PVNS: experience and causes

Bones, joints and muscles
1

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?

2

I'm actually in the US but there seems to be more cases in the UK. I found an excellent support group on Facebook called "PVNS is Pants".

I was diagnosed with PVNS just last month and have surgery scheduled in 2 weeks for an arthroscopy of my right knee. This will be my first surgery to remove the diffused PVNS or as much as they can. I was told it will come back and was given a 10 year time frame before the next surgery will be needed. However, PVNS is very unpredictable.

Some of us are finding that we share other strange ailments like cysts in our thyroids. Unexplainable sudden swelling or muscle tears is another strange commonality.

There is speculation that PVNS can be triggered by an injury but no one really knows. There is no definite cause. There are people all over the world at many different age ranges from children to the age range of 40 to 50 who are the majority of those diagnosed.

I have had strange knee issues since I was 16 and about 10 years ago I noticed a bump had formed on the lower left side of my right knee. But in the last year I've noticed I was becoming disabled. I turned 40 last month. But there are some who had surgery and within a year needed another surgery. Some last longer.

So far I haven't heard of any medications found to be effective at slowing it down. The only thing that seems to help is radiation treatment, usually by injection and very painful.

There are plenty of PVNS bloggers out there. PVNS is different for everyone. Some even have it in multiple joints!

3

Thanks for the reply! I was beginning to think it's so rare nobody has heard of it, let alone experienced it!

There's several similarities interestingly between our stories... I also just turned 40 when I got diagnosed and after symptoms manifested...

I had the arthroscopy and a biopsy taken from the knee 2 months ago. The tumour biopsy result showed to be benign as expected with PVNS. The procedure actually improved considerably the mobility and use of my leg again.. after the op within a couple of weeks I felt like 3 years had been rolled back and I was even able to go jogging daily.. the stiffness was almost gone, and no burning pain, so I've been able to come off painkillers.

At the post-op arthroscopy consultation the doctor told me that it's common with that procedure for the symptoms to improve but emphasised it would be temporary and eventually surgery would be needed. How temporary he didn't say and I forgot to ask. As you say though, it's probably different for everyone and hard for doctors to give a general answer.. it could be weeks, months or a year or two for some lucky people maybe, depending on how progressive their PVNS and how active they are.

I subsequently contacted the Royal Orthopedic Hospital in Birmingham (a great hospital by the way, one of the best in world and they know this condition well) and basically asked them how long I can expect before I need surgery and the symptoms return. Well I got my answer before the hospital replied. The daily joogging and other activities which involved bending the knee a lot and putting pressure on it, caused swelling and the symptoms have begun to return gradually in the last 2 weeks. The joint is getting stiffer, some burning pain is back...but still nowhere near as bad as before the arthroscopy procedure during which I I believed they drained some fluid.

I was fortunate? to have the localised version of PVNS and not the more difficult to treat and progressive version you have, the diffused type of PVNS. I was told initially from my scans that the treatment would be very straightforward, by arthroscopy, one small hole. They told me I'd be back to normal in 3 weeks and not need any physiotherapy. However, after I had the explorative arthroscopy procedure, they said the PVNS was more progressed then they first thought.. I've been told that open surgery will be required and half my knee joint will be taken away.. I'm looking at 3-6 months of physiotherapy.

Now I'm facing a dilemma. Do I go ahead with the surgery and just get it over with and have a terrible summer.. or I do ease off the physical activities I enjoy so much (exercising etc) and just hope the symptoms don't get worse and ride it out until after summer.. I planned to travel overseas this summer, but obviously that will be no fun with a crutch.

4

I have also had quite an issue with scheduling surgery. I had to wait for the end of the school year for myself and my children. I went back to school because it's better for me not to work which I wasn't anyways because of our lovely economy and knew I needed to take care of the knee because it was slowly getting worse. So now that I'm slow and unable to bend my knee any longer I'm ready to get mobile again so I can work!

If I were you I would eliminate any kind of exercising that has significant impact on the knee. No more running! Especially because it can cause an unnecessary muscle tear. I'm not sure that it caused by localized or diffused but it's not a fun side effect. I had one last summer and it was awful. Also a girl that lives near me with PVNS was told to not try to have kids because she has a job where she stands all day.

