What is a craniopharyngioma and what are we doing about it?

My original intent in writing here about my daughter’s pituitary tumor was not to use this blog as a continuing portal of information on her health challenges.  But, that initial post (and subsequent ones from my wife and from me) led to help as we navigated difficult medical decisions, and to many more people simply providing wonderful support.   A number of people have asked us to continue providing updates on both our experience and on the nuts and bolts of the process.   We’ve decided to do so going forward, in part because we hope it may help others who face the same or similar challenges.  

The post that follows outlines two things:  1) information on craniopharyngiomas, and 2) where we stand (and why) in treatment decisions.   As always, thanks for your support.

UPDATE:  All of what’s below remains relevant to understanding craniopharyngiomas and the ways in which we are evaluating Neva’s specific care plan going forward.  But the section entitled What will we do? is no longer entirely accurate, in that upon further review and consultation, we’ve decided on a somewhat different surgical approach.  More details on that decision will be posted shortly.


What is a craniopharyngioma?

Taken broadly, pituitary tumors are far more common than we – and probably many of you – realized.  Cadaver dissections suggest that 20% or more of adults will have some form of pituitary growth by the end of their lives.  The vast majority of these are asymptomatic, so unless they are discovered for other reasons, most people never know they have one (that’s usually a good thing!).   Nearly all forms of pituitary tumors are also benign in the sense that they do not metastasize into systemic cancer.  Obviously that’s also a good thing.

But…because they occur in the brain within and near sensitive structures, they can cause multiple significant health issues.  The pituitary gland plays a central role in hormone regulation of thyroid and adrenal function, growth, sexual maturation, reproduction, and the body’s response to stress and illness.  A pituitary tumor can disrupt all of these, and removal of that tumor surgically (and/or control via radiation) can cause further pituitary damage, often leading to a condition called hypopituitarism – the widespread failure of the gland to do any of its main jobs.

The radiologists, oncologists and neurosurgeons all believe that the odds are very high (>90%) that Neva’s tumor is a craniopharyngioma. These can only be diagnosed for certain following surgery. Craniopharyngiomas are one type of pituitary tumor, and a pretty rare one.   On numerical odds alone, Neva should be the only person in the city of Boulder who has one.  They occur in both children and adults, though the biology of each is typically different.   In children, they arise as cellular remnants of a structure known as the Rathke’s pouch or cleft, and there are no known risk factors or genetic links.  They often get bigger than a golf ball prior to diagnosis.  One fortunate aspect of Neva’s specific condition is that her tumor is on the smaller end – roughly the size of a large almond.  It is contained to an area known as the sella and supersellar space – i.e. near and above the pituitary gland itself, which sits atop a depression in the middle of the sphenoid bone known as the sella turcica (literal translation: Turkish Chair).

As with most pituitary tumors (sometimes referred to as “lesions”), craniopharyngiomas are not typically malignant.  However,  they often cause pituitary endocrinopathy (varied failures of the pituitary gland), and they can interfere with neighboring structures, at times with additional very serious effects.  Such interference can include the optic nerves (loss of visual function is one way they are diagnosed), the hypothalamus, carotid arteries, and cranial nerves. They can also lead to hydrocephalus, an accumulation of ‘water’ in the brain, by preventing proper drainage of cerebrospinal fluid. Finally, they can cause severe headaches, a symptom that we have seen in Neva. Sometimes their effects are due to intrusion of the tumor cells into other structures, sometimes it’s simply about the pressure of the tumor on those structures.  Craniopharyngiomas are variable in their morphology, but typically contain a mix of solid matter and a fluid filled cyst.  This is true for Neva: much of her tumor’s volume is fluid, and her tumor’s composition is a major contributor to the likely treatment option we will choose (more on that below).

Lots more information is out there, in both web sites and primary literature.  One good place to start is this site from the UCLA Neurosurgery group.  But because this is a rare condition with varied treatment options – and varied opinions on those options – the information can be confusing and stressful to navigate.  If you’re facing your own decisions on any kind of pituitary tumor, please do not hesitate to contact Diana or me.  Our overall picture of both the risks and the best treatment options certainly evolved as we continued to gather information.

How are craniopharyngiomas treated?

Here’s where things get tough.  There is no widely accepted, single approach to dealing with this tumor, other than the fact that nearly all of them need to be surgically removed.  Surgery is necessary because they do not respond well to any kind of chemotherapy, and while radiation can help control or even reduce small tumors, it is not hugely effective as a sole approach.  So, as long as they are in a location that is considered operable, you have to go get them out. While they are slow growing, most will continue to grow and in so doing create new and more serious effects.

