PVNS: experience and causes
Posted , 91 users are following.
I recently got diagnosed with the fairly rare condition called Pigmented villonodular synovitis (PVNS) a disorder which affects joints. In my case, it was my knee joint, as is often the case.
I'm posting this to help others better identify and understand this condition, and also to explore any possible causes.
PVNS is very easily misdiagnosed by GP's. It was misdiagnosed twice in my case, by two different doctors.
Because it's more common in the 30's and 40's age group, and the symptoms are similar to arthritis, it can be misdiagnosed as the early onset of arthritis of the knee/joint in people who are in the upper end of this age bracket.
In the case of the PVNS affecting the knee joint which results in a tumour growth at the back of the knee (as in my own situation), because the condition often arises after a trauma to the knee joint, the condition can be misdiagnosed as a simple lingering knee injury, leaking joint fluid, giving rise to a Bakers Cyst.
The symptoms of PVNS are both sporadic and very similar to both an ordinary knee injury and arthritis as the condition progresses. The joint becomes stiff and movement restricted. It's usually painless in the early stages but as it progresses it causes mild to moderate discomfort with a persistent burning pain around the joint area.
If left untreated, PVNS in its advanced stages, can render the joint completely unusable, requiring a complete joint replacement.
Here's my own experience of PVNS:
I was taking Tramadol (a painkiller) for back pain and was heavily into exercise and was also carrying a heavy bicycle every day up and down stairs. I injured my knee. This was due to the Tramadol I believe. My pain tolerance threshold was at a much higher level, so the injury didn't fully register and I continued exercising and carrying the bicycle, further straining the already injured knee.
Two years later, the knee joint gradually became stiffer and a lump appeared at the back of the knee. It became so stiff I could no longer crouch or bend down to tie my shoe laces. I went to the doctor and was misdiagnosed as mentioned. The second GP ruled out arthritis from an x-ray and to confirm his diagnosis of a leaking joint and Bakers Cyst, he requested an ultrasound scan of the joint. The scan showed an overgrowth on the lining of the knee joint and a tumour growth, resulting in a PVNS diagnosis by an orthopedic consultant.
A proof-positive diagnosis is carried out from sample by examining cells under the microscope from the joint which have the typical PVNS-like abnormal characteristic.
There's two forms of PVNS, localised and diffused. The localised version is what I have. It is a lot easier to treat using key-hole surgery with a better prognosis for recovery. Diffused PVNS affects the entire joint lining, requiring open surgery to remove all of the knee lining and it has a much higher risk (almost 50%) of returning, and requiring extensive physiotherapy.
PVNS is quite a rare condition as I mentioned. Therefore it's not encountered very much by GP's and there hasn't been much research done into the causes of the condition or why it returns in many sufferers. It's not thought to be hereditary, and as I mentioned, trauma to the affected joint is a common feature.
This leads me to wonder if certain medicines, or activities, may cause the condition. As I mentioned, in my own case I was taking Tramadol (at a normal dose of 50mg once daily) for several years, both prior to, and after my knee injury.
I'd be interested to hear from anyone else out there who suffers with PVNS and their backgrounds, in particular:
Anyone who has developed PVNS while being on Tramadol or similar drugs?
Anyone who has developed PVNS after a sports or work related injury, which they ignored?
How much time elapsed from any initial injury to PVNS symptoms and diagnosis?