I find that riding bicycles, swimming and dancing are the best ways to stay active without the stress on the joint that can instigate growth of the PVNS in the joint. Also yoga seems good for getting back to normal. I heard physical therapy before surgery helps you get back to normal quicker after surgery. So I would say go for surgery after the weather turns. I think planning your life around the disorder is the name of the game.

5

I am 18 years old and last week had open surgery to my knee to remove 3 growths. It took 9 months of seeing 3 GP's and 2 surgeons at my local hospital before they diagnosed it as PVNS. It was only when they sent my second MRI scan to The Royal National Orthopedic Hospital that they could give me an answer.

As I was informed by the surgeons there it is not caused by anything in particular however most forums I have read the patients have been very active and I was a keen dancer, dancing EVERY day before this.

I actually regret the surgery to some degree as I was not told before there was a 50% chance of it returning, and I am now having trouble with my other knee. Before the operation I was not in too much pain and was managing to do most of my day to day tasks (except for dancing) but I now realise that is probably not a career option for me now.

6

Hi Alice, I'm sad to hear that you have this awful condition at such a young age and that it's ruined your dancing.

I'm astonished they didn't tell you about the 50% risk of return... that was brought up early by my consultant even before we had the biospy results..

Then after my biospy they gave me the choice, and basically said, you can have the surgery now, or wait a while until the disease progresses worse, because of the risk of it coming back and needing further surgery some people decide to postpone it if they feel improved after the biospy.. rather than rush into surgery and then have it come back a year or so later then have to go through it all again.

After the biospy I felt much mobility improvement due to the fluid draining from swelling etc and was able to do jogging again, something I love doing daily.. when I couldn't jog for a year I got very unfit and depressed.. so I can understand how you must feel not being able to do your dancing, something you enjoy doing.

Maybe when you're fully recovered from surgery you will be lucky not to have it return and can do your dancing again but be careful not to overdo exercise like I did which could cause injury and trigger a new PVNS episode.

Keep optimistic!

Paul

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PVNS - MY SURGERY EXPERIENCE & ADVICE TO PATIENTS

A quick update for anyone following this thread..

After my biospy in March I decided to postpone surgery.

Reason being, I was told it would be full open surgery instead of the key-hole surgery I had been told initially. Open surgery is a whole different scenario to key-hole. The risk of months of physiotherapy would interfere in my plans for this summer for a holiday/home move. I'm also in the middle of a business degree course and work full time...

Since I had greatly improved mobility afer the biospy, I was able to go jogging again (or so I thought) and lead a fairly normal life.. no pain in the knee hardly, just stiffness.. I wondered how long this would last and hoped it would last the summer at least. Unfortunately the PVNS came back with a vengence this month. I'm pretty sure this was due to the jogging and increased physical activity. If I had avoided that, I would have lasted the summer at least I'm sure before I needed surgery.

I called my consultant and got a quick surgery date within a week at the Birmingham Royal Orthopedic and had my surgery last Thursday. I didn't get out from hospital until last Sunday, even though I was told I would be out the same or next day. That was due to the drain being kept in longer.

OK so here's what to expect...(for anyone undergoing open surgery on the knee for PVNS)

Prepare yourself. Don't expect to be walking out of the hospital with a stick as I rather naively did. Don't expect to be leading a normal life again quickly within days. You need to be realistic. You will have to adapt your life around your recovery and physiotherapy for at least a month, maybe longer.

SURGERY & HOSPITAL STAY

I don't want to scare anyone who is awaiting open surgery, but it's no walk in the park. The pain is terrible. So terrible I was having to take morphine in hospital, even days after the surgery. You can expect to be on dihydrocodeine for weeks upon release. The constipation from these is terrible. I was glad to get out of hospital because of it in the end. No bowel movement for 4 days!! If the hospital staff tell you, you'll be out the same day or next day, take it as an optimistic assessment and prepare yourself for a longer stay.. you can expect to be in 4-5 days probably, so plan accordingly. Remember PVNS is a rare condition and treating it is not straightforward. Each person is different.

PAIN MANAGEMENT

The post-surgery pain is the main problem.. its excruciatingly bad when you're moving, standing, but ok if you're resting lying down. If you keep taking the opiate painkillers you have all the discomfort and unpleasantnesss of constipation.. the laxatives they give take days to work and have only minor effect in my case.