As with much of medicine, the specifics of the case matter a great deal.  A big tumor that has intruded into other structures is tougher to deal with than a smaller, well-contained one.  Kids are different from adults.  And so on.  But, what gets confusing is that different surgeons will look at exactly the same tumor in exactly the same individual and recommend different approaches.  A widespread debate about many craniopharyngioma cases hinges on the following:  is it best to try to remove the whole thing (known as a Gross Total Resection, or GTR)?  Or is it better to remove some percentage of the tumor, often followed by radiation (Subtotal Resection, or STR, combined with External Radiation Therapy, XRT)?

In your gut, a first response is typically:  WTF, get it all out!  But this approach carries big risks.  Trying to remove all of the tumor results in high rates of further damage to the pituitary gland itself, thus a patient who has only partial pituitary endocrinopathy going into surgery usually emerges with bigger problems, including truly crappy ones like diabetes insipidus and poor regulation of cortisol production.  As well, GTR runs a higher risk of damaging optic nerves, the hypothalamus and other key structures.  Optic nerve damage has obvious consequences, and hypothalamic damage usually creates a host of new, very difficult physiological and behavioral issues that can greatly reduce quality of life.

In addition, the recurrence rate for craniopharyngiomas even after GTR is unfortunately high.  It is higher still if you go for the STR approach…but the latter can carry much lower risks of adding to a patient’s burdens right away, especially if the STR simply targets removal of the fluid from a largely cystic tumor (such as Neva’s).   In that case, fluid removal alone has low risks of further damage, tends to “collapse” the tumor to a much smaller version of itself that now does not exert pressure on the pituitary or other structures, and because of this may even allow recovery of some pituitary function. The downside is that because STR results in a higher recurrence rate, the need for another surgery down the road – maybe even multiple surgeries – is elevated. And once an initial surgery is done, follow up ones tend to be trickier and riskier because of scar tissue, potentially carrying even higher risks of collateral damage.

All of this starts to illustrate why there is no one-size-fits-all approach.  No course of action is ideal, no course is without risks.  Some surgeons are bigger advocates of GTR because of the recurrence risks, others advocate a more conservative approach that favors buying time and minimizing additional damage.   The debate can get especially tricky in tumors like Neva’s, where (happily) the effects appear largely contained to the pituitary gland, and not to surrounding structures.  A common response for a well-contained tumor like that is:  get it out!  Easier to do for a small tumor, with lower risks to the hypothalamus, etc.  True enough.  But….the pituitary damage risks remain high, and that’s especially worrisome for young children who have not yet gone through their full development and growth.

What will we do?

We continue to solicit opinions from multiple surgeons and other physicians (endocrinologists, oncologists, etc), but we are highly likely to choose a conservative, fluid drainage only, approach to Neva’s tumor.   This was the recommendation of Dr. Nalin Gupta, one of the world’s experts in pituitary surgery and in craniopharyngiomas, and it is a philosophy supported by the surgeon we will likely use here in Denver (Dr. Todd Hankinson), who is also a leading craniopharyngioma researcher.  The main reasons are outlined above.  Neva is very young, so the consequences of further pituitary damage are higher than they would be for someone already through puberty.  Her tumor is largely cystic, so fluid drainage should remove the pressure, and make it quite small.

A key missing piece of our decision-making process is the need to map Neva’s current endocrine function. And, frankly, getting straight answers and consistent advice from the pediatric endocrinologists has become the most frustrating aspect of dealing with Neva’s condition. For us, it is essential to understand Neva’s current endocrine function to make decisions about surgical approaches – if Neva already displayed hypopituitarism (unlikely, see below), then an attempt at a GTR may be the most logical tactic.  It is more than a bit surprising that assessment of endocrine function to help inform surgical choices (not just manage the surgery itself, the reason often given for pre-operative evaluation) is not standard practice. We have learned quickly that we must ask questions, read the literature and be willing to engage in active debates with Neva’s caregivers.

While we still don’t know for certain how much of Neva’s endocrine function is already damaged, early evidence suggests it may be restricted to reduced production of growth hormone and thyroid stimulating hormone.  We will thoroughly assess her current pituitary function before a surgical approach is finalized, but assuming she still has considerable pituitary function, that means the fluid-drainage (i.e. conservative STR) approach should maintain that function and maybe even improve the gland.   In other words, it has the best chance to keep Neva closest to who she already is.

