Ray - Chondrosarcoma of the Skull (Cranial Base)

Chondrosarcoma of the Skull (Cranial Base)

Ray Kujala

Published here with permission
of Beth and Ray

Initial symptoms
My husband’s ongoing cancer battle began in October 2002, when he was 47 years old. He made an appointment with his primary physician because he was unable to breathe through his right nostril. When he had no improvement after two courses of antibiotics, his doctor didn’t waste any time -- he ordered a CT scan and referred Ray to a local Ear, Nose, and Throat specialist. When we met with this doctor to go over the results of the CT scan, the news was bad: there was a large mass in his sinus area.

That weekend was our first introduction to the fear and uncertainty that a cancer diagnosis sets in motion. We met the following week with an ENT surgeon at the Cleveland Clinic, and he scheduled a biopsy. We were so hoping that whatever was in there, this mysterious mass, would turn out to be something benign.

Meeting with the surgeon to discuss the pathology results, again we knew the news was not good. He wrote it down for us and handed me the paper, since it was such an unfamiliar word: CHONDROSARCOMA. He had seen only three cases in his career. A bony cartilage-based tumor, in my husband’s case it arose from the skull base and involved the sphenoid and ethmoid sinuses and the right orbit.

Treatment options
The surgeon estimated that he could remove 90% of the tumor, by going through the palate and above the gum line and possibly through the side of the nose. We met next with an oncologist who oversees chemotherapy treatments, and he told us that with chondrosarcoma, chemo “does not usually have a role to play.” Then we met with the Clinic’s radiation oncologist, who explained the options available locally, such as IMRT (Intensity-Modulated Radiation Therapy). But, he told us, in conventional radiation treatment for chondrosarcoma you have only one chance to get it exactly right, and even if you do, there is a fifty percent chance that it will come back (“roaring back,” is the phrase he used). He had heard though, that a Boston hospital was offering something called proton beam radiation, and he thought that it might be used on skull-base tumors. He made a call and came back to tell us that Ray would be a candidate for this type of therapy, which had shown significant benefits over conventional radiation. So it doesn’t happen often, but the Cleveland Clinic made a referral to another institution, and we were grateful for Dr. Crownover’s awareness of new technology.

We spoke with Dr. Norbert Liebsch of Massachusetts General soon after. He requested a very detailed history and an MRI. After meeting with his tumor board, they determined that proton beam therapy would be appropriate, after first de-bulking as much of the tumor as practical. The idea was simply to clear Ray’s airways, as radiation would not shrink the tumor appreciably. (Proton beam therapy renders the tumor inactive, like scar tissue, but does not really lessen the size.)

Dr. Liebsch mentioned a few surgeons with experience in resecting tumors of the same type and location, including one in Philadelphia, one in New York City, and another across the street at Massachusetts Eye and Ear. We knew that it was important for the surgeon to work closely with the radiation oncologist – the surgical plan would need to mesh with the future radiation strategy. Also, the Boston surgeon had said he would use an endoscopic approach through the nose. This sounded much better to us at that time than the more invasive approach suggested at the Clinic. At this point, there was reason to believe that proton beam would take care of the remaining tumor. The percentages (for skull-base, 98% local tumor control at five years, 95% at ten years) certainly seemed to be on our side. So for these reasons we did not investigate other surgeons, and decided to have the de-bulking performed at nearby Mass Eye and Ear.

An important consideration
A major factor that Dr. Liebsch was carefully considering was the proximity of Ray’s tumor to the right optic and cranial nerves. Ray was born with amblyopia and had always relied on his “good eye” – the right one. So any treatment that was undertaken had to be balanced with the possible risk of damage to the right optic nerve, resulting in dramatic vision loss.

Other CS patients with a tumor in the same general area usually complain of headaches, which Ray had, and double-vision, which he did not have -- because he really was only using one eye his whole life. He had never made a big deal out of this, and until this tumor made itself known, I wasn’t even aware of his amblyopia. If he’d had double-vision, it might have presented sooner than his blocked airway, giving us more of a head start on diagnosis and treatment.

All his life, Ray said, he’d worried about something happening to his “good eye.” So of course, preserving the vision in that eye became a great priority in the planning of his treatment.


Treatments begin
The partial resection was completed in Boston in February of 2003; proton-beam therapy was eventually scheduled to begin in June of 2003. In between, I noticed that Ray had trouble with movement in his right eye. He could not move his eye to the right.

Seven weeks of proton-beam radiation included 10 photon and 25 proton treatments. Ray stayed at the Hope Lodge in Worcester, Massachusetts.

This is a home-away-from-home for cancer patients receiving treatment in Boston, and it is funded by the American Cancer Society. Volunteers drive patients to and from their appointments every day.

Ray’s right eye not only didn’t move to the right, it was now continuously “cocked to the left.” It seemed that the tumor had grown enough after the initial resection that it was now impinging on the cranial nerve that controls eye movement to the right. It was hoped that some weeks after radiation was completed, the residual tumor would shrink minutely and come off of the nerve.