SELF-ADMINSTERING BLOOD CLOT INJECTIONS

Then there's the injections to prevent blood clotting.. these have to be done daily in stomach, either administered yourself, or a relative/friend can do it. They are not bad though. I was terrified to do it and insisted the District Nurse come and do them, but she coaxed me into trying one injection and I honestly was surprised. It was dead easy to do and I didn't feel any pain at all. It's just getting past the initial fear of sticking the needle in yourself..but it's such a slight tiny needle you won't even notice it. So have no fear about the injections if you're on your own. Just try one with the nurse, and you'll see it's nothing. Don't expect the nurse to do these for you for 2 weeks. As I found out, they will not, not matter how much you argue!! The alternative is to go to local practice nurse at drs surgery (trust me, the pain of going anywhere in first week is not worth the hassle..try it yourself at home).

PHYSIOTHERAPY

You will get an assessment from the hospitals physiotherapists a day or so after surgery and they will see how able you are to manage by yourself at home.. if you can get on and off the bed, go to toilet unaided, walk up and down stairs, they will most likely let you do the physiotherapy exercises at home, (as in my case)...

This may sound a better option, but some words of caution here.. its not easy to do these exercises alone and takes A LOT of time and mental discipline. If you live alone, as I do, it is very challenging to find the time and to get into a routine. However you absolutely must do all the exercises, daily, three times a day in fact (and not stand on leg or sit for more than 30-60 mins, nor lie in bed for more than 1-2 hrs without moving your leg).. else you will have problems with the joint and end up having to go into hospital for physiotherapy.

ADAPTING TO LIMITED MOBILITY

After surgery, you can expect massively reduced mobility. Even walking across a room is a major effort on two crutches..carrying meals/drinks/equipment etc, all very difficult if you live alone.. you will definitely need help preparing meals and someone to act as a carer for the first week or two, unless you're lucky enough to have relatives or a partner living with you. If you live alone, you will certainly need to make a lot of adjustments to your living environment. In my case, it's even worse because not only do I live alone, but I'm also in a 2nd floor flat which has 3 flights of stairs!

Funny irony.. before I had my surgery, I had stiffness and pain from PVNS and would walk with a stick but could walk briskly and would pass these old people with zimmer frames and two sticks, crippled up from arthritis moving slowly and think, my god.. how terrible. Now, these same old people are moving quicker than me, and looking at me in sympathy!

DON'T OVER DO IT!

Even just walking a few yards is a huge effort outside..I went to the shops just 2 minute walk away from my flat and was physically exhausted and in a lot of pain by the time I returned.. If you overdo it and don't do exercise gradually (as I did by throwing myself straight into a load of business work, with lots of standing around) you will get massive swelling and that causes further muscle/joint strength loss and increased pain. All you can do then is put ice on it and rest it up elevated.

PLAN YOUR LIFE AROUND RECOVERY

I've been told it will take at least 6 weeks of physiotherapy for my own recovery due to open surgery and having more of my knee lining removed (but even so, I had the lesser localised version, not diffused). If you're fortunate to have the keyhole surgery and very minor joint lining removal, you may get away with much less mobility problems and physiotherapy. I was told by the surgeon that in diffused/worse PVNS, where they remove the entire knee lining, it can take 6-12 months of physiotherapy. So I consider myself lucky it's only 6 weeks..even if it is hell right now in the first week!

I've been told it does get easier as you regain strength, but it's vital you do the exercises and rest. Don't be tempted to rush back into work or lots of moving around, until you have your strength back, else you will just prolong the agony.

So, in summary, here's some key points and advice...

- If you feel much improved after the initial biospy and fluid draining (pre-surgery) don't assume it will last long if you go straight back into using the knee/joint and doing strenuous physical activities. If you need to postpone surgery for any reason and want to retain mobility, my advice is to avoid doing anything strenuous that uses the joint.. even light jogging. Else you risk requiring surgery in a few short weeks, as the PVNS symptoms may return fairly quickly. Make the most of the improvement after the biospy by not exercising the joint strenuously and I think you could maybe postpone surgery (if you need to) for many months or even a year if you're lucky.

- If having open surgery, prepare yourself for major lifestyle change for several weeks/months, and realise you won't be the same for a while or able to do much beyond physiotherapy and recovering.

- Plan ahead, adjust your living space and make sure you have a carer available.. you will need easy access to toilet (bed bottles/pans else), meals cooked by someone or easy to prepare meals... everything you need should be in easy reach of the bed.

