therapy was associated with longer overall survival (median OS 73.0 months [95% CI, 0–177.9] vs. 19.0 months (NS)
https://t.co/ZTaX8b7qq8
CDX2 expression as a predictive and prognostic biomarker of 5-FU response in cancer of unknown primary in @ESMO_Open. Higher ORR and CBR as well as significantly longer mPFS1 and mOS with 5-FU- vs non-5-FU-based regimens in pts with CDX2+ CUP. https://t.co/oAPIVdV6Ba
📣What is the role of immune activation on the prognosis of early #TNBC if chemo is not given? We evaluated ~ 2,000 pts w eTNBC from 13 institutions around the globe to answer this. See our results published today in @JAMA_current
https://t.co/N0fMyRWjP3
https://t.co/pb66TroSSj
Introducing a one-stop site to estimate risk of progression in myeloma, SMM, MGUS. Bookmark it!
#MedTwitter@myelomaMD@eamadoutoure
Alternate Link: https://t.co/OpldwF6gsy
@DavidSteensma@MayoClinic@MayoHemeOnc Dr. Bob Phyliky mentored me as well and was definitely one of the wisest, kindest benign hematologists I had the privilege and joy to know. Thank you David for sharing your memories of training under him. Bob’s teaching on copper deficiency eg helped me diagnose a few. ❤️
Our new rapid guideline update recommends that clinicians now offer low-dose olanzapine once daily to improve weight gain & appetite, as well as offers guidance for those pts that cannot tolerate the low dose. https://t.co/7zHOJxjCn7 #ASCODailyNews#SuppOnc
Cervical cancer is a preventable disease, but inequitable access to the HPV vaccine puts the lives of many women and girls at risk. Work by Indonesia’s Minister of Health and many partners to bring HPV vaccines to girls across the country will save lives.
@OncoThor Thor, thank you for highlighting this interesting paper on using genomic data and a machine learning approach to help better manage patients with CUP. Even in this diverse group it appears that targeted therapies carried the most benefit. Nice to see that!
@VincentRK@NEJM One of the great physicians at Mayo Rochester whose work on myeloma and its precursor diseases is truly fundamental to our understanding. All the best to him and his family as he enters a more than well deserved retirement.
@OncoThor @DrSteveMartin @hfuentesbayne Right on both points @OncoThor A patient presenting with metastatic disease and a working diagnosis of CUP poses diagnostic and therapeutic challenges that a dedicated „intake clinic“ can help address and offer these patients access to treatment trials.
@OncoThor You put it well. CUP patients deserve the full benefit of modern genomic testing, and circulating cell free tumor DNA and RNA gives them that chance, when tissue is hard to access. E.g. FDA approved tissue agnostic treatments for BRAF, NTRK, RET, dMMR, high TMB.
@DrJoelGordon Joel, I am confident your experience and humanity in matters EHR/medicine and adult education for medical professionals will make your presentation shine! Have a safe trip. Stephan
Good news for NET patients! Mayo Clinic Laboratories (@mayocliniclabs) is now offering plasma 5-HIAA measurements. Much more convenient than 24-hr urine collection (overnight fast) and as accurate. Turnaround time only 3-6 days! More information below.
https://t.co/y4e9zfM2vn