Ray got through his seven weeks of radiation therapy with one major downside: fatigue.

By November of 2004 the eye movement was noticeably worse, and more worrying than that, Ray was having visual difficulty as well. We met with the Cleveland Clinic’s neuro-opthamologist. His tests indicated that the optic nerve was being compressed … and it had to be that the tumor was now again active. His first thought was additional radiation, but since he had already received the maximum dosage, he referred Ray to a Clinic neurosurgeon.

A series of craniotomies
Based on these symptoms and new MRIs, the neurosurgeon wanted to very quickly operate, since the immediate concern was to save the vision in Ray’s good eye. Dr. Liebsch recommended very conservative surgery, as he felt strongly that the tumor was not really active, but had calcified enough to compress the nerve. So this is the type of surgery that was performed, a partial resection via craniotomy. The immediate result was very good: Ray’s vision was dramatically improved. Unfortunately, the tumor certainly was active again, and four months later, after similar symptoms, a similar surgery was performed. Tumor removal was complicated because of the previous radiation, which had turned much of the tumor rock-solid. So new tumor was removed while radiated tumor was drilled into, on the theory that only part of it was active. The hope was that the remaining tumor had been irradiated sufficiently that it would remain inactive.


An agonizing decision
After this third surgery, the surgeon indicated that if the tumor continued growing, there would be only a final option available: aggressive resection, including sacrificing the optic nerve. Unfortunately, in August of 2005, this is the decision that Ray had to make in the hope of finally stopping this unrelenting beast of a cancer. A partial angiogram was performed, in which the blood supply to the brain through the right carotid artery was gradually cut off, to verify that Ray could survive with the blood flow through the other carotid artery. The results were good, and it could be that his brain had already developed alternate blood vessels to compensate for the compromised blood flow on the right. So, Ray braced himself for what was termed an “aggressive” surgery, but was still only a partial resection. His right internal carotid artery was clipped, his right optic nerve and other cranial nerves were severed, and as much tumor as possible was removed.

Ray recovered from this surgery but the loss of vision in his good eye was certainly a profound blow for him. He struggled with depression but eventually battled back and was much more functional with his remaining vision than we had ever thought would be possible.

Return of bad news
Unfortunately, as happens so often with chondrosarcoma, a recurrence eight months later put us back into survival mentality yet again. A “routine” MRI revealed that all the space cleared by the last resection was completely filled up by new tumor. Though this news was a complete surprise, maybe it shouldn’t have been: I had noticed that Ray’s right eye, which is closed because of the nerve damage, didn’t look right. It was actually bulging slightly from the buildup of fluid behind it and the involvement of tumor in his right orbit. Attempting a total resection was now the only hope.

Complete removal of the tumor had never really been discussed up to this point. The difficulty of the location, in the cranial base, made the possible complications (leaking of the cerebrospinal fluid or CSF, stroke, blindness, infection) very dangerous. The size of the tumor meant that repairing the separation of the brain from the sinus area would be extremely challenging and risky. The Cleveland Clinic neurosurgeon decided that the risks were too great to proceed.

A ray of hope
We knew of Dr. Chandraneth Sen from St. Luke’s Roosevelt Hospital in NYC, because he had succeeded in complete removal of a very similar tumor from an online friend (from this group). He was probably also one of the surgeons that Dr. Liebsch had mentioned to us initially. His team specializes in cranial-base surgeries; these involve the very difficult area at the base of the skull, behind the eyes and below the brain.

Immediately after receiving the devastating decision of the original neurosurgeon not to go forward, Ray was on the phone to Dr. Sen. Ray had already sent him the latest MRI for his opinion, and Dr. Sen and his co-surgeon Dr. Peter Costantino evaluated the scans and agreed that they would perform the surgery. Not an hour later, Dr. Sen called back and said that they had just seen something on the MRI that no one else had mentioned: the tumor was dangerously close to the other optic nerve. They would operate, and the situation was “not completely hopeless,” but if they found that their approach would mean severe damage to that optic nerve, they would have to abort the attempt at complete resection. Still, we had only this option, and we were very grateful for it.

In a planned two-stage operation, a couple of months apart, Dr. Sen and Dr. Costantino removed all of the massive tumor bordering the brain and did an extensive repair, inverting the “chewing muscle” on the right side and using it to rebuild the damaged barrier between the brain and the sinus / orbit. Abdominal fat and synthetic materials (pioneered by Dr. Costantino) filled in the space and supported the area. In a later surgery, a shunt from the brain to the abdominal cavity was implanted, to drain away excess cerebrospinal fluid that was putting downward pressure on the repair. In a third surgery, the remainder of the tumor (further away from the brain, involving the Eustachian tube and right mandible) was removed.

