Low-Grade Fibromyxoid Sarcoma (Evans Tumor) 101...Explained by a Sarcoma Pathologist

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).
Low-grade fibromyxoid sarcoma (LGFMS; aka Evans tumor) is a very unusual low grade sarcoma with fibrous and myxoid stroma. It has several important clinical and histologic features that I discuss in detail in this video. Also discussed is hyalinizing spindle cell tumor with giant rosette, which is a unique morphologic subtype of LGFMS (click here: 27:55).
Note: despite the similar sound of the name, this tumor is unrelated to myxofibrosarcoma.
Additional relevant videos:
- FIBROmyxo vs MYXOfibro Sarcoma Made Simple: kikoxp.com/posts/3705
- Low grade fibromyxoid sarcoma (LGFMS): kikoxp.com/posts/4189
- Myxofibrosarcoma (full video): kikoxp.com/posts/4190
- Perineurioma (vs LGFMS at 14:50): kikoxp.com/posts/3694
Additional suggested reading:
Evans HL. Low-grade fibromyxoid sarcoma: a clinicopathologic study of 33 cases with long-term follow-up.: www.ncbi.nlm.nih.gov/pubmed/2...
Thway K, Fisher C, Debiec-Rychter M, Calonje E. Claudin-1 is expressed in perineurioma-like low-grade fibromyxoid sarcoma. www.ncbi.nlm.nih.gov/pubmed/1...
Billings SD, Giblen G, Fanburg-Smith JC. Superficial low-grade fibromyxoid sarcoma (Evans tumor): a clinicopathologic analysis of 19 cases with a unique observation in the pediatric population: www.ncbi.nlm.nih.gov/pubmed/1...
Sedrak MP, Parker DC, Gardner JM. Low-grade fibromyxoid sarcoma with nuclear pleomorphism arising in the subcutis of a child. www.ncbi.nlm.nih.gov/pubmed/2...
Doyle LA, Möller E, Dal Cin P, Fletcher CD, Mertens F, Hornick JL. MUC4 is a highly sensitive and specific marker for low-grade fibromyxoid sarcoma. www.ncbi.nlm.nih.gov/pubmed/2...
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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Kiko: kikoxp.com/profile/jerad_gard...
Facebook: / jmgardnermd

[@cyanofelis]

Awesome! You're the best pathologist ever especially for enabling so many others!!!

[@ShashidharVenkateshMurthy]

Just like attending slide tutorial 24/7...! thanks for the good service.

[@AnatomyHelper]

absolutely incredible. Thank you!

[@rabiataseer8369]

Thanks, you are excellent.God bless you and your family.

[@missknowall]

Just watched this video for the nth time. Amazing how every time I learn something new out of it.

[@bethunewang5632]

Great talk. Thanks.

[@shadana.s.3575]

i'm speechless. u r a life saver. your videos are fantastic and full of fabulous informations.

[@gowripriyag1213]

Thank you so much. Great video.

[@jennywallace7918]

you are wonderful for doing this for all of us. thank you!

[@JMGardnerMD]

Thank you! That’s so kind. It is my honor to do this to help my colleagues better help their patients.

[@trishitabhattacharya3189]

wonderful video Sir! the way you explain the intricate details is so captivating..Thanks again Sir

[@sonja30]

I was just diagnosed with this kind of cancer. I’ve had the tumor since at least 2020. My Primary had me do physical therapy when I first went to see her. At least it’s getting done now.

[@aswathymahesh3255]

Really appreciate ur work sir... Path residents like me are going to benefit a lot from these videos... Please add normal histology and histopathological cases from other organs and systems too

[@SomeJustice19k]

My best buddy's son just got diagnosed. Should he be worried? Any advice?

[@znrsmsaa3655]

Thanks alot 😊

[@alaelidrissi2207]

V. Nice and informative. I hope in next videos if you can show us some of the deferentials for comparison and better clarity. Thanks alot

[@foodcookingandfun7702]

Thank you Dr Gardner for demystifying low grade fibromyxoid sarcoma - truly needed tutorial, thank you!