5 likes, 296 replies
allyfl1003 morphix
Posted
I too have recently been diagnosed with lpvn. Again, 2 types. Localized being the easier to treat with surgery. Some of the post sound like the other type being diffuse. I have been with the best doctors possible from the start. I see the sport doctor at Stanford and all other clinics associated. A radiologist who came from Harvard started a study with a new PET/MRI scanner at the University of Stanford. He found it. When going back to a previous MRI to a compare from 3 years ago, you actually can picked it up in those images. It was assumed to be post scar tissue from a previous surgery. Meanwhile, I did have those 2 surgeries 6 years ago for nerve pain and scarring. I was pain free 2 years. Then became symptomatic. Since 2013, I have seen neurologist, rheumotologist, pain clinics,..... several other procedures, injections, but not surgeries. Nothing stuck. Again, all my doctors the best Stanford has. This diagnosis is really reliant on good imaging and a doctor who can read them. I have been on diclofenac for 6 years. I believe my lesion came sometime after my first surgeries in 2010. I do believe those surgeries were sucessful. I can list many of my struggles and frustrations which you all know to well. The time spent going to doctors and the limitations in my life have been alot. I am an athletic person. I have been able to swim for all these years. However, this has taken a long toll on myself. I still have to meet with a face-to-face with my surgeion. I am hopeful.
OConnor allyfl1003
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You are in a good place. Stay with the doctors at Stanford. Wish I were there. PVNS is difficult to diagnose, as you say, and in my case the radiologist called to check on me after her reading of the MRI. She confided that she is really good at doing this and dispite the movements that my leg was doing while in her MRI machine, she felt that she made the right diagnosis. (My leg was swelling up big time!) I wrote her up for a reward from the medical center I attend. It was the answer to a 15 year problem that was finally solved. But maybe not really, since I have elected to not have surgery for bleeding issue reasons of my own. PVNS is not that curable. Some folks on the forum have had surgery and feel that the misery will end now and others have a return of the miserable thing. With no correction, we face unpredicable severe swellings with pain off the charts that, for me anyway, take 3 weeks to resolve. I have had 5 of them in the last 4 years and if I could believe that one simple surgery would eliminate it for me forever, I would jump right in and do the suffering. Removing the lining of the knee cavity is no picnic. It usually involves a total knee replacement. Folks here on the forum can tell you all about that. I know of which you speak when you recount the toll it has taken on your life. It is hard to get our heads wrapped around the fact that we are kind of disabled if we spent all of our lives being active and mobile. Depression can set in. Don't let it! Stanford saved my life once. I hope it will save your knee. Sending peace and love.
Oldfatguy1 allyfl1003
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I am fortunatevthat my med center is also the hospital for 3 professional sports teams. My ortho and ortho oncologist certainly bring a lot to the table. My original surgeon plus his 7 partners combining over 100 years experience has never seen a case of PVNS and referred me to thevortho oncologist just fully expecting a rare cancer or blood disease
allyfl1003 OConnor
Posted
I wish there was more you could do. Is your diagnosis of the type that is called "difuse"? I hear it is much harder to treat. I have only read of radiation and total joint replacements. I am hopeful that Stanford will fix this. As you have touched on, it is really hard to diagnose. I am curious to hear what my surgeon will say after all these years of managing this. He has been gone this last 2 months since a radiologist did the diagnosis. Last time I saw my sport ortho, he had felt he had done all he could do. I knew there was something still wrong. I was determined to go back with something in my hands to show him Well, now I have it. I hear a doctor has to see 1,000,000 patients before he will usually encounter 1-2 of these cases. My radiologist said my lesion is localized and should be able to be totally removed. I figured if the Stanford doctors could not figure it out who could. Best of everything to you!
Oldfatguy1 morphix
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15 year history that didnt surface till age 65. No previous history when it broke open with severe bleeding. I had. Over 1000cc of blood removed by way of surgery and needle draining. It isn't just primary care Dr's that aren't atune to the problem. I was in a practice of 8 orthopedic surgeons and none of them had ever seen it. I had 30 rounds of radiation plus a synovectomy before finally the tkr. The tumor returned after 9 years and caused the prosthesis to come loose and needed a tkr. I won't be able to get rid all of it as there is a small growth in the center of the a nerve bundle and there is too much danger of losing the leg. I had a pediatric ortho tell me of a 9 year old that had it and needed 16 surgeries before age 16. Hers jumped from joint to joint. Another surgeon said he had seen it in the jaw of a young adult. If you google PVNS there is a lot of info available. It seems every region of every country has a different way of approaching treatment and those treatments have evolved in the 14 years I've been involved. I go to a regional teaching university hospital where they see several cases a year and the have have 2 orthopedic oncologists on staph. I guess there is research going on but it is sure low key
allyfl1003 Oldfatguy1
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Oldfatguy1 allyfl1003
Posted
Nice to be close to quality health care. We were at my sister in law's in Menlo park the night my wife had her 1st episode with Parkinson's and I had to have her taken to Stanford by ambulance. I have a nephew that owns an engineering firm in Santa Rosa and my son is headquarter and travels out of Stockton, covering all of CA and the Pacific northwest selling to the commercial food industry.
