i have paraganglioma, what does that really mean?

There's more than you probably want to know on

http://en.wikipedia.org/wiki/Paraganglioma

Most medical terms are descriptive and the name tells you that they develop around the glomus cells, which normally detect the levels of oxygen in blood vessels. The "oma" ending tells you its a cancer.

John

Hi Helen -  I, too, have paragangliomas, so does my son and my sister.   My sisters was in her neck, my son's was in his abdomen, next to his kidney, and my (first) was in my adrenal gland.

I was diagnosed 2.5 years ago.  Have loads of info that could help you, I was pushed from pillar to post at first, as doctors realised they were out of their depth.  But now I'm under St Bart's in London,

Ours is a genetic cancer, caused by a variation in one of our genes, some paragangliomas are hereditary, and some aren't.  You should have genetic testing to find out if yours is or not.

My sister's and my son's were benign,  but mine isn't, so I have secondary tumours in my liver, pelvis and lymph nodes. 

What hospital are you under?  And what tests have you had?   Please send me a private message if you want - but this is such a rare condition I'm amazed to find someone else on this site that has it.

Look forward to your reply,  Jeanie x  

Hi Jeanie

It is good to find someone else who has this as all the information i have been finding is as if it comes straight out of medical textbooks and i feel like a need a degree to read it.

i am under a consultant at castle hill hospital in cottingham,hull.  i was originally with scunthrope due to where i live but they seemed to transfer me accross pretty soon after some of my first tests.

heres my story in a nutshell - i have always had stomach problems which we put down to IBS initially. i then started having pains in my abdomen, so was sent for an ultrasound which is where they discovered this lump (they did think there was 2 to start with).  i then had a ct scan and an mri.  they then did a laperoscopy to try and locate the lump to take biopsy.  they looked in the wrong place!! and couldn't find it.

so i had to have another mri to try and work out where it was.  it was behind the mesentery layer which is sort of the back of the intestine bhind a membrane.  so then i had a laperotomy to remove it.   they think they managed to remove it all.

i had to wait quite a while for my results as they had always thoguht they were looknig at lymphoma. i then had to be transfered to another consultant as the one i had had never seen a case of paraganglioma.

so i am now waiting (and chasing) for a date for another type of scan to seen if  they did manage to remove it all and to check for any anywhere else in my body.  i had to do a 24hr urine collection and bloods.

i suppose luckily i haven't acutally felt ill, the worst i have felt in this recovery from surgery. but am bit tired of all the waiting.  the first ultrasound was january. and i got the diagnosis on 2nd august.

thy haven't mentioned to me about it being a genetic cancer,  will have to ask about that especially if it is something that i may pass on if i had children (i don't yet).

thanks for your reply jeanie x its really good to find someone to talk to about it

love helen xx

I too have just found out i have this rare cancer called paraganglioma they can be benign or malignant . My oncologist didn't even call it a proper cancer but said that it acted like one. They can spread throughout the body and spread anywhere from the head down to the pelvis area but are usually slow growing. Ive got them in my neck ,shoulder, liver and bones. Ive had a barage of tests so far MRI, CT scan and a MiBG scan (radioactive scan) going for some radiotherapy at the end of this month with the aim to either stop the tumors growing or shrinking them.

it is comforting to know ther are others out there who also have paraganglioma.  when they tell you how rare it is it made me feel a bit alone.

i am still waiting for a new form of scan to see if they managed to remove all of my lump hopefully will get the call tomorrow as been waiting nearly monthand half since i saw my consultant.

x

Hi Helen I know its all a bit daunting but I hope all is well with your scans. I think the waiting is the hardest part and you sometimes feel that life is on hold . You are not alone in all this and there are people out there for you. Hope it is good news for you.

Hi Notent

Thanks for your words.  I hope everything goes well for you also. xx

Hi Helen and Notent - Just wanted to give you a bit of my medical history - my sister had a tumour in her neck approx 14 years ago, it was removed and we were told it was benign.  They didn't know then that there could be a hereditary link - so when I started to get pain in my back, around my kidney area, didn't dream it was connected.  After 3 years of doctors fobbing me off, (even did an ultrasound on my left kidney and said it was clear - if they'd gone just one cm above the kidney they would have seen tumour in my adrenal gland) I eventually asked to be referred, I was getting pain in my pelvic area by now, and after having a CT scan, 2 huge tumours showed up.  Mine were malignant, one in adrenal, and a secondary tumour on pelvic bone.  Also small tumours in my liver.   After being told this could be hereditary, I mentioned my sister - they looked at her medical notes, and it was obvious it was linked.  After genetic tests, we found out our dad carried the mutant gene.  My son also carries the gene.  

Usual tests are CT scans first, then possibly a PET scan to see if there's anything else lurking inside, then possibly surgery to remove tumours, or MIBG or Octreotide to try and shrink them.  These are forms of radioactive radiotherapy.  But my tumours didn't absorb them, so it wasn't a suitable treatment option for me.   I've had major surgery to remove the large tumours, and a course of chemo to shrink the remaining ones.   The thing to remember is that they are slow-growing, so try not to panic (I did!)

They can also produce lots of side-effects, especially high blood pressure, anxiety, palpitations, dizzy spells to name a few, but luckily I've never had them, my tumours only produce dopamine, which is quite unusual.

There are a couple of excellent websites you can go on - one is Dr Pheo's blog - which gives you loads of info about pheos/paragangliomas,  and the other is a US based support group called - pheochromocytomasupportboard@yuku -  very very helpful.   Notent, what hospital are you under?

I hope the tests go well for both of you,  please let me know how you are getting on?

Anything I can help with don't hesitate to message me,  Jeanie x

Hi Jeanie

Im under Weston Park Hospital in Sheffield at the moment im going for Mibg treatment at the end of this month as my tumors have a good uptake , this will be my first treatment so far ,they haven't mentioned chemo? .I have also got to be tested to see if I am carrying the gene mutation and have got two sons aged 4 and 6 so I am really worried about them at the moment. I dont really suffer with high blood pressure or palpatations but I am very anxious , mood swings and dizzy spells ,fatigue .I've been reading your older posts and hope you and your Son are doing ok and are getting on with life  .Ive been off work now for over 3 months and hoping to get back to some kind of normality after the treatment. Take care x

Hi Notent - Yes, we're ok at the moment, thanks.  My son has a MRI scan every year now to check for re-growth, and also any others popping up, and I have a CT scan in Oct to check how much my tumours have grown.    My Prof and his team at St Bart's are fantastic - my first Endocrinologist at another London hospital wasn't as good, so I switched.

I had chemo last year (temozolomide) which is usually given to brain tumour patients - but it had been discovered that it could be effective against phaeo/paragangliomas in about 33% of sufferers.   They decided to try it on me as I had no uptake at all with MIBG, and limited uptake with Octreotide.  Luckily it shrunk my tumours in the liver and lymph nodes, so when they grow to a certain size again, I think they'll give me another course.

I am in contact with another person who has paragangliomas, and he was given MIBG treatment which dramatically reduced all his tumours - I think if you havve uptake it's certainly the best treatment to have.

I started to suffer with anxiety about 5 years ago - thought it was just the lead up to menopause!

But don't really have any other symptoms.  I'm sure you'll get back to work soon, and yes, push for those genetic tests, as it's a long, slow process.   Let me know how you're getting on?

Jeanie x