- Be prepared for major constipation from daily pain relief. Drink plenty of fluid/water constantly to hydrate.. get plenty of fibre and take laxatives with senna tablets.

- Use your crutches properly at all times to keep weight off the bad leg and don't be tempted to hop around or walk on the bad leg without support. Definitely do not undertake any strenuous physical activity like climbing or lifting things in first week from hospital. The pain relief may give you a false sense you can, but remember the joint is fragile and you will end up with massive swelling, inflammation over the joint and severe pain. Once the painkillers wear off the pain is 10x worse then and they no longer work at normal dosage!!! And the more you take, the more constipation/nausea you get.

- Do all your physiotherapy daily as instructed and don't be tempted to skip them or think you can. You can't. If you don't do it, you will be sorry the next day as you feel stiffness and pain set in. The exercises help the strength return to joint and ensure you retain mobility. It's vital you do them as instructed.

- Don't worry about the anti-blood clot injections. They're nothing to be scared of. Once you try one you will see it's nothing at all, you won't feel a thing most likely as the needle is hair line thin and the pain killers block any sensation.

Hope that helps and hasn't scared anyone! I will post further updates on my progress in the coming weeks...

Paul

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SURGERY DETAILS

Oh for those not bothered by the gory details of the surgery, when I said "drain" I mean a tube which is inserted into the joint to let the excess blood and a saline solution they pump in, flow out post-surgery. You are basically attached to a bottle with a hook which you have to carry around with you on your crutch until you're ready to be released from hospital. You can only be released after they think all the blood and fluid has come out, for me that took 1 and quarter bottles (500-750ml).

Having the drain removed is nothing to worry about. It looks bad, but it's only a small pipe and doesn't hurt when they pull it out. You're left with a small fairly painless wound (small 2mm hole) which quickly closes up under a dressing.

For the main wound: if you're having open surgery on knee, as I had..the wound is a vertical incision measuring about 9-10 inches right over the knee joint on front...it's closed with metal staples which are not too pleasant .You can feel them pulling if you move in the wrong way or strain yourself, so be careful. The staples come out 3 weeks after surgery.

NOTE: Don't be surprised if the surgeon leaves in any tumours you might have on the back of the knee. That's normal procedure with PVNS in open surgery, as the surgery is so traumatic to the joint/body, they prefer to minimise that trauma and amount of surgery to joint... if they opened back AND front, it would be much harder to recover.. so they generally remove the tumours as a separate (later) surgical procedure, once you have had the joint lining part done. It's normally done that way around, because the tumours are benign and don't really affect mobility as much as the lining overgrowth problem. So that's the preferred way... knee lining removal, then tumours later. It sucks having to go through two lots of surgery, but it's the best practical way for recovery.

9

Thanks for your message Paul.

It was 4 weeks yesterday since my operation and I am definately on the mend. Stopped using crutches last Monday however wasnt using them at home since 2 weeks post-op. I also live alone but seemed to manage fine (only using one crutch so i could carry food/drink). Physiotherapist told me to put as much / as little weight as I could as there was no way of damaging my knee.

I was also VERY naive about the hospital, they also told me before surgery that I may be going home that day / the next morning so hadnt prepared for the 4 day stay. I even told my Dad before surgery I would get the train home! Painkiller wise I did not take to any very well so took morphine / codine for 2 days in hospital after that just ibruprofen and paracetamol so that i was actually able to get out of bed. Haven't had to take any for a week now as rarely in pain mostly stiffness.

With regards to the injections for blood clots i had them done every day in hospital but I found them extremely painful and they left me black and blue due to the fact i don't really have any fat on my stomache. When i left the hospital they forgot to give me any injections to take home so was advised by the doctor to carry on wearing the surgical stockings instead.

I have found my recovery surprisingly good and there has rarely been times when I havent been able to get out and do things. The first week was difficult to adjust to showering / sleeping / and having to move that leg with the other foot! But since then have been out and about as I usually would and working and am now driving too. Last week my quad muscle came back to life for the first time, very achey doing exercises with it as it lost ALL strength.