Oldfatguy1 morphix
Posted
Thanks to auto correct it turned staff into staph. You mentioned not hereditary.....yet the consensus of opinion in the Medical profession that it's genetic. What little research I've done outside of the Google search, has shown it is predominantly a disease of white, northern European extraction. I can only find a couple cases of African based blacks and none in the Latino community. My surgeon has seen 25-30 cases all Caucasian. He indicated its such a relatively new disease not much long time history available. I can't find when and where the 1st diagnosis took place. My guess only since the advent of The MRI. It's interesting butbim not sure I'm pleased about being part of this cuturial subset. I only hope that someday one of my grandkids doesn't have to telln a dr. .........oh yeah I heard about one of my grandpa's way back when, had something like this. Story was it turned him into one hell of an old grouch.
cherrie33 Oldfatguy1
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Here is your Latino case! I was just diagnosed with it yesterday. A releif to to have an answer to the last 14 years of suffering and being told "your fat come back and see me when you've lost the weight" from my last ortho...who I might add is not the picture of a fit man! The weight I might add was put on after my knee surgery for a miniscus tear and the depression I suffered from not being relieved of the pain I experienced for the last decade. I suffer from several auto immune diseases one of which is due to my bodys inability to correctly process fat. I read somewhere that PVNS also has a link to the bodys inability to correctly process fat. I am currently doing everything I can with my diet to help correct some of my other health problems but have just begun my journey with PVNS.
Oldfatguy1 cherrie33
Posted
I was diagnosed in late 2002. My 1st group of ortho's with well over 100 yrs of practice combined, had never seen a case. I saw 2 different ortho-oncologists that diagnosed and confirmed the tumor. I've had 11 surgeries and 3 different prosthesis, a couple due to staph infection. My current surgeon is an asst professor at a teaching medical center. He has seen around 3 dz cases by now as he gets referrals from a wide area. In my personal research and from his discussions, I have never heard that assessment. It is primarily a disease that hits in early teens to mid 40's. I was 65, so much for averages. There was one lady on here that had 20+ surgeries in her 32 years. Another one, 15 or 16 before age 30. A Dr in a local children's hospital had one girl who had a large number of surgeries between 9 and 16. These figures belie the fat theory. When mine broke, I bled heavy in the joint (my knee blew up by close to 4" in 3 hours) and bled to the point it was collecting in my lower abdomen and groin.
Although every area isn't blessed wit h having ortho oncologist, if you can find one it mighty help clear some of your questions. As I have said on here before, its not fun being part of tis kind of cultural subset.
I'm an old man burbs can tell you, there were times the pain had Kevin tears.
My surgeon recently told me he had a 39 year old that might require amputation because of a very, very rare and extreme case. They were having to operate almost monthly because of the bleeding.
Google kumed orthopedics/ Dr. David Anderson and see if you can find any of Dave's research
tinisha1973 morphix
Posted
Hello Morphix,
My name is Tinisha and I too was just diagnosed with PVNS. I have not had my second consultation to discuss if its local or diffuse but will be finding out very soon.
My knee pain journey has actually been going on for almost 10 years. Sometime in 2006 I feel while I was roller skating and injured my knee. At the time I did never went to a doctor and just stayed off it until felt better about 2-3 days. After the initial injury I did not have any other issue with my kneee. Im guess about 2 years later I started having issues with my knee like now being able to wear heals and having trouble going up and down stairs.