[@asifabu4609]

How you feeling now?

[@dangthinh90]

A lot of wonderful take-home basic lessons in this video, especially those wavy nuclei. Sir, thank you very much!

[@JMGardnerMD]

Glad you liked it! I’m working on a video all about the wavy fibroblastic nuclei.

[@dangthinh90]

Awesome!

[@dangthinh90]

Awesome!

[@sarahharris-bow4554]

Another great video. These are invaluable resources for studying for FRCPath Part2 exam. Thank you!

[@JMGardnerMD]

So glad you like them! More coming very soon. Have 8 videos almost ready to release

[@sarahharris-bow4554]

@@JMGardnerMD Fabulous! Thanks!

[@lopamudrakakoti]

Thank you so much sir. It's extremely helpful.please it will helpful if you do a similar video on ossifying fibromyxoid tumour

[@JMGardnerMD]

Already did a video on OFMT. kzbin.info/www/bejne/n4DQmIKYmNhob9E

[@shalemnick]

yea i am having one of this

[@Anonymous-zf7rv]

Please sir do a video on DSFP.thank you for this very informative video

[@JMGardnerMD]

Will do dfsp soon!

[@jessicamccracken254]

All of your videos are great and have helped me so much following my diagnosis. I was also thrilled to see that you've done some work in Arkansas as I live in Jonesboro. I'm being treated in Memphis and have had 3 pathologists label me as having a low grade fibromyxoid neoplasm-presumptive diagnosis of LGFMS; however, an NGS/RT-PCR test showed that I do not have any translocations. Is this possible? CD34 "focally positive" and MUC4 shows "weak/equiocal staining".

[@AnatomyHelper]

Hi! I'm in the Low Grade Fibromyxoid Sarcoma (LGFMS) Support Group on FB... if you ask your question there you might get an answer? (maybe you are already in it?)

[@LOLsaudi]

Very helpful. Fibrous lesions in particular need a lot of teaching in my opinion. Thanks for making all these videos. One question; what about the cellular arrangement in those cellular areas does it help at all? Or is it more of a component diagnosis plus the cells being bland are the only way of diagnosis of these type for lesions.

[@JMGardnerMD]

Yes there is a pattern to the spindle cells in LGFMS but it’s subtle and hard to explain and not always obvious.

[@made3698]

Very informative and rich presentation ...how to diagnose LGFMS on biopsies and have you had a case clinically suspected as GIST that turned out to be LGFMS

[@JMGardnerMD]

I have made a diagnosis of LGFMS on a core biopsy multiple times. Low grade bland spindle cells with fine collagen and subtle myxoid change and s100 negative?…think of LGFMS and do MUC4 to confirm.

[@gvsf5946]

Doctor Jerad they did biopsy of a 5.1 cm sarcoma that is enhancing and the biopsy pathologist final diagnosis was a benign solitary fibrous tumor showing perivascular spindle to ovoid neoplasm with prominent hyalinized vessels. No signifcant atypia noted or mitotic activity nor necrosis present. Immunostains show diffuse positive CD34 and STAT6 also ERG weak patchy staining also Ki-67 low grade, when they did the biopsy the doctor at the hospital decided to do an ultrasound and just mark where he was going to do the biopsy 4 samples but he did not use the ultrasound after to guide where he was going to take the samples and my Ortho Oncologist wanted a guided CT scan, when I felt the clicks the 3rd one he did hit my nerves and it shot down my leg to my foot severe pain and now it's been 8 days and I still get electric shock and burning pain on my foot and the bottom of my foot, my MRI showed invade so my question is my test benign or do I have concerns because it is pressing on my nerves and painful

[@JMGardnerMD]

