Neurofibroma, Neurofibromatosis-1, and Low Grade MPNST
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- čas přidán 10. 09. 2024
- A complete organized library of all my videos, digital slides, pics, & sample pathology reports is available here: kikoxp.com/pos... (dermpath) & kikoxp.com/pos... (bone/soft tissue sarcoma pathology).
Learn about the different types of neurofibroma and their pathologic features in this video. I also discuss the scenarios where NF-1 should be considered, the features that are concerning for transformation into low grade malignant peripheral nerve sheath tumor (MPNST), and the immunohistochemical staining patterns of both neurofibromas and low grade MPNST*** (see note below).
Entities/topics discussed (click timestamp to go to that part of the video):
Solitary/Localized neurofibroma: 0:20
Schwann cell cytologic features: 4:25
Myxoid change in neurofibroma: 12:00
Plexiform neurofibroma & NF-1: 13:00
Diffuse neurofibroma: 17:35
Diffuse NF vs DFSP: 19:00
IHC in neurofibroma: 21:00
Degenerative atypia in neurofibroma vs Low grade MPNST: 38:00
IHC in low grade MPNST: 47:00
A list of my nerve sheath tumor videos: (links coming soon)
- Schwannoma:
- Malignant peripheral nerve sheath tumor (MPNST) - classic, epithelioid, & Triton:
- Nerve sheath myxoma vs cellular neurothekeoma:
- Neurofibroma, Neurofibromatosis-1, and Low Grade MPNST
- Palisaded encapsulated neuroma: kikoxp.com/pos...
***Please note that there is some debate about the term "low grade MPNST". A 2017 consensus statement suggested use of the term “atypical neurofibromatous neoplasms of uncertain biologic potential (ANNUBP)” for neurofibromas in the setting of NF1 that display at least 2 of these features: Nuclear atypia, loss of neurofibroma architecture, high cellularity, and/or mitotic activity greater than 1/50 HPF but less than 3/10 HPF. Nuclear atypia alone without these other features is thought to be merely a degenerative phenomenon that is not concerning for malignancy in this setting, as discussed in the video. For more info, read this paper: www.ncbi.nlm.n...
Please check out my Soft Tissue Pathology & Dermatopathology survival guide textbooks: bit.ly/2Te2haB
This video is geared towards medical students, pathology or dermatology residents, or practicing pathologists or dermatologists. Of course, this video is for educational purposes only and is not formal medical advice or consultation.
Presented by Jerad M. Gardner, MD. Please subscribe to my channel to be notified of new pathology teaching videos.
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A complete organized library of all my videos, digital slides, pics, & sample pathology reports is available here: kikoxp.com/posts/5084 (dermpath) & kikoxp.com/posts/5083 (bone/soft tissue sarcoma pathology).
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@Thaddeus Joseph Glad I could help :)
One important differential diagnosis for neurofibroma with atypia that I forgot to mention: desmoplastic melanoma. They can have areas that very closely mimic neurofibroma, and they often have scattered pleomorphic cells. The majority of desmoplastic melanomas arise on the head and neck of older patients with a lot of sun damage, so be very careful about apparent neurofibromas in that setting if they have atypia. Mitoses, overlying melanoma in situ component (only present around 50% of the time), and/or lymphocytic aggregates are all clues to the possible diagnosis of desmoplastic melanoma. I'll do an entire video on desmoplastic melanoma at some point in the future.
Hope you will also do a review btwn the follicular neoplasms / apocrine&eccrine / sebaceous neoplasms as well! Thanks for these great videos.
doc i want to send you my pictures what my face looks like ..
Have you ever seen a case of angioneurofubroma? what should I look out for in such a case.
what is neurofibroma's treatment??
Please note that there is some debate about the term "low grade MPNST". A 2017 consensus statement suggested use of the term “atypical neurofibromatous neoplasms of uncertain biologic potential (ANNUBP)” for neurofibromas in the setting of NF1 that display at least 2 of these features: Nuclear atypia, loss of neurofibroma architecture, high cellularity, and/or mitotic activity
greater than 1/50 HPF but less than 3/10 HPF. Nuclear atypia alone without these other features is thought to be merely a degenerative phenomenon that is not concerning for malignancy in this setting, as discussed in the video. For more info, read this paper: www.ncbi.nlm.nih.gov/pubmed/28551330
Jerad Gardner . God bless you for your humble way to teach!!
