As we wrote last week, treatment decisions about craniopharyngiomas are complex, with no easy answers and no consensus approach. In that initial overview post, we described why we were leaning towards a very conservative approach to Neva’s surgery – one where the fluid portion of her cystic tumor would be drained, but nothing else removed. This is one type of what’s known as a subtotal resection (STR). The alternative general approach to removal of craniopharyngiomas is known as a gross total resection (GTR), or an attempt to remove the entire tumor.
Each approach carries risks and benefits. The main benefit of an STR – especially a fluid drainage only STR – is that it has a lower chance of damaging the pituitary gland further, and a lower chance of damaging sensitive surrounding structures like optic nerves and the hypothalamus. The biggest argument for a GTR is that, if successful, it substantially lowers the odds of tumor recurrence, a major problem with craniopharyngiomas.
As we also noted last week, the specifics of Neva’s tumor create hope that she can do well going forward, because it is relatively small and does not currently invade surrounding structures. That puts us right in the zone where the decision between STR and GTR is perhaps most difficult. For large tumors that already affect other structures, most surgeons now feel that a GTR is too risky and likely not possible anyway. In other words, you’ll go in, create new damage, and still not get it all out. But in Neva’s case, the surgeon quoted a 75% chance of a GTR with minimal new damage – maybe even none. Overall, the chances of new endocrinopathies following pituitary surgeries are high (up to 70%), odds that we are well aware of. However, we are optimistic that the size and location of Neva’s tumor puts her on the hopeful end of these statistics.
A further complication is that STRs are usually coupled with radiation to help lower recurrence odds. But radiation is not a good idea in young kids. According to the literature and to Neva’s oncology team, this means that the odds of rapid tumor recurrence following a conservative STR are frighteningly high – maybe 80% or more. Worse yet, such recurrence is most likely to happen quickly. As Neva’s oncologist put it: “Odds are that she’d be back in here facing the same problem in less than two years.”
Ouch. When we coupled those daunting statistics with the fact that subsequent surgeries are much riskier (because of past scar tissue), and carry with them even higher chances for new endocrinopathies, we realized that the most conservative STR approach is probably not the best one for maximizing Neva’s overall safety and her chances for the highest quality of life. Remember that a major part of the original justification for the STR approach was to maintain Neva’s endocrine function for as long as possible. Given the odds discussed above, we no longer can conclude that an STR maximizes those chances, while we can conclude that an STR may carry other more serious risks in the near future. Moreover, new approaches to tumor-related endocrinopathies are already on the way – ranging from time-release mediations to implants to even stem-cell-based gland regeneration in the more distant future. Thus, managing endocrine deficits in Neva – while not trivial – should get easier and less risky as time passes. But the risks of repeated surgeries are not likely to decline. So we have swung back to Dr. Hankinson’s original recommendation of trying for a GTR.
There is a critical caveat to how Dr. Hankinson will approach the surgery: the GTR won’t be pursued at all costs. The idea is to minimize overall risks, and that means having to make some decisions during surgery itself. In some cases, a cystic tumor like Neva’s will simply fall away from surrounding structures with ease following fluid drainage. If that happens, the complete removal carries low risks of new damage, and that is unquestionably the best of all possible outcomes. In other cases, the calcium that is part of these tumors can create adhesion between tumor and native structure cells, causing the removal of those tumor cells to be far riskier. There may even be a mix of these conditions – i.e. some parts of the tumor wall fall away and are easy to get, others need to be left in place. After talking with the oncologists, it seems that even that latter scenario would be better than just fluid drainage in terms of lowering recurrence odds.
Boiled down, that’s how we will probably ask Dr. Hankinson to proceed, and it is his original recommendation. He would go into the surgery with the hope of getting it all. And he would remove everything that can come out easily. But he would not push things if any part of that removal appears risky. His overall philosophy for these tumors is still a conservative one, and his balance and good judgment are obvious, so we do trust him to make these decisions while in surgery itself. Obviously, we all hope that the full removal goes smoothly, with minimal new damage. If so, it gives Neva the best chance for the safest, fullest life going forward. Her surgeons and oncologists feel this is a chance worth taking, and we agree.
For better or worse, it appears we will have a few weeks to continue evaluating this surgical decision. Her operation requires both Dr. Hankinson and an ENT surgeon to be in the room, and it does not look like that can happen next week, which means we are looking at early January. As we learn more, we will share it here.