I cannot give official medical advice since I’m not your doctor and you are not my patient. But I’m happy to share general info for educational purposes. Please discuss your concerns with your doctor. A tumor (benign or malignant) growing next to a nerve could potentially press against the nerve and cause pain or nerve symptoms. So pain by itself doesn’t tell me that a tumor is necessarily malignant. Solitary fibrous tumor is a bit complicated. In the past we divided it into benign and malignant. Nowadays we divide them into low, medium, and high risk groups based on features like mitoses, size, age of patient, necrosis. Low risk almost always acts benign, but for my own patients, I still recommend complete excision of any solitary fibrous tumor (when feasible) to allow for the entire tumor to be examined just to make sure there are no other more atypical areas. I’m glad to hear you are being seen by an orthopedic oncologist. They are experts in dealing with sarcomas and any rare bone or soft tissue tumor (even benign ones). I work closely with these docs and that’s who I would see if I or my family members had a soft tissue or bone tumor. Again, Please discuss all of your questions and concerns with your doctor. Best wishes for good health.

[@gvsf5946]

@@JMGardnerMD Thank you just found it is low grade cancer and they are going to remove it but it invade in my vessels

[@elliotbigland4300]

I have what they said is a lipoma in my chest below my left pectorial its about 2-3cm and is deep seated in between my ribs, on ultrasound they were certain its a lipoma... im sure its something more sinister it isn't growing but im loosing weight and cannot use my bowels and my abdomen feels inflamed... any ideas? Thank u x

[@StephOrlove]

Thank you for the informative video! I've been dealing with a well- differentiated liposarcoma/ ALT in my glute for 18 years- 5 surgeries. My last surgery was to remove tumors in my glute, sciatic notch and abdomen. The tumors in my glute and sciatic notch are back - 1 1/2 years after the last surgery. Doctor is concerned with appearance of the sciatic notch tumor and recommended a biopsy. Had a biopsy at MD Anderson and pathology report states it's a well -differentiated liposarcoma with no miotic activity, necrosis or dedifferentiated component identified. The tumor does have fibromyxoid cellular areas. Is a fibromyxoid celluar area the same as a Low-grade fibromyxoid sarcoma. I'm trying to understand the pathology report, but I'm not getting a clear explanation from the surgeons. Help! Should I be concerned?

[@JMGardnerMD]

I’m sorry for your situation. It sounds like they are just using that word “fibromyxoid” descriptively. Lots of different tumors can have fibromyxoid appearance. But that is not the same as low grade fibromyxoid sarcoma, which is a specific unique type of tumor. Coincidentally, it was actually Harry Evans, a retired pathologist who spent his career at MD Anderson, who first discovered low grade fibromyxoid sarcoma. He also wrote some very important papers about well differentiated liposarcoma. I spent time at MD Anderson during my residency training at Houston Methodist and I know the sarcoma pathologists there. They are excellent. If you are curious to see what well differentiated liposarcoma looks like under the microscope, I have a video about it here: kikoxp.com/posts/2634. Best wishes for health and healing.

[@raychand4776]

Is there a benign variant that could be called a fibromyxoma and if so how do you differentiate it from fibromyxoid sarcoma? Or are all tumours showing the pattern you have described all low grade malignancies?

[@JMGardnerMD]

No there is no benign counterpart to LGFMS. There are other tumors that mimic it as described in this video.

[@urielcabanas2695]

I just want to say Thank you so much! This is a masterclass with a lot of pitfalls. I feel like a resident again. Is it possible this tumor could have necrosis?

[@JMGardnerMD]

Thanks! It may have necrosis but most cases I’ve seen did not. 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).

[@SandhyaRamachandran]

Hi Jerad...Thank u for the excellent vdos on LGFMS & MFS. Are LGFMS & Myxofibrosarcoma , var of Fibrosarcoma? var fibromyxoid and var myxoid type, respectively.I also see in Enzinger 5th ed that the term MFS shd only be used for tumours that are > 50% myxoid & of low nucl grade. Does this still hold? Then what would be gr 4 Myxofibrosarcomas? So that brings me to the Q: If the above is true, can one variant be a Translocation sarc and the other be aneuploid?Thanks in advance.

