Giant Cell Glioblastoma

I posted a couple of days ago about my wife's recent diagnosis of an astrocytoma.  The initial diagnosis (based on the radiologist's review of the MRI) was a glioblastoma multiforme.  The frozen section taken during the resection was of a stage 3astrocytoma.  We just got the results of the detailed pathology and it gets even stranger.  I guess it is rare enough for a 37 year old woman to get a glioblastoma, but she appears to have a very rare subtype called a "giant cell glioblastoma."

We have a meeting scheduled with the neursurgeon on Wednesday to discuss the next course of treatment.  I understand initially that the process is essentially the same, radiation and chemotherapy.  There appears to be some literature out there pointing to a slightly better prognosis for this subtype than for a "common" glioblastoma.  However, cases of this type are very rare so there isn't much information available.

Anyone out there have experience with this?  Does the rarity potentially mean that she would be excluded from clinical trials focused on regular GBMs... or does the rarity make her a potential patient of interest for researchers?

Thanks again for all of the kind words of support to my last post, by the way.  My wife has an amazingly positive attitude and is ready to take this thing on with everything she has.  Our two boys are 5 years old and 4 months old, and she is focused on being their mom as long as possible.  She is an inspiration to me.

Hi again, and I'm glad to hear that your wife has a positive attitude, because it sounds like that means she is pretty comfortable and stable. Yes? Hope so.

Haven't heard of Giant Cell GBM. What next. My son Andy has a variation which is a GBM and a primitive neuro-ectodermal tumor, all mixed up together. It too is very rare. I know of one other, through this board.

And my understanding is that his unusual cancer type is unfortunately going to disqualify Andy for most clinical trials. They like to create an "apples to apples" study, without some weirdo X factor skewing the results. How else can they know how their trial therapy compares with conventional therapy.

I have heard that there are exceptions made for "Compassion". Everyone with brain cancer deserves compassion, of course, but I can't imagine a person more worthy of an exception for a trial than a young mother. At the same time, you understand she would be entering into a contract, and an experiment. There are no guarantees with any treatment. It's a huge emotional and psychological journey on top of the medical.

Wishing you all the best, all the breaks,

Sarah

mom of Andy 27 dx GBM/PNET 1/09

While I do not have experience with Giant Cell Glioblastoma, I do have some experience with Glioblastoma. My 30 year old son was diagnosed with a Grade IV Glioblastoma Multiforme on March 28th, 2009. His tumor was the size of a softball and was resected on March 31st. The surgery removed 98% of his tumor. Since that time, he has completed radiation therapy and his first round of Chemo (Temador), which lasted 6 weeks. He had an MRI done on July 3rd, which showed that the remaining tumor is lying dormant. On July 16th, he went back on Temador in an attempt to keep the tumor from coming back. The doctor told him that he will need to be on the chemo for 14 months. At this point, we wait and pray. Like you and your wife, my son has 2 small children. When I read your message I felt I had to respond because I know how hard this is for you. If you need someone to talk to who understands, please let me know and I will provide you with my email address. - Diane

Giant cell glioblastoma is a histological variant of glioblastoma characterized by bizarre, multi-nucleated giant cells, an abundant stromal reticulin network, and a high frequency of TP53 (p53) mutations. Giant cell glioblastoma is considered a WHO Grade IV astrocytoma. 

 Epidemiology

Giant cell glioblastoma accounts for about 5% of all GBMs. The mean age of occurrence is 42 years. There is a male predominance (1.6:1).  

Clinical presentation

Giant cell glioblastomas are de novo lesions with symptoms at presentation similar to those of GBM.

Imaging/Macroscopy

Because of their abundant stromal component , these lesions may appear both by imaging and surgery to be well-encapsulated lesions, and are often mistaken for a metastasis.

Histopathology

Giant cell glioblastoma is composed of numerous bizarre, multi-nucleated giant cells and small fusiform syncytial cells within an abundant stromal reticulin network.

Molecular genetics

TP53 mutations are present in nearly 75-90% of these lesions. PTEN mutations are present in a third of these tumors. Giant cell glioblastomas typically lack EGFR amplification/overexpression or LOH of p16.

Prognosis

Mean survival time following surgical intervention appears to be somewhat longer than that for other variants of GBM, possibly because of a less infiltrative behavior.

On 7/18/2009 PacosDad wrote:

I posted a couple of days ago about my wife's recent diagnosis of an astrocytoma.  The initial diagnosis (based on the radiologist's review of the MRI) was a glioblastoma multiforme.  The frozen section taken during the resection was of a stage 3astrocytoma.  We just got the results of the detailed pathology and it gets even stranger.  I guess it is rare enough for a 37 year old woman to get a glioblastoma, but she appears to have a very rare subtype called a "giant cell glioblastoma."

