ANA Discussion Forum
General Category => Inquiries => Topic started by: blairs on July 05, 2011, 05:44:11 pm
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Hi,
I have recently had two schwannomas removed from my left hand, one was much older than the other. In addition, in the last year I have also developed a strange low whooshing sound in my right ear. Are these two things likely to be related? The Drs. don't seem to know much about Schwannomas and no one has asked about hearing issues etc.
Your thoughts and experiences would be greatly appreciated.
Many thanks!
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Hi blairs and welcome.
Interesting topic and certainly piqued my interest. I have had many benign "lumpy bumpies" that I have had removed over the years and then my AN was discovered......
Schwannomas are nerve sheath, (typically) benign tumors........ at the recent ANA Symposium in Cincinnati, Dr. Elizabeth Claus did a workshop re: the genetics of AN's and some research that will be done is the very near future. Will be interesting to see that outcome. I don't have time right this minute to do a "Google" to see what medical articles may be out there re: relationships/genetics between schwannomas but if anyone else has the minute, I'd certainly be interested as well.
Again, welcome.
Phyl
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Hi Blairs,
Welcome to our special and wonderful group!
To answer your question: YES, schwannomas anywhere on the body and ANs can be related. There are 4 genetic mechanisms possible here: NF-1, NF-2, schwannomatosis and somatic-mosaic. The word "genetic" here means "genetically controlled" which in turn could be either inherited from one or both of your parents or a spontaneous genetic mutation.
NF-1 is characterized by presence of subcutaneous scwannomas (like the ones you had), increased chance of some other conditions and relatively few unilateral AN's.
NF-2 is characterized by bilateral ANs (appearing either simultaneously or within a few year of each other), other CNS scwannomas and cancers and relatively few subcutaneous tumors.
Somatic-mosaic means that the person does not have a fully blown condition and genetic defect is undetectable in the blood but it is present in some cells in the body. It is impossible to predict which ones and there is still an increased chance of tumor formation. Generally (but not always) somatic-mosaics have fewer problems than the ones with a full blown disorder.
Schwannomatosis is a poorly understood disorder where there are subcutaneous schwannomas that usually present with pain that can be significant. Patients are unlikely to develop CNS involvement but because of constant pain, there is a lot of morbidity.
All of these conditions are genetically separate and that means that it is possible theoretically to have more than one. In reality, those who do indeed have more than one are very, very rare. I am not going to go into genes and chromosomes here in order to avoid making this post extra long. You can just google each one, there is plenty of information.
I would strongly recommend going to a local Neurofibromatosis (NF) clinic that exists at most big hospitals. They know all about schwannomas and you will not encounter lack of knowledge or indifference. They can suggest genetic tests to see if you have anything as well as put you on a surveillance schedule. It is true that nobody can predict if you will form a tumor or how fast it will grow but if you learned anything at this forum, it is that with small tumors one has a choice of treatments and doctors, better outcomes and faster recoveries. None of it is present with huge tumors. Progress in AN removal has been made primarily because the tumors are discovered at a much earlier and smaller stage.
Finally, I would like to mention that your age and gender are of paramount importance. Generally, the tumors form faster and grow more in young women of childbearing age. Tumors grow slower in men over 55.
Best of luck to you and keep us posted!
Eve
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I had my an removed in November and when I had my follow-up MRI in March, another tumor showed up in the salivary glands. It was a Parotid Gland Tumor. I had it removed in May and the pathology said it was the same schwannoma cell as the acoustic neuroma. So I have to go for genetic testing in August. It sort of makes you nervous because I did not have any symptoms. I was lucky it was spotted when checking the an.
Sandy
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I too am a member (unfortunately) of the multiple-schwannomas club. In addition to my AN, I have a small hypoglossal schwannoma. When I asked Dr. Chang why I got a second, even more-rare brain tumor (1 in 10 million people get a hypoglossal tumor), his answer was something to the effect of (paraphrasing heavily), "Some people's brains just like to grow these things, for reasons unknown."
Like Sandy, it makes me nervous when lightning strikes twice. It's not supposed to do that! In the back of my mind, I worry that there is a 3rd tumor in my future. But Dr. Chang thought it was unlikely. Ummm... how unlikely? Like, uh, a 1 in 10 million chance?
Best,
TW
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I am probably off topic here, but one of the doctors I saw during my consults was asking me if I had any "cafe-au-lait" spots on my skin. Naturally I was curious about his question, especially because my son has some of these spots, and I did a bit of research on the internet afterwards, where I read that these spots may be an indication of genetic predisposition (I think NF1). Has anyone heard anything about this?
Marianna
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I read once that over 6 spots count. I have 2 and one daughter has 1 and I see my youngest grandaughter has 1. I know people who have had some and no AN or NF2. I am just hoping no one else of my family has NF2. It is not the mom of the granddaughter who has it.
I don't remember if my surgeon ever asked if I had any. I need to look this up again.
Cheryl R
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My neuro also asked me ablout the cafe au lait spots- I have probably two million freckles on my body.....
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...one of the doctors I saw during my consults was asking me if I had any "cafe-au-lait" spots on my skin... I read that these spots may be an indication of genetic predisposition (I think NF1). Has anyone heard anything about this?
Marianna
According to Wikipedia:
Café au lait spots can arise from diverse and unrelated causes:
* Having six or more café au lait spots greater than 5 mm in diameter before puberty, or greater than 15 mm in diameter after puberty, is a diagnostic feature of neurofibromatosis type I, but other features are required to diagnose NF-1.
* Familial multiple café au lait spots have been observed without NF-1 diagnosis.
* They can be caused by vitiligo in the rare McCune-Albright syndrome.
* Legius syndrome
* Tuberous sclerosis
* Fanconi anemia, a rare genetic disease.
* Idiopathic
* Ataxia telangiectasia
* Basal cell nevus syndrome
* Benign congenital skin lesion
* Bloom syndrome
* Chediak Higashi syndrome
* Congenital naevus
* Gaucher disease
* Hunter syndrome
* Maffucci syndrome
* Multiple mucosal neuroma syndrome
* Russell-Silver syndrome
* Watson syndrome
* Wiskott-Aldrich syndrome
Best,
TW