[@JMGardnerMD]

I think of LGFMS and myxofibrosarcoma as totally unrelated. Some books categorize them as variants of fibrosarcoma But I don’t personally find that to be useful because fibrosarcoma is a controversial and confusing waste basket diagnosis historically. I essentially never use the term “fibrosarcoma” by itself in my practice. In the past, some pathologists preferred using “myxofibrosarcoma” mainly for low grade lesions and using UPS with myxoid change for high grade lesions (because clinical behavior is very different). But the WHO uses myxofibrosarcoma for both low and high grade forms of the disease, so that’s what I do. Low grade is grade 1, high grade is grade 3 and intermediate is grade 2 (I most often use at least grade 2/intermediate grade for cases where I have a partial sample of a large mass of myxofibrosarcoma). In my practice I use either two tier (low-grade vs high-grade) or three tier (grade 1,2,3) grading for sarcomas. I don’t ever use grade 4. Both low and high grade myxofibrosarcoma are aneuploid. Low-grade fibromyxoid Sarcoma is a translocation Sarcoma and is totally unrelated to myxofibrosarcoma. I hope that helps answer the questions.

[@SandhyaRamachandran]

@@JMGardnerMD Thank you so much for the clarification. I love your videos more than the books! v much apppreciate your time & patience ! thanks again!

[@pavelboico9283]

Many soft tissue sarcomas are growing veeeery slow so the patient have a time to die from stroke or sentinel demetia. Yet, there is no efective systemic therapy, only surgery whenever possible.

[@ED-ms7ho]

THANKS SOOOO MUCH FOR SHARIN!!! Can it be affecting bone? I had a case of the same patient with two synchronic lesions, one subfascial and a lytic lesion in the large bone of the same limb

[@JMGardnerMD]

I’ve never seen a case arising in bone but perhaps it can happen. You may want to do a literature search to see if it has been reported. I would want molecular proof before accepting a diagnosis of LGFMS in bone personally.

[@ED-ms7ho]

@@JMGardnerMD thanks so much, indeed!

[@marylandsmann1175]

How do I find a specialist in chestwall sarcoma?

[@asifabu4609]

I have this canser! Fibromyxoid sarcoma!..but i removed that tumor in my forearm ..i have feeling sad ! Please reply me dr!

[@spartalane13]

How can you differentiate between myxiod liposarcoma? especially if you are only getting the myxiod area? Both have bland cells with curved vessels (in 25:48 for example) please help!

[@JMGardnerMD]

They look quite different. Myxoid liposarcoma lacks the pink fibrous background (unless it has been radiated and then it becomes sclerotic). It’s vessels are much smaller and more delicate and more plexiform and branching than the vessels in LGFMS. I plan to make a liposarcoma basics video soon which will cover the details of each type. It’s on my short list of things to do

[@JMGardnerMD]

Here’s a classic example of myxoid liposarcoma. Contrast this pic to the features of LGFMS shown in the video. twitter.com/jmgardnermd/status/853609446043516931?s=21

[@spartalane13]

@@JMGardnerMD That helps, thank you for replying. I will look out for the liposarcoma videos. Your videos have been really helpful in my preparations for FRCPath 2 exam in the Uk. God bless!

[@JMGardnerMD]

So glad! Also I’m working on a basic liposarcoma video. Hopefully will have it done in next few weeks.

[@amaliyadipita6906]

❤❤❤❤❤❤❤

[@Ri-yf5oq]

Whats difference between myxoid and mucoid

[@JMGardnerMD]

I usually hear “mucoid” used more as a gross descriptive term for wet gooey gelatinous tissue. whereas “myxoid” and “mucin” are more often used microscopically to describe blue gooey glycosaminoglycan deposits in tissue. In dermpath we tend to use myxoid and mucin interchangeably. elsewhere in pathology myxoid usually refers to hyaluronic acid produced by fibroblasts in stroma or by myxoid soft tissue tumors whereas mucin usually refers to sulfated mucins produced by epithelial cells/neoplasms. I’m not sure that these are strict rules; this is just my observation of how the terms are typically used in practice.

