PVNS: experience and causes
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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 know your post is older. It is hard to find people with localized PVNS. I have been in a study at Stanford. The thoughts I hear are a trauma may set these things off. There is also interest as to if you had any injections into your knee at anytime. How was your lesion treated. Total removal or was it burned out?
Oldfatguy1 allyfl1003
Posted
Ironically, 14 years ago today is the day mine blew up. To my knowledge I had no trauma. I had hit a bucket of balls at the driving range the night before but I was working on my short game and wasn't really turning hard on the ball.
I had to be put under 3 times and nearly 200 cc of blood removed each time (appx a pop can full). Also had a half dozen needle removals of 65 to 75 cc over 4 months.
Then I had 30 rounds of reduced level radiation, 6 months later a synovectomy with a scope done by an ortho oncologist at the university of Kansas med center. She advised me they couldn't get it all due to location ( it is located in a cluster of nerves). A year later the TKR.
9 years later the tumor had eaten enough of the bone it allowed the stem of The prosthesis to break loose and came the revision. The pvns was found in numerous spots on the bone and prosthesis. Due to a broken femur and staph infection I've had 5 additional surgeries and traces if the rumor have been evident each time.. For me this started @ age 65 and I'm now pushing 80.
allyfl1003 Oldfatguy1
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Oldfatguy1 allyfl1003
Posted
Roach allyfl1003
Posted
Hi there allyfl1003,
I was first diaagnosed with accute diffuse PVNS back in 1988. It took a specially observant & aware Surgeon to suspect & test/confirm it. In my instance, yes there was a truama to my knee three years prior (Somebody knocked me over and fell on the inside of my leg, bending & tearing some medial ligaments. The next year, I began to notice crunching and locking sensations in that knee. The rest is history.
My fight went for 27 years, involved 10 Arthroscopic post/ant synovectomies, a Yttrium treatment, 15 open post/ant synovectomies, and then finally I lost the fight altogether when in 2014 I had my whole joint & lower end of thigh bone replaced with a Distal Femur 'mega-prosthesis'
My Surgeons believe that Yttrium is NOT an option for this disease, as each injection will accellerate the degeneration of articular cartilage & other structures ...which is essentially what happened to me.
Thankfully that has been a raging success, and I am smashing out over 10km walking per day & through massive amounts of work, have regained full muscle tone & balance.
I hope this info is of use in your study. Cheers & regards, Roger. =D
Oldfatguy1 Roach
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Roach Oldfatguy1
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Hey thanks OFG, its sure been a long, painful, expensive & life shaping journey. Its really hard to get people to understand. You're kind of 'on your own' due to the innocent ignorance of friends & family. I've found it's easier for peops to make up their own conclusions & stories than it is for them to get their minds around DPVNS and the reallities for sufferers.
OConnor allyfl1003
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Hi Ally,
?Care to update me on your journey in the study at Standord? Somewhere....maybe not your post....I remember hearing that PVNS may actually belong in the arena of Rheumatology. Could it be that i am wasting my time firing Ortho guys who don't want to understand what I am going through when all along, I should be talking to a Rheumatologist? After all, I see where they do deal with osteoarthritis and the bulk of most of our complaints come from the that condition. I am 500 miles from Stanford, but if they achieve success with PVNS, others will follow. Anything you are willing to share will be appreciated. Thanks.
Oldfatguy1 OConnor
Posted
I guess they are finding out more about the problem as the years go by. I was sent to a rheumatologist early into the quest for diagnosis. The ortho oncologist made the PVNS diagnosis but sent me downstairs to the rheumatologist and in short order she called the ortho and said she returning me as damaged merchandise. That's when I started the radiation.........which I wish I had the synovectomy 1st.
allyfl1003 OConnor
Posted
Hi
I am home recovering from my surgery this week. I got my pathology results back and i have a synovial limoma aborescens. It is actually more rare than the PVNS They did the synovectomy..... i had many discussions in the last week about PVNS My surgeon says he has done hundreds of surgeries for it. I was suprised. He reminded my that Stanford is a place many people get referred to. They do have trials and doctors who are working on methods to treat the diffuse PVNS. The localized can be controlled with a proper removal. Go into their website and search. You will see they have been looking for patients in the past and some of the things they are talking about. I know it is tedious. But, as you well know you do not get to be just a "patient" when you have something thing like that. The main tumor surgeons I know of are Dr Avedian and Dr Mohler. You need a referral. I hear Dr. Avedian is wonderful with his patients. I am not sure under what department the trial doctors are. All my best to you.
allyfl1003
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OConnor allyfl1003
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Thanks , Ally. My morning post did not go through, so here I am again. I am glad that you are over your surgery and now looking forward to good care. As for me, I have reexamined my original MRI and with my layman's knowledge and a good medical dictionary have concluded that I may not have diffused PVNS , but also have synovial osteochrondromatosis in addition to aPVNS tumor. And to think that 2 ortho guys have never explained it to me and that I never demanded an explanation. I was too busy bleeding and swelling . If this is true, then an artho procedure may be the way to go for me. Although my bleeding condition will have to be addressed at the same time.