As for the scar, 2 days after staples came out I was back at the doctors because the wound has re opened (only on the surface) so was back bandaged up. The scar is rarely painful except for when the nerves ping and they sometimes make me jump! Twice a day I use E45 and give the whole knee a massage and my scar is coming along really well, another couple of weeks until I can start using bio oil though. Still have a lot of swelling so may need to have it drained in the next few weeks will find out Thursday.

Hope some of this helps you Paul - you will be surprised how quickly the pain eases each day I was seeing improvement and regaining the strength to walk. And as for biopsy I never had one, I didn't even know what PVNS stood for until after the operation and in a way was better not knowing I wish I never googled it! So many sad stories and hardly any success stories. But definately staying positive for the time being. Wish you a speedy recovery.

10

I have just been diagnosed with this after a year and ten months of hearing "nothing wrong" from my doctors.

The first MRI was three months after I started have discomfort and swelling, and MRI showed swelling but no tears or other injuries. After three more months they did an x-ray and drained my knee with no luck either draining or with a diagnosis of any problems other than the swelling. So they gave me a cortisone shot, no help. I went home in more pain then I've ever experienced, and my knee was about the size of a basketball.

I was afraid to go back after that experience and was so frustrated with the lack of any diagnosis, I gave up on the doctors and started doing therapy exercises in water and reduced my activities substantially . This went no where so I went back to the doctor 1 year after symptoms started, requesting that we figure it out, because I couldn't continue to not do anything and there was definitely something wrong. They drained again and tested-nothing, cortisone shot-nothing, Ultrasound-nothing, blood work-nothing, so the sent me to a rheumatoid arthritis specialist .

The specialist gave the diagnosis of PVNS mostly by readying all of the tests, and now they have scheduled my surgery without seeing the second MRI results. They are also saying that it is a simple procedure and I will be out the same morning. After reading the above, I'm seriously scared but at the same time I can not move on this knee and it is starting to effect my hip and strength in my leg.

11

I think that is how we all feel when we are first diagnosed with PVNS and headed for our first surgery. There is a support group on facebook called PVNS is Pants that is very helpful with answering questions and getting all around support since it's difficult to find people who can relate and give the support you need both physically and emotionally. Hang in there.

12

I have been very active through out my life; playing sports; riding bicycle; and alot of walking.My jobs is physically demanding. I have never had a knee injury; but I would have the

cracking; popping, it was giving out and a sudden pinch (like a pinched nerve in your neck); both of my knees ache often. A few years ago it seemed like the constant ache was only in the winter time and my mobility has lessened over the past 4-5 years. If I stop moving it is very difficult to get the legs to move again. I had that same popping and pinch one day going up the stairs; the next two days my knee was the size of a grapefruit; by the 4th day I could not straighten or bend my knee. I went for x-rays and than to an orthopedic doctor. The X-rays showed very little Arthritis; but there was a cell mass on the left side of my knee cap; the MRI showed hemosiderin (iron) build-up inside the joint but the doctors say that when I bent my knee the mass would interfere with the tracking of my kneecap. june 23rd they cut along the outside of my knee to remove the mass for biopsy. It came back as diffused PVNS. The orthopedic surgeon did not do anything else to my knee other than remove the mass because they were not sure what it was. It has been about a month and I can not squat or kneel; which my job will require me to do, my knee gets hot; burns; gives out; snaps and catches still. Nothing has changed since before the mass removal. The mass

was not the reason I went; it was just an accidental find. I go back to the surgeon on Tuesday the 20th, I don't know if I should have a

synovectomy (removal of the synovial membrane) or just leave the knee with minimal

swelling,pain and hope that it doesnt progress in a short time period.

What are the chances of this progressing quickly and should I have the synovectomy? The surgeons had said that with the draining of the synovial fluid, the joint expands and fills

the whole cavity with fluid and eventually the arthritis would be severe enough that I would need a knee replacement. Would a synovectomy prolong that process? I have to make a

decision and I am not sure what the right decision is.

13

Hi Katie, I'm sorry to hear this and about your diagnosis.

To respond to your points and questions:

>synovectomy (removal of the synovial membrane) or just leave the knee with minimal

The normal treatment of advanced PVNS is synovectomy by open surgery, to remove all the affected joint tissue affected, and for diffused, possibly radiotherapy to kill any remaining affected cells to reduce the risk of it returning.

PVNS as you probably know has a high risk of returning (especially diffused PVNS)..about 50%. If any

affected cells remain, there's a chance the entire synovial membrane will be affected again in time.