Jerad, thankyou for posting these videos. My 8 year old with NF1 was just diagnosed with a MPNST in his abdomen. This information in increadably helpfull in understanding this.
I am so very sorry to hear about your child's diagnosis. As a father of three young kids, it particularly hurts whenever I have to make a malignant diagnosis in a young child. I am glad that this video gave you some additional information. There are a variety of sarcoma Facebook support groups. I have volunteered in some of these; patients seem to find them very useful. You may wish to join some of these groups for additional support. My best wishes for healing and peace to you, your son, and your entire family during such a difficult time.
Jerad Gardner thank u so much. I have like this. What can I do? I live at Australia. Do u have any recommendation doctor for me?
Sorry I didn’t see your post sooner. I think that genetic counselors are good professionals to help coordinate care for NF-1 patients. Many different types of doctors may need to be involved in the care of a patient with NF-1 but it’s important to find someone who has a lot of NF-1 experience (if possible) to be the coordinator and oversee everything in my opinion. I feel like genetic counselors in the USA at least often can fill that role or at least refer the patient to someone who can. There are NF-1 patient groups on Facebook. You may find other Australians who can refer you to good doctors via those groups. Good luck!
Thanks a lot Dr. Jerad Gardner Sir. I heartily appreciate all your efforts and valuable time spent to teach us. Very useful tips given in the lecture which can be used while reporting cases of Neurofibroma. The cases selected by you for teaching are awesome.
Here are two recent papers regarding neurofibromas with atypical features and transformation to MPNST in the setting of NF-1: www.ncbi.nlm.nih.gov/pubmed/28551330 & www.ncbi.nlm.nih.gov/pubmed/29409029. This is a very challenging area of soft tissue pathology, and our understanding of these lesions is still evolving. I will discuss MPNST in much more depth in the near future in a full length video. I'll add link here once it is live.
I’ve been looking for a good explanation of what a neurofibroma is after having one removed. I do a lot of pathology and ICC/IHC work so this was fun to watch :)
I’m glad you enjoyed it!
These videos are so helpful! Thank you!
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Thank you profoundly Prof. You make clear discussion.
Thank you Jerad Gardner and congratulations for this superb work.
I think the lesion shown at minute 22:00 is Hybrid Schwannoma/Perineurioma. I hope to hear a feedback after you do EMA immunostain to high light the finger-print pattern that could be appreciated in your S100 stain.
You are making a remarkable impact in pathology education.
Really fantastic videos.
Thank you for the feedback! I believe the lesion at 22:00 is neurofibroma not schwannoma. But I think you are right about a perineurial component being responsible for the whorled pattern. Neurofibromas sometimes have prominent perineurial component like this, but I usually just call them “neurofibroma” rather than invoking the hybrid tumor nomenclature. I prefer hybrid tumor term when there are clearly two different areas of tumor merging into one another. I don’t use that term very often, although
some other pathologists do. I think it’s a matter of style and preference mostly.
Jerad Gardner
Your prompt reply is appreciated. May I take chance to suggest hybrid benign peripheral nerve sheath tumors as a coming video? I believe you are making a difference by doing that. Personally I'll get to learn from it as I did with your previous videos.
I agree that the concept with its proposed criteria is relatively recent and might not be agreed upon universally in the word of histopathology. I find it interesting. I am sure you are following that better than me and being part of such debates.
For readers interested in the point:
www.ncbi.nlm.nih.gov/m/pubmed/19623031/
superb video Jerad. I always wondered why plexiform NF has an intact perineurium around the "worms"... now I know! also thanks for including the important clinical aspects of this and the other conditions. pathologists need to be wary of making life changing diagnoses!
One of the most important lessons I was ever taught was from my amazing mentor, Dr. Mary Schwartz: “always know what will happen to the patient because of what you write in the pathology report”. That pearl has served me well throughout my career and always made me focus on the patient.
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Thank you so much. Extremely helpful presentation.
Thank you a lot.
Your videos are so helpful.
You are intelligent.
Very informative video.
U are amazing ...with so clear concepts.... I m a big big fan...
