One Patient’s Path to Diagnosis – “maybe it’s not a pheo?!” By Anonymous
It was quite an odyssey, but I can happily say that my tumor was taken out a month ago, and I am almost completely recovered. My voice is still a bit weak, but it should go back to normal in the coming weeks/months, other than that my life is completely normal. That’s the short story. I share the long version of my story below because I think there are many lessons to learn.
I am a 52-year-old male, in great health overall. For 5 years I had dealt with sleep apnea possibly caused by what looked like an enlarged left tonsil.
- OCTOBER 2020: I started feeling some pain when swallowing and visited an ENT doctor who felt as if my left tonsil was not really enlarged but was being pushed forward. She ordered a CT-scan which determined that there was a large mass (6.5 cm) in my parapharyngeal space at the base of my skull, probably a paraganglioma, or perhaps a schwannoma. This was followed by MRI/MRA scans and several tests, which did not change the diagnosis, but confirmed that there were no immediate risks or other symptoms to worry about.
- NOVEMBER-DECEMBER 2020: I started a long process of reading every paper on the topic I could find and visited several great doctors at top institutions in the Washington DC area, where I live. This is what they said (doctors and institutions shall remain nameless):
- Doctor #1 (ENT surgeon) said that it was a paraganglioma, surgery was the only option but had a very high chance of permanent nerve damage impacting swallowing, speech and potentially other functions, believed it would be necessary to embolize an artery and split the jaw during the procedure, risk of stroke was 15%, radiation was not even a possibility.
- Doctor #2 (radiation oncologist) said that it was a paraganglioma, radiation was definitely an option but had many potential risks, so thought that surgery was a better one.
- Doctor #3 (radiation oncologist) confirmed what I had seen in several scientific papers regarding the fact that radiation was a perfectly viable option with lower risks than surgery in my specific case. However, he elevated my case to the tumor board of his institution who determined that the scans showed that the tumor was likely a vagal schwannoma and not a paraganglioma. He then recommended to consider surgery given the lack of extensive data regarding success of radiation on large schwannomas.
- Doctor #4 (ENT surgeon from same institution as #3) indicated initially that surgery was viable, risk of permanent nerve damage was 5% and it would require splitting my jaw to extract the tumor. He called a few days later saying that after some research of existing literature he felt that the risk was a lot higher, but surgery was still my best option.
- Doctor #5 (neurosurgeon specialized on neck) confirms with a Dotatate/PET scan that the tumor is not a paraganglioma and is likely a vagal schwannoma. Surgery is recommended and the risk of permanent nerve damage was at 10-15%. No need to split the jaw.
- Doctor #6 (ENT surgeon from same institution as #5 who would work with him): Estimates the risk of permanent nerve damage at 50% and recommends radiation as my best option.
The only point in which they all agreed was that, given the size of the tumor, waiting was not a good idea. The other thing they had in common: like most top doctors in the US, they treat no more than 1-2 paragangliomas (and even fewer schwannomas) per year, so their experience with this kind of tumor is extremely limited, which, in my opinion, explains the diversity of the advice they provided. Few surgeons were familiar with intracapsular enucleation, few used nerve function monitoring during the operation or combined ENT and neurosurgeon skills.
In January of 2021, thanks to Google, I found first the website www.vagalschwannoma.com and then a facebook group for patients with neck schwannomas. I got lots of great information from both and learned about Dr James Netterville (Dr N) at Vanderbilt University Hospital in Nashville, TN, who is a surgeon and a specialist in schwannomas (he has done around 200 operations in the last 30 years, probably more than anybody else).
Dr. N. looked at my scans and we had a Facetime call without setting an appointment to give me his opinion. He confirmed that just by looking at the CT-scan it was clear that the tumor was likely a schwannoma. He said he felt confident about a good outcome of surgery in my case and that he would be honored to treat me. He explained in detail how it would be done (no need to split the jaw), he even showed a video of a recent, similar operation to explain how nerve function is monitored and preserved. I felt overwhelmed by his kindness, expertise and confidence. I was lucky to schedule the procedure for Feb 9th, after an office visit the day before.
Doctor #4 and #5 confirmed that Dr N was the best in the field and that my choice was very wise.
On February 9, I had surgery with Dr N and his team at Vanderbilt (4.5 hours). I felt at all times that I was in the best hands. I was talking almost normally just after I woke up and started drinking without any issues shortly after. I was moved to a room just a few hours after waking up. Everyone taking care of me was wonderful.
- FEBRUARY 10th: I was released from the hospital less than 24 hours after the end of surgery and had a normal lunch and dinner with solid food at the Airbnb we rented.
- FEBRUARY 11th: I went for a 3-mile walk. Other than the drain tube sticking out of my neck, it would be hard to know that I had major surgery just two days ago. I feel zero pain (only having Gabapentin and Tylenol). My voice is still recovering slowly (slightly lower pitch than before) but I can communicate without any issue and it’s getting stronger every day. I have been sleeping normally, my apnea seems to be gone for good.
- FEBRUARY 15th: Final office visit with Dr N who confirmed my nerves were fully functional and removed the drain tube, heading back home the next day.
From this journey I have learned the importance of reading many scientific papers to understand the problem and know what questions to ask, getting multiple opinions from the best experts you can find, not being afraid about checking a surgeon’s real experience treating a rare condition, and finding resources through support groups created by patients that share the same disease. There is no perfect option but take your time to assess alternatives and make the best decision.