When I first started seeing a doctor about this problem an x ray was taken and I was told that ther were no breaks, they gave me a leg brace and said I just needed physical therapy. I did as the doctor asked but still had issue with my knee. At that point another eray was taken and again they stated they did not see any problem and I was told that I needed to lose wait. I was maybe 10-15 lbs "over weight" at the time. Still the issues in my knee were stil there. The next thing that I was told by my doctor was that she though I had patella femoral syndrome and the best thing to fix it is physical therapy. During this time I had requested a n MRI but this particular doctor never ordered one.
During this time the pain in my knee was coming and going and the doctors still had not given me any clear indication as to what was wrong with my knee. I ended up having medical issues non related to my knee that spanned about 7 years.
Just recently my knee started to bother me again and at this time I had a whole new doctor and medical plan. So I went my new doctor and told him of the issues I had been having for several years about my knee. This entire time I still thought that the problems with my knee had to do with the fall I had had several years prior. With the new doctor I initially went through the same steps as I had did with the previous doctor. I got a new brace and went to physical therapy for 6 weeks. At my following appointment this doctors orders an MRI. That was last month.
When my doctor got the result from the MRI back he told me that I had GCT a giant cell tumor and that the only way to fix it was to take it out. He recommended I see an orthopedic specialist. The orthepedic specialist order a bone biopsy and it shown that it was not a giant cell tumor but PVNS.
It seems that since the bone biopsy that my knee has gotten worse. Before my bone biopsy I had a lot more range of motion in my knee than I did before and it seems that the pain has gotten worse. Before my bone biopsy that the radiologist stated that my doctor will discuss my options with me once he gets the results back. That appointment is this August 25th.
After discovering that I had this rare condition I tried to do some resesrach and as you know there is not much on it. I was not given tramadol until I requested to have someting stronger than Naproxen for my pain. I was never told by my orthopedic doctor or radiologist that this condition developed from my fall. I had no other knee injuries prior to that one. Is it possible that this tumor developed from that untreated knee injury that I am not sure of.
I hope I have been able to provide you with some of the informaiton that you were looking for. I am here to really get some moral support from others with this conditions. Although, y friiends and family are very supportive but they really dont undetstand what having this condition really feels like.
OConnor tinisha1973
Posted
Hi Tinisha,
?Welcome to the smallest club in the world...those of us diagnosed with PVNS. All of us have different journeys, but what we have in common is the apparent lack of any real injury that could have caused the miserablle disease and a long trip trying to get it diagnosed properly. For me it was 15 years. I am quite old, but was always so mobile and interested in doing so many things, It has been hard to realize that I am somethimes temporarily disabled by it.. As you now know, this ailment takes the wind out of your sails, so to speak, and keeps one in a state of anticipation waiting for the next great swelling and the next pain med.
Some patients have had surgery and I have not due to another rare problem, a blood bleeding issue that is not commonly understood by doctors either and hence they defer surgery on me fearing they will do more harm than good. I agree, so I manage on my own with the periodic swellings by taking tramadol, and using Diclofenac Sodium Topical Gel. But even there, because I am in the U.S., the doctors are skitish about prescribing tramadol for constant use. I have been told that regular use of it may result in addiction so I alter my dosages and skip some, etc. trying to not rely on it. I don't have an ortho that I respect and it may be because they shy away from trying to deal with this PVNS, knowing that it comes back 50% of the time even with stripping the lining to remove it. They take one look at my age and decide that I can only come back for drainage shots and cortisone shots, but also then say that they can't give too many cortisone shots. So that leaves me confused and upset with their "treatment". One thing that this PVNS has given me, almost like a gift from on high, is the ability to speak up and demand what I want and need from doctors or medical providers. I have become very pro-active on my own behalf. They refuse to research what I have already done and refuse to take action because they fear the outcome will not be great. I understand this reluctance, but still resent it. You may have found the ideal specialist. I am not sure what country you are in, but it has been difficult for me to find the right medical provider. With only 1.8 people in one million with PVNS, I can appreciate that this is not studied in medical school.