>swelling,pain and hope that it doesnt progress in a short time period.

When I had my biopsy done, they removed a lot of fluid and some tissue sample and that stopped the pain. I got a lot of movement back and was even able to go jogging again. But doing that, and intensive physical movement, seem to aggravate the condition. Pretty soon (within 2 months) I was back to where I started, with burning pain, worse swelling and limited joint movement.

>What are the chances of this progressing quickly and should I have the synovectomy? The surgeons

>had said that with the draining of the synovial fluid, the joint expands and fills

That's the $1000 question Katie, and we just don't know enough about this disease really to give a definitive answer about how quickly if will return, if at all. I'm not a doctor, so this is going by just my own research and reading of many sufferers experiences around the world, if I'm brutally honest, there is a high risk of it coming back fairly quickly after surgery, if you're unlucky to be in that 50%. I've read many reports of patients who had it return within a year or so, and required repeated surgeries. It's a daunting thought.

>the whole cavity with fluid and eventually the arthritis would be severe enough that I would need a knee r>eplacement. Would a synovectomy prolong that process? I have to make a

>

>decision and I am not sure what the right decision is.

I faced the same decision you are, in those 2 months after my biopsy. The surgeon advised me it was not uncommon for the symptoms to ease and the disease to slow down after that procedure, and that I might want to delay surgery for as long as possible, or just get it over with right away. I decided to delay, because I wasn't feeling any discomfort and had mobility back..but within 2 months as I said, it was back, worse than before..so I had no choice but to go ahead with surgery.

Your age, must surely come into this decision too, as you mentioned a complete knee joint replacement. My understanding is, surgeons don't normally do that unless you're in your 50's or older..because they only last 20 years or less, and need replacing and there can be complications/risks involved with repeated replacements. In your case though, maybe it's the best option even if you are younger.

I think you need to discuss it with your consultant more fully and decide what's best for you and your

circumstances. If you're in constant pain and have reduced mobility still, I don't think you can really manage like that for years on heavy duty painkillers and suffering adjustments/major limitations on your lifestyle. So perhaps you should have knee replacement if its offered, or else go ahead with the synovectomy and radiotherapy and hope for the best.

It's taken me almost 2 months to recover from my surgery for localised PVNS in the left knee. I'm now walking without any support, pain-free, but I can't run and the knee mobility is not 100% and is still weak. I still have tumors from the PVNS at the back of the knee, but the surgeon said he wouldn't remove those until later.

14

Hi all, and fellow PVNS sufferers!

A quick update on my recovery progress for this interested and following this thread about my localised-PVNS of the knee.

I've just got back today from my 6 week (actually turned out to be 2 months!) follow-up consultation at the Birmingham Royal Orthopedic Hospital. They were very pleased with the joint and results of 6 weeks of physiotherapy. I'm now completely off painkillers as I no longer feel any pain. I'm able to walk without a stick and even have begun cycling again (at the recommendation of my physiotherapist) to further strengthen the knee muscles. The only thing I can't do still is jogging. My knee just isn't strong enough yet and make take many months I'm told.

Some interesting findings and discussion took place a the follow-up consultation today which interest some..

I asked when the surgeons would be looking to remove the tumour(s) I still have at the back of my affected knee. I was told, they probably would not be doing it! When I asked if that posed any risk of leaving them, in terms of them growing larger, affecting the joint bone, or the affected cells spreading to the knee lining again, their reply was this:

With tumours in localised PVNS, it's generally accepted that unless they're causing the patient pain, or mobility problems, they're best left alone. The risk of the cells spreading into the lining and sparking a new cycle of PVNS is GREATER if surgery if performed on the removed the tumours, and it's just a risk that surgeons prefer not to take, so long as the patient remains stable and the tumours don't increase in size etc. Instead, the approach is regular check-ups every 4-6 months, to monitor the condition and access the need for treatment. It would be at least a year after synovectomy surgery, before removing tumours would even be considered, if it became necessary. This I found rather interesting!

The second question I put to them was radiotherapy. I asked would I be needing it, and would it lessen the risk of PVNS returning, particularly in view of the tumours being left for a year or longer.