Newrofribama any titmant please. help me my Newrofribama problem 😭 any madicin
Very nice presentation.
superb video.
your great efforts are highly appreciated.
thank you so mutch doctor
Thank you so much Sir
Thank you very much!
Wow ! Amazingly usefull video ! Subscribed ! I'm a thoracic pathologist currently working on a Neurofibroma case in a NF1 patient that was previously operated for a pheochromocytoma. Mine is a 5 cm mediastinal mass that was discribed by the thoracic surgeon as being centered on the left vagus nerve. I see lots of degenerative atypia, but haven't find any hypercellular fascicles yet nor mitosis or loss of S100 areas, but I will sure look at it very closely and refer it to our BST/Dermpath specialist if I find any worrisome features you discribed. I really enjoyed your video ! Thank you
Glad I could help! FYI: A complete organized library of all my videos, digital slides, pics, & sample pathology reports is available here: kikoxp.com/posts/5084 (dermpath) & kikoxp.com/posts/5083 (bone/soft tissue sarcoma pathology).
Thanks alot ❤
Thank you for great knowledge..
very good
Thank you so much!!
Thank you soo much
Hi my name is Agustín Aguirre senior pathology with private practice.
How can I share with you a case of neurofibroma like lesion?
Sir I have neurofibroma in chest and belly all are under skin, moving and soft. Today dr. Said after ultrasound. Sir I am 27 years old with no family history of it. Please Sir is it dangerous? Internet says it increases the chance to cancer and is the tumour comes to skin?
Great one. I appreciate how you acknowledge anyone who has taught you something. Gratitude is rare to find these days. I guess universe will give it back to with people saying , Dr Gardner doesn’t like the word WAVY nuclei, somewhere in a reporting session... Have you come across plexiform schwannoma too. Is palisading going to differentiate that?
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Tanx so much Jerad! u r an awesome teacher. Now I know why S100 stains focally in a NF (vs a Schwannoma). I think the nuclei of Schwann cell resembles the tilde symbol ~ :)
Oh i like that too! ~~~
This was amazing!
Sir, If i have to classify or enumerate ''Soft tissue tumors of Oral Cavity''. Can u help please. Its really important, there are many confusions, i cant prepare the seminer.. Plz help me.
can you help me with systematic approach to diagnosis of sarcomas, The basics. Thank you.
Nice is so useful, so many practical tips thank you! @2:19 it looks like some of the nuclei here have intranuclear inclusions at approx 1 O'clock and 9 o clock relative to the mast cell in the middle, is it just some kind of artifact of processing? Similar changes can be seen @6:26 as well
Not sure what those are. Many cells can have variable numbers of nuclear inclusions. I do not think of that as a diagnostically useful feature in this setting.
Thank you ı apprecıate you
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Dr Gardner, can plexiform neurofibroma be mistaken for a nerve sheath myxoma? Both are multinodular, myxoid and express PS100. Thank you.
They look pretty different to me, but I suppose they could occasionally get confused with one another. Here’s my video about nerve sheath myxoma. kikoxp.com/posts/4103
@@JMGardnerMD Plexiform neurofibroma often have a collagen background (sometimes shredded carrot collagen), and mast cells, and even if they often have myxoid material, they are not as myxoid as nerve sheath myxoma. Therefore neurofibroma are not true myxoid lesions (is that the reason you did not include them in the myxoid lesions playlist ?). Besides, in nerve sheath myxoma, the cells are more epithelioid and have tendency to form cords. That is what I understood from the two videos. Thank you very much for your time dear Dr.
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Have you used H3K27me3 in the setting of MPNST transformation in conjunction with S100/SOX10?
Yes indeed. Here is an example from my full length MPNST video: czcams.com/video/5szCMG1EIAs/video.htmlm18s
Sir neurofibromatosis bajse kya cancer ho sakta??? Plz boliye
NF has a 10% chance of turning cancer.
@@MrJamiez sir nf bajse kya log andhe ho jate he kya & spinal cord effect ho sakta lekin mera kehne ka matalab paralysis nhi ho ga naa??
@@Sushreescreation_607 English?
@@MrJamiez people will not be blinded and paralized for neurofibromatosis?
@@Sushreescreation_607 type 2 neurofibromatosis, can cause blindness but not all the time & sometimes deafness. I have NF1.
I want to meet u so much