Recuperation from these procedures, especially the first one, was somewhat long and difficult, but the major risks did not materialize: the first night Ray was able to count fingers, so his left optic nerve was saved; there was no brain damage or stroke; a CSF leak was encountered but successfully patched; and no major infections followed. Ray was on a ventilator for several days after the first surgery, and he was in St. Luke’s neuro-ICU for two weeks. His right eye was extremely swollen and the lid was sutured shut to protect it. Ray reacted badly to anaesthesia, becoming temporarily delusional and combative after every major procedure. With each day after the first week though, things gradually became more normal: he came off the ventilator, tubes were taken out , IVs discontinued, he slowly resumed eating, and finally the nasal stuffing was surgically removed. After three weeks, Ray was ready to leave the hospital and head home to Ohio.

One year later
Subsequent MRIs have shown no clear evidence of residual tumor and as of the most recent one, no recurrence. Ray has gotten much of his strength back. He still has headaches but they are considerably less severe. He also has some nerve damage and pain and numbness around his mouth, which is helped with Nortriptyline. After completing a special low-vision program involving a sight aid called a bioptic telescope, he has gotten his driver’s license back. Ray walks the track at the gym daily, and recently gave kickboxing a try again (!).

Recurrence is certainly an ongoing concern, knowing the nature of chondrosarcoma. But we have had a solid year now of no bad news … and last May I would not have thought that was possible.

In hindsight
We wish we would have explored the possibility of total resection as our first option rather than our last. Because of Ray’s vision difficulties and the proximity of the tumor to his optic nerve and carotid artery, I’m not sure that even Dr. Sen would have been able to recommend that approach – not with the great success of proton beam therapy in most cases. But it is much harder for surgeons to deal with the scarring and complications of previous surgeries and radiation, than it is to operate on a site for the first time. We were lucky that Dr. Sen and his team have much experience working with these conditions.

It could be that we should have pushed for the initial surgery and radiation to have taken place sooner after diagnosis. There was a span of seven months between diagnosis and radiation. Acting sooner might have prevented involvement of the optic nerve and other cranial nerves. It could well be that in the end it wouldn’t have mattered anyway, but we do know that in the intervening months, the tumor grew and maybe the radiation strategy had to be adjusted because of this new growth. When a chondrosarcoma tumor arises in this part of the skull base, there just isn’t much room between critical structures, so maybe time is more of the essence in this location.

For more information on the treatment of skull-based tumors, including a link to other cranial-base surgical teams in the U.S., go to:

http://www.cranialbasecenter.org/

Newer developments
In November of 2007, a follow-up MRI showed “something.” Cleveland Clinic radiologists could not say definitively whether it was fluid, tumor, or even an aneurism. PET scan did not show activity normally associated with cancer. Dr. Costantino was somewhat optimistic that this was something other than tumor, but when Dr. Sen reviewed everything, he seemed to know that it was a recurrence.

In February 2008, Ray had another ear-to-ear incision and craniotomy to remove this very aggressive and persistent tumor. The surgery went smoothly and we headed home for Ohio after only a week or so. Two weeks later he was re-admitted to St. Luke’s with a severe infection. Somehow, air had leaked into the sinus and created a space for infection to develop. (We don’t know for sure, but possibly the pressurization of the airplane so soon after surgery was a factor. We don’t fly anymore.)

The next day, Dr. Sen and Dr. Costantino re-did the frontotemporal craniotomy
and cleaned everything up. There were no complications. This time they kept him for two weeks on IV antibiotics before discharging him, and we stayed in the area another week, just in case, before going home to Ohio.

Then, in a matter of weeks, a routine MRI blew our minds. There was yet another recurrence, this time just below and abutting the right eyeball. In May Ray had surgery to remove this tumor as well as the right eye. This was the blind eye, wher e the optic nerve had been severed in 2005. Dr. Costantino did the surgery, this time approaching through the cheekbone. Ray recovered well and again we headed home after a couple of weeks.

We went back to NYC for a follow-up MRI and appointment with Dr. Costantino. As of June 17, 2008, Ray was apparently tumor-free. We will go back in September to follow up again.

As of right now, Ray is feeling pretty well though the headaches and facial pain and tightness may be a chronic condition. He is walking every day, doing yardwork, and still driving. We attended our daughter’s graduation from Ohio State in June and are looking forward to the wedding of Ray’s god-daughter this weekend.

Gratitude
Ray’s story is unique and his journey has been difficult, but because of his determination and force of will, his refusal to quit, his resilience, and his resolve to find the best treatment and follow through with it, he is a chondrosarcoma survivor almost six years after the onset of symptoms. We are extremely grateful for the knowledge, support, and friendship provided by this group, and for Elizabeth, who makes it possible.

Beth Kujala
August 7, 2008

Note:
In the last sentence, Beth is referring to the Chondrosarcoma Support Group of which they are members.

November 2010: Ray is still doing well