[@bellarence4879]

Is this different from low grade fibrosarcoma? What does that mean?

[@JMGardnerMD]

Yes. “Low grade fibrosarcoma” is a term that is rarely used in modern times. In the old days we used the term “fibrosarcoma” often, but in modern pathology we recognize that most things that used to be called “fibrosarcoma” are actually something else. If you are a patient and have been given this as a diagnosis, you may want to have your pathology specimen reviewed by a sarcoma pathology expert to ensure the diagnosis is correct.

[@bellarence4879]

@@JMGardnerMD thank you so much, my brother was diagnosed aftr his operation that it has low grade fibrosarcoma and we all are worried. We really dont knw what exactly that means...before the operation The result of the biopsy was benign tumor but after the operation it has that result. Pls help wat shud we do thank u

[@JMGardnerMD]

It may be a good idea to have a sarcoma pathology expert review the Pathology slides to be sure of he diagnosis. Christopher Fletcher at Brigham and women’s hospital in Boston is one world expert.

[@ResidualSelfImage]

cell morphology of low grade soft tissue sarcoma and magnification levels and stain/markers to identify tumors in slides. Life cycle of soft tissue sarcoma ... great review of identifying cellular morphology (histological) related to soft tissue sarcoma... another video to focus on the details on test and evidence that differentiate between non maligant tumor to low grade sarcoma ..would be helpful .

[@kpopkomedy200]

I have this cancer! Fibromyxoid sarcoma!

[@JMGardnerMD]

I’m so sorry to hear this. There are a variety of sarcoma patient support groups in Facebook. You might want to join one of those to get additional emotional and social support as you deal with this disease. Best wishes for healing.

[@coldxwave]

How have you been? I just got a result that is saying I have a Low-grade fibromyxoid sarcoma.

[@kpopkomedy200]

@@coldxwave I wish I have been better. Thankfully my method of treatment as of yet is surgery, but the fear of it coming back is still high given it’s at 70%. I hope you’re doing okay.

[@coldxwave]

@@kpopkomedy200 can you send me an emal to [e-mail]. I hope we can support each other

[@asifabu4609]

@@kpopkomedy200 how you feeling now?

[@kjaythebeast2419]

I dont want to die please help me please

[@JMGardnerMD]

I’m sorry. Can you please clarify? Are you a patient who has this type of tumor?

[@kjaythebeast2419]

@@JMGardnerMD yes evans

[@kjaythebeast2419]

Im only 11 i dont wanna die

[@JMGardnerMD]

I’m so sorry about your diagnosis. This is a serious tumor but many patients survive it. Even when it recurs or spreads it is often slow growing. I can’t give formal medical advice as I’m not your doctor. Also I only diagnose this tumor I don’t treat it. This is a very rare and unusual cancer, so it may be helpful to seek medical care from sarcoma experts at a major cancer center. My best wishes for your healing and long health.

[@AnatomyHelper]

@@kjaythebeast2419 feel free to join (or have your parent join) the facebook group for this cancer. My son has it as well. Low Grade Fibromyxoid Sarcoma (LGFMS) Support Group (on facebook)

[@JMGardnerMD]

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).

[@JMGardnerMD]

Check out this myxoid tumor review article by Dr. Nicole Riddle & me (free fulltext): bit.ly/myxoidtumors

[@pathologygeek7360]

You can get a great Dermatopathology review course of 15 lectures from the Oakstone institute or payloads store: m.kzbin.info/www/bejne/gJ6xnJiohJh3ibM store.payloadz.com/details/2573416-movies-and-videos-educational-dermatopathology-oakstone.html

[@binmoos2561]

Jerad Gardner professor, please speak slowly ,you speak more like a machine-gun fires !😂