We have a meeting scheduled with the neursurgeon on Wednesday to discuss the next course of treatment.  I understand initially that the process is essentially the same, radiation and chemotherapy.  There appears to be some literature out there pointing to a slightly better prognosis for this subtype than for a "common" glioblastoma.  However, cases of this type are very rare so there isn't much information available.

Anyone out there have experience with this?  Does the rarity potentially mean that she would be excluded from clinical trials focused on regular GBMs... or does the rarity make her a potential patient of interest for researchers?

Thanks again for all of the kind words of support to my last post, by the way.  My wife has an amazingly positive attitude and is ready to take this thing on with everything she has.  Our two boys are 5 years old and 4 months old, and she is focused on being their mom as long as possible.  She is an inspiration to me.

 

Hello,

My finacee has also been diagnosed with Giant Cell GBM 4. He had surgery March 15th 2009..nearly all removed. Followed by 6wks of rad/temadar(140mg).

His regime 5/23 (285mg temadar) has began and he just completed his first round.He is absolutely fine..the temadar did wipe him out!

We are having problems determining. if what is showing up on his MRI Spec...and PET scan is necrosis or regrowth...the last thing we want is another surgery, but not sure of any other options.

Anyway...you are correct about the lack of information found with Giant Cell...and it's very frustrating. I will keep you posted on any information we find regarding Giant Cell. In the mean time stay strong and postive!

Best Wishes

My finacee has also been diagnosed with Giant Cell GBM 4. He had surgery March 15th 2009..nearly all removed. Followed by 6wks of rad/temadar(140mg).

His regime 5/23 (285mg temadar) has began and he just completed his first round.He is absolutely fine..the temadar did wipe him out!

We are having problems determining. if what is showing up on his MRI Spec...and PET scan is necrosis or regrowth...the last thing we want is another surgery, but not sure of any other options.

Anyway...you are correct about the lack of information found with Giant Cell...and it's very frustrating. I will keep you posted on any information we find regarding Giant Cell. In the mean time stay strong and postive!

Best Wishes

Best of luck to you and your fiance and thanks for the offer to keep me updated on his condition. 

I can't get over how helpful boards like this are right now.  While our family has really rallied to help with the kids and care for some of our immediate needs, it does help to know that one isn't alone in dealing with the fear, doubt, and all of the scary new science we never thought we would need to know about.  Though I honestly wish nobody else had to experience this stuff.

Have you attempted to locate any clinical trials?  I've been looking at online sources (e.g. www.cancer.gov) and the trials involving GBMs don't specifically exclude GC-GBMs but one never really knows until the case is reviewed by the trial administrators, I guess.  My wife is eager to try everything and hopes that something good could come out of helping to test some newer treatment options... for her own sake and for future patients. 

In answer to a previous question, my wife is feeling amazingly well right now.  Other than her lovely new "GI Jane"haircut and scar one wouldn't even know she is sick.  Considering the serious risk that she would lose her ability to communicate and perhaps experience some right-side paralysis (tumor was 4 cm and located in her left temporal lobe) she appears to have none of these symptoms following the surgery.  If anything, she is feeling better than she has over the past few months.  The incredible results of the surgery certainly have boosted her morale.

In hindsight, of course, we know understand that she was having some minor speach issues like forgetting certain words, she was more tired than usual, and of course, the headaches that started about two weeks before her diagnosis and progressively got worse.  With the tumor out these symptoms have seemingly disappeared and perhaps the steroids they have her on now are making her more energetic as well.

The doctors estimate that the tumor had been growing for 9 months or more... meaning that she most certainly had it while she was pregnant with our son who is now 4 months old.  I guess this means it is a strange blessing that it wasn't found earlier, not that there was any reason to believe she needed a CT scan before now.  Her minor symptoms were dismissed earlier as normal exhaustion from a late pregnancy and then nursing an infant by us and her doctors. 

I think we're in this temporary lull at this moment, waiting to begin radiation and chemo and not exactly sure what the future entails.  The waiting is the worst part for her as I'm sure most of you can relate.  She wants to know the plan and to start the treatment so she can feel as if she is doing something to fight this.  I'm doing what can by talking to different doctors, exploring clinical options, planning for the care of our kids (the baby hadn't been in daycare before, for example) and trying to concentrate on some of my work which has been a real challenge, to say the least. 

Best of luck to everyone and thanks again for the kind responses.  Keep the information coming.

Hello my name is kristi and my 5 yr old was diaganosed with a  level 4 gbm im a so afraid for her! I could not amangine living life with out her. My heart and prayers go out to you and your family. I was wondering if you have heard any thing postive about people surviving,because all ive heard is negative. Just to let u know my daughter is in her second week of radation and her second week of chemo. She is taking temdor. So far it has not made her sick,though she does seem to be more tired than usal 

Hello my name is kristi and my five yr old was diagnosed with a level 4 gbm 1 mo ago. I am so afraid!!! All ive heard about the survival of gbm is awful. Please tell me have you heard of any long term survivers. I just want to let  you know my heart and prayers go out to you and your family.