The biggest risk to this approach is an elevated chance of tumor recurrence.  That could well mean a second surgery, and because the first surgery will be via her sinus cavities (an increasingly popular and less invasive approach), the second one might have to be a more traditional craniotomy.  Ugh.  And the chances of a “clean” (i.e. low damage) GTR are worse in a second surgery.  Like I said, no easy choices here.

But…all patients with craniopharyngiomas are monitored for life via regular MRI’s, and Neva will be no exception.  That means that if the tumor grows again, we will know quickly, and it will never again get as big as it is today.  As such, it will never threaten structures like the hypothalamus or optic nerves in the future – another reason we favor STR now, because a GTR approach does run some risk of damage to these structures.  Fluid drainage alone runs almost no risk of such damage. The one caveat here is that even slow-growing benign tumors can exhibit non-linear growth as cysts form and can be unpredictable.  Again, proper monitoring via MRIs will be essential.

Doctors do not favor radiation for children so young, and we agree, so her STR (again, just the fluid removal in all likelihood) would not also include XRT (radiation).  But she may need that radiation in the future – maybe even the near future – as an initial control step if regrowth begins.  The hope and major intent of this more conservative strategy is that she gets through most or all of her childhood development before any major recurrence happens.  And if she is lucky, no major recurrence ever occurs –that too is possible.  Following STR, some of these can simply sit for good in a largely or completely inactive form.

We emphasize that we continue to learn all we can and talk with experts prior to finalizing a decision.   Our top priorities are Neva’s survival first (of course), and maintaining her cognitive function second.  After that, we prioritize minimizing risk to the hypothalamus and optic nerves, followed by maintaining all possible pituitary function.  Our read of the options and specifics of her case is that choosing the conservative STR approach is best for all of those priorities, even when weighed against the risks of higher recurrence.  But if you face a similar decision, your specific circumstances, and/or your read of the various risks and benefits might differ.  If you are in that unfortunate situation, please don’t hesitate to contact us, and please do learn all you can.  Craniopharyngiomas and their treatment are complex entities where anyone’s eventual choice will inevitably be a trade-off of risks amongst many shades of gray.   That’s tough, but it’s reality.

When will the surgery happen, and then what?

We don’t know yet, but surgery is likely to occur either sometime during the week of December 9th, or just after the turn of the year. We are nearly certain to have Dr. Hankinson perform the surgery, as we feel good about his training, experience and exceptionally thoughtful and balanced approach.  (In fact, we are enormously grateful to both Dr. Hankinson and Dr. Gupta for being the opposite of any bad surgeon clichés – their balanced, intelligent, caring and responsive approach has been a gift beyond measure).  If all goes well, Neva should not need to be in the hospital too long (hopefully 3-4 days at most).  But we will not schedule surgery for any later in December than the week of the 9th, because we don’t want to run the risks of her being hospitalized during Christmas week, when many of the most experienced staff may be on vacation.

Once her initial recovery is complete, we will then need to assess her endocrine function again, and hone in on longer-term treatment plans.   Almost nobody with one of these tumors can avoid some kind of endocrine replacement therapy, and we expect that Neva may need long-term courses of thyroid and growth hormone supplements at a minimum.   The latter involves a daily injection, which is a bummer, but both hormones can be replaced quite effectively, meaning she would get the kind of help her body might need.  Our hope – as described above in the surgical rationale – is that we can keep her long-term therapeutic needs to these hormones and not have to worry about steroid replacement and/or management of diabetes insipidus, both of which are most worrisome in younger children. Our dream is that medical technology continues to evolve and hormonal implants or even pituitary replacement devices become a reality. Time to change our sabbatical research plans…

That’s where things stand today.  As key developments occur, we will try to share them here.

As I finish writing this post, Neva is dancing about the living room, mimicking The Nutcracker Suite she saw today with her best friend Luna. She twirls, hops and bows, full of smiles as usual.  This morning, she ripped around the local bike park as never before (and this is Boulder remember, so the bike park here is, well, not your average bike park….).  In other words, she spent the day reminding us once again that she will lead us through.



14 thoughts on “What is a craniopharyngioma and what are we doing about it?

  1. Dave Jemielity

    Hey Al,
    My first reaction is: that girl was astute in her choice of parents.
    Second reaction: recent article in NYT on surgical skill evaluation suggests that you two would do well to ask all the embarrassing, prying questions you need to to assess the manual dexterity (which isn’t the same as worldwide authority knowledge, right?) of whoever operates. Differences in outcome are huge. Medical/surgical profession: not super-open about this.
    Third reaction: hang in there. Pulling for you.