?I have had several therapies on the knee , most of which caused me more swelling and were not suited to the PVNS. I just walk slowly and try not to twist or turn my knee in any direction. I do have other bone problems which may be causing some of the pain, but the huge swellings are purely the PVNS. I know this beuase while in an MRI machine trying to get diagnosed, my knee hemorrhaged badly. Bad bones don't do that. I know about my gentetic bleeding disorder, but the PVNS causes bleeding all on its own, so there ocurred the perfect storm- two rare conditions coming together to make my life miserable.
?I hope that your PVNS is of the tumor type which maybe has a better chance of removal and ultimate permaent recovery. I think mine is diffused although no doctor has told me that. I am thinking of contacting the radiologist who diagnosed it and asking her to explain it all to me. Surely radiologists know as much about it as any ortho doctor would. Maybe more.
?My internist who has been supportive has told me that it is uselfess trying to explain what is bothering me to other people, even doctors. She said that they will not understand and will only make me feel more frustrated. She is right. Most people just advise me to have a total knee replacement (TKR) or just rub some cream on it like I have arthritis or something. People won't understand. Those of us at the Forum will. Please write when you have been through your August 25 appointment.
You wil be in my thoughts. I wish you well and hope that your treatment plan will benefit you. Let me know how you make out.
?Sending peace and love................
Oldfatguy1 OConnor
Posted
you mention ageoften. I was y5 when mine we up and the bleeding was unbelievae. The knee increased in size by over 4 " in 3 hours. Bleeding back into my groin. The best bet for diagnosis is an orthopedic oncologist at a medical school. As they see more of it than Anyone.. I had a synovectomy plus radiAtion and they stl couldn't get it all. I'm now 79a d still battling it as I know there is a spot on a. Nerve cluster that wll never go away.
OConnor Oldfatguy1
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tinisha1973 OConnor
Posted
Good morning O'Conner,
This is a one club that I never wanted to be in. I am in the United States as well California to be exact. From my research on my doctor he is supposed to be one of the best in my area. You know when I first found out that I had PVNS I wanted to do as much research as possible little did I know that there was not much research out there.
I was lucky enogh to find this forum as well as a FB group, but I tell you sometimes I wish that I had not. lol Some of the experiences that I have read about have been downright scary. To imagine that I may have to deal with this for the rest of my life is a hard thing to get used to. I have yet to read any where there is a situation where one surgury is enough.
Due to so other medical porblems that I have had between 2009-2014 I was having a surgery almost every year. After that horrifying few years I thought my ordeals were over but no. Now I have been blessed with PVNS. Lucky me right.
I am quite anxious about my doctor appt on Thursday but I an eager to get this process started. I will be use to give you an update once I speak with my doctor.
I must say despite everything I am glad that I now have a place to go to voice my frustration with people who understand what I am going through.
I hope that you have a blessed day and will longer forward to speaking with you again.
Oldfatguy1 tinisha1973
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tinisha1973 Oldfatguy1
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I'm in northern California. I would have to look into that because I was just told that my tumor in next to all the nerve endings in my knee and very hard to get to. They are going to try to do the arthroscopic surgery but I am most likely headed for open knee surgery. Not looking forward to that.
tinisha1973 OConnor
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Hello, had consult today. The tumor is in an very difficult location. She said it was really close to my nerve endings. It's in an area that controls the movement of my knee. I have an arthsvopic surgery scheduled to remove what she can but she is not sure if she can get to it. She said I may be looking at complete knee surgery with a tumor removal specialist. I thinking this is just the start of a long painful process.
OConnor tinisha1973
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Best of luck. I will virtually be holding your hand. Keep in touch.