The answer was surprising again. Radiotherapy is not recommended for PVNS which can be treated with surgery and is usually only a last resort option after surgery options have been exhausted or proved ineffective. They explained that radiotherapy carries its own risks, particularly of tissue scarring. which can result in thick tight skin over the treated areas, and sometimes nerve damage that sometimes never returns to normal.

Speaking of nerve damage, I mentioned that half my knee was still totally numb with no sensation or feeling and I asked whether this would correct itself in time. The answer was "maybe, sometimes it does, but often it doesn't". The reason there is numbness down the left-side of the knee is because that is where a large nerve normally resides which was removed during the synovectomy surgery. I was told I might get partial nerve sensation returning, but that my knee on that side might not feel normal again and may have a "rubber-like" feel when pressed, but that lighter brushing or stimulation of the skin would be felt more normally.

Finally, I assured that the outlook was good with my localised PVNS, and that the doctors were confident it wouldn't return, but would need to monitor me regularly from now on, to check for any signs of recurrence. If I experienced any pain returning, joint mobility issues etc, I was advised to contact the hospital.

My next consultation review is set for 3 months time, just before Christmas.

As far as the physiotherapy goes, I must confess, I have been a bit lazy and haven't been doing it the exercises very much (which is very naughty and silly I know, but I am fairly active in other ways which compensates somewhat).

My physiotherapist has trusted me to do the exercises at home and has put together a special routine of progressively more challenging movement exercises designed to strengthen the knee joint and improve the range of movement. The latest exercise involved supporting myself between two chairs, while keeping my good leg out straight behind me, and bending my bad leg down as close to touching the floor as possible! This is still a very difficult exercise, but I've been told to keep pushing it further each day.

Hope some of this is interesting or perhaps even useful to anyone undergoing surgery or facing surgery for PVNS. It's not too bad, although difficult for the first few weeks, recovery can be fairly quick and strength and mobility quite soon within 6 weeks.

15

Sorry for some of my poor grammer and missing words... I'm a bit tired after a long day and only having 3 hours sleep last night due to stressing over the hospital visit today. Time for an early night and then a fresh start tomorrow with the physiotherapy and cycling!

16

Thank you to everyone for sharing your stories even if some are a little scary to get through.

I have been diagnosed with a PVNS deep in the middle of my knee, between the ACL and PCL ligaments.

Prior to a simple arthroscopic meniscus trim in 2006 I had significant pain in my knee and some swelling. I had an MRI which indicated a small mass the size of the top of my 4th finger. This did not garner any comment from any of the doctors and being ignorant or naïve I did not ask to see the report.

My Ortho, because of insurance requirements, delayed the meniscus trim for 6 weeks. So I continued with the pain and lessor swelling. After the surgery my Dr. said all the pain and swelling would be gone, and come back to see him in 6 weeks. 6 weeks later I had the same pain but the swelling had gone. Since then I have had varying bouts of pain and swelling until my PCP requested an MRI in 2010. Interestingly they identified a small mass approximately the size of the top of my 4th finger. This time I was directed to go see a specialist and they identified the lump as PVNS. Since it had not changed in size significantly they assume it is benign and it is not causing the pain I have had in my leg from groin to ankle It varies day to day. Some weeks can pass and it will be mild. Other times it handicaps my activity and the pain is moderate or above.

Two things that stand out for me. 1. this was not influencing my life until I had the meniscus trimmed. I clearly had this in my knee prior to the meniscus issue. 2. When this was discovered I was 58Yrs old--surprising because of all that I have read.

Lastly I want to thank all of you for describing your condition. I will act a lot sooner than later now that I have a clearer patient point of view!!!

Last but not least for anyone who has "tests" of any kind done make it a purpose to ask for copies of all reports!! It is the only way to help yourself manage your own health!!!!

17

wp4934 thanks sharing your story and experience of PVNS..it's an interesting case yours is. PVNS has that 50/50 chance of returning post-surgery and also it's not always easy for surgeons to get it all out in some cases, so I'm wondering if yours has either returned, or the surgeon just missed some affected areas, or some affected areas have proliferated into a mass again. From what I've read online, unless they get every affected cell (it's a bit like cancer) there's a high probability (50/50) that the disease will return at some stage, and usually (from the may cases I've read) it comes back fairly soon if it's going to come back.. within a year or two. If it does return, it's just a question of how long before you notice pain and limited movement I guess..

Now I've had my surgery, my surgeon wants to monitor me regularly for a while (every 6 months) to see if the disease returns..