?Thank you for sharing. I hope your recovery is seamless and you'll be back to being productive in no time at all.
OConnor Oldfatguy1
Posted
Since I have been observing a lot of other little joints freezing up and hurting, like in fingers, I started to get the idea that rheumatoid arthritis could be at fault in me at least in joints other than my bleeding joint. I seem to remember some past blood test indicating a high number in a rheumatoid test. It was passed over because the hematologist was looking for a bleeding condition. (Out of her field, off her radar) Since I have been waved off from all NSAIDS, I have relied on generic low dose Vicodin and low dose Tramadol for my swelling and joint pains. Well, you know that vicodin is a Structure 1 med now and a doctor cannot give you a refill without personally presenting yourself and justifying more meds. They can't even electronically send on the prescription for you to a pharmacy. So i rely for the most part on Tramadol (Structure 2), which is monitored by my internist who knows me best and knows my strong mind and will and bent toward natural refief, not opiods. However, our governor has introduced a bill that will tract all Structure1 and 2 drugs in an attenpt to stop doctor shopping for opiods. A large data base is being created to follow even my little meds' use. This will put pressure on my doctor who is an employee of our big medical center. Internal medicine is not a big user of opiods on a regular basis. That is why I want to look into rheumatology to get permission to use some drug that will not be an NSAID, maybe will be and fits my profile of this bleeding condition or ???? If rheumatologists handle joint destruction from arthritis, then maybe they can also treat my destroyed knee joint that has multiple problems en lieu of actual surgery. Until I can find an ortho guy to do an arthoscopic procedure and assure me that I won't bleed to death, I have to keep searching.
?You've had a ton of experience with this knee problem. Am I off track looking in this direction, at least at this time?
connor_20694 morphix
Posted
Hi, I'm 19 and I've suffered from diffuse pvns in my right for that past two years. I did in fact break my left ankle a few years before in a road traffic accident. When it started the from of my knee quadrupled in size, so I have an op to remove it, to then return to hospital 6 months later to have another 6.5cm tumour from the back of my knee. Unfortunately they were unable to remove it all so I am awaiting another MRI scan to monitor its growth.
OConnor connor_20694
Posted
Oh my goodness, you are far too young to have to deal with this miserable health issue. Your mobility is impacted, you have to deal with pain at a time in life when you want to be most active, and the quadrupled knees are known to all of us with PVNS. I have not yet had the surgery, but as you know, the PVNS can continue to grow. My hope is that you have a really good surgeon who fully understands the disease which is very rare. I believe that we should have an artho/oncologist to go in and excise the tumors because that is all they do as a profession. PVNS used to be regarded as a cancer and chemo was used to try to stop it. But medical people found out that it did not metastasize to other joints so they abandoned that approach. Radiation was tried, but damage was done to the bones. Because the disease is so rare, only 1.8 per 1,000,000 people, there are no funded institutes to study it. Alas, we are stuck to deal with it on our own.
?Pleae continue to tell us how you are doing. What happens to you can help us as well and we will relate what works for us. I am quite old, and am resisting surgery at this time because of a serious genetic bleeding issue. As you know, PVNS can produce bleeding at will and even an hemorrhage as I had one miserable day.
?Sending you a big cyber hug from this old granny. Stay strong, find a lot to do with what mobility you do have and don't let depression rule your day. You are a very special person with a very special disease.
?Keep in touch.
Oldfatguy1 OConnor
Posted
You're right on all accounts. I had no idea until the bleeding started and they had to put me out 3 Times in 2 months to take out a total of 600 c.c's or 3 soda cans full. On top of that, 6 times with a needle taking 65 to 75 cc each time. Probably thecwirst thing was radiation. They were using a reduced amount but it still severely burned the tissue. With all the radiation and surgeries (11 total) I still have a piece of the tumor in an inoperable area, imbeded in a nerve cluster. A little slip and .....boom goes the leg. I was 65 when this was diagnosed. Unfortunately, no one in the practice, 8 ortho surgeons, had ever seen it before. Now 80 I still fight pain but hopefully, the bleeding is under control.