  2. Kelly Beeland

    Thank you for sharing so much about Neva’s diagnosis and your decision making process. I read this post with great interest and with tears in my eyes by the end. I have so much respect for you and Diana because of how your are handling this situation. And I love Neva!! I’m looking forward to seeing you next year and seeing all the kids together again. Big hugs from DC! Kelly

  3. Jenny Haan

    Thank you so much for sharing all of these details with us. Neva is so very lucky to have you both. Alan, you and Diana are so very special and will make all of the right decisions as hard as some may be. You are definitely, right…Neva’s spirt and vibrant energy will overcome all of this. Hugs to you all. I am thinking about each one of you everyday!! 🙂

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  6. Patty

    My daughter was also diagnosed in Aug.2013 with 3 craniopharyngiom…we live in Atlanta, Ga and see doctors at Children’s Hospital. We decided not to do surgery due to the risk factors, so we are doing a experimental drug called peg interpheron. It has been seven months and so far it is all stable. They will do another MRI in april to see if the cysts and tumor are getting smaller or stable. If they have gotten bigger, then they most likely will do surgery. but so far they are stable!
    She is almost 15 yrs old ( ready to get her driving permit) and doing awesome! She is in the 9th grade and has made all A’s! We pray that this drug with help not only Elizabeth, but also all other children who are going through this!
    Please look into this drug…it has been tested on recurring cyst and they have shrunk the cyst and tumors! Which is great news, however,Elizabeth is the first ever with a primary tumor. The doctors are very pleased so far.
    I will be praying for your daughter and I hope this information helps you!

    1. Guila Ouaknine

      Hi i would like to learn more about your daughter and her experience with the interpheron. Any shrinking of the tumor? Her hormones dealing? Please I need to here about all that because my daughter was diagnosed with the same kind of tumor. Thank you, Guila

      1. patty ryan

        The tumor is stable. The team of doctors feel it is best for her to stay on the meds for another 2 years. All her hormones levels are GOOD! She continues to get her period too. Because the cysts and tumor is not growing and her eyesight is good they feel this is the best plan for her.
        I’m sorry that I am just seeing all of this. I just saw this come over in mail.
        I hope this info helps anyone who is looking for info of this.

  7. Caroline

    I hope all is well with your daughter, I understand only too we’ll the rock and a hard place you find yourselves in. I was diagnosed with the same condition, but at the age of 23. I new something wasn’t right for a couple of years, but doctors were telling me it was nothing, just stress. The headaches got progressively worse, until I would have done ANYTHING to relieve them, pain killers just varied the amount of the headache. Eventually I think (luckily) things were taken out of their hands as the I developed a form of meningitis to do with the tumour leaking, at least this is the theory. I also lost peripheral vision, the medications were having no effect on the tumour. I had to have surgery. This is where I want to emphasise the positives, the recovery. As soon as I regained consciousness my sight had fully returned, no more being in the tunnel. After some weeks of monitoring I was able to withdraw from the cortisol completely. I have been left with some permanent damage to the gland, and I am on thyroxine and female hormones. My point is though, my life is so much better now, even with the supplementation. I can honestly say I couldn’t have continued in the state I was in prior. Even if your daughter has to go down the surgery route, there is light the other side. God Bless what ever road you take

  8. Guila Ouaknine

    Please give us an update with your Neva. We are desperate since our daughter Shirel was diagnosed with a craniopharyngioma. We are seeking for answers.
    Guila Ouaknine

    1. patty ryan

      Please email me. Pattyryan4@comcast.net

      My daughter Elizabeth was diagnosed last year at the age of 14. She did not have surgery, but is on experimental medicine at childrens hospital in Atlanta, Ga
      Elizabeth is doing ok. She is marching through 10th grade and has some side effects from the medicine, but its more like flu like symptoms.
      Her cysts and tumor are staying stable as of now and drs are continuing to watch her. There is not a lot of info out there for this so please contact me if you like!

      1. Amber

        I am just reading this now and wanted to say that my husband had a craniopharyngioma right around the time of your daughters and we live 30 miles from Boulder! How crazy! We used Dr. Lillehei at CU hospital in Aurora. He was able to resect the entire tumor and now we just follow up with an endocrinologist in Longmont every 6 months and do an MRI once a year. So far so good. How is your daughter doing? I will keep her in my prayers. It is no fun being on hormone replacement but at least we know the entire tumor is out and my husband can go on with normal life again. He is 28 yrs old.

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