After you had your surgery in 2006, did the surgeon monitor you, or send you for regular MRI scans to keep an eye on the condition?

Good advice on asking for copies of reports. I wish I had done that in hindsight.

Another good piece of advice is to do your research so you're able to ask the right questions when you have the opportunity with the consultant and/or senior nurse post-surgery. There's a lot of info they don't volunteer or mention, which unless you ask, you're just left in the dark about.. like in my case, whether I'd have further surgery to remove the tumours, or whether I'd need radiotherapy on them etc. It was never mentioned until I brought those subjects up.

18

Has anyone heard a definition or read a distinction between localized and diffuse PVNS tumors? I assume just by the names of the 2 versions of this that diffuse means these tumors occur multiple times in different place, and localized means they are singular, in one place.

Thanks for your help

19

wp4934 I discussed the differences with my consultant from a treatment point of view, after i researched it myself and understood the differences from treatment/prognosis prospective... I was diagnosed with localised PVNS and initially told it would be relatively straightforward to treat, with arthroscopic surgery. It turned out, that I needed open-surgery and synovectomy. Whether that was to minimise the risk of it coming back, or because the area was more wider affected than first thought, I don't know. But the surgeon and consultants changed their treatment after the biopsy results at the last minute.

There seems to be some confusion online about which is the rarer form of PVNS and how rare it is, I've seen conflicting data which may be due to the sources/different countries and lack of a universal understanding of the disease still. I've read that localised is more rarer affecting about 1 in 9 million people, but also seen reports suggest the diffused form is rarer (perhaps they mean where it affects more than one joint).

The key difference from what I've read and my understanding is, that the diffused version has the potential to not only affect an entire joint site, but also can be more prolific and can spread throughout the body to other joint sites in some cases. It's therefore more difficult to treat and has a higher likelihood of returning than the localised version which is often (but not always) limited to one specific small area of a joint, and so easier to treat with less invasive surgery.

You can have multiple tumours and multiple affects (in the same joint) and still have a localised PVNS diagnosis, even though it appears you have two or more different affected sites (as in my case, front/side and back of the knee joint).

I'm no doctor and only going by what I've read and discussed with my consultant to surmise, but think the actual diagnosis of localised v diffused is a clinical diagnosis based on the extent of the cells affected in the joint.. if it's uniformly affected right across the joint area, then it's diffused, whereas if some areas appear normal and it's one or more localised/contained sites are affected, then it's localised and hasn't spread to the entire joint. I've read that in localised PVNS its more common to see the tumour-like growths (my consultant said they're not actually tumours like cancer, but rather more like cysts and harmless, unless they increase in size and affect the bone), whereas in diffused you may not see any tumours but may have more swelling over the joint area, perhaps more periods of pain and limited movement since the entire joint is affected.

One thing I do know, as you pointed out..a common theme in both diffussed and localised PVNS, is that the symptoms in terms of pain and limited mobility can come and go from day to day or week to week.. you may feel ok and other days you may really be suffering. That's why it's often misdiagnosed as arthritis which is similar, symptoms wise. The tell tale sign with PVNS is any joint swelling or tumours protruding..even tumours at the back of the joint can be mistaken for Baker's Cyst (as in my case). All these misdiagnosis can delay treatment and cause the disease to progress further.. I think X-Ray and/or MRI scan should be standard early procedure where a person presents with any arthritis-PVNS like symptoms, particularly after any trauma or injury to the joint which again seems to be a common theme in the development of PVNS in patients.

So little is fully understood about this disease due to lack of clinical research and clinical trials (probably due to so few people affected by it worldwide) that still many questions remain unanswered.. it was though to be a type of cancer originally until around the 1950's I believe. It does have many characteristics of a cancer and in some ways is more prolific and difficult to treat than some cancers I think (at least the diffused type).

20

There was a clinical study and report carried out in UK in 1970's with PVNS patients in UK I think (I don't have a link for it handy, but I'm sure it won't be difficult to find on Google).

The hospital where I was treated (Birmingham Royal Orthopedic) treat about only a dozen cases of PVNS every year the surgeon said..and of those, the majority are patients from outside the UK on a private-patient basis. Royal Orthopedic is considered one of the UK and world's leading orthopedic hospitals, so that's give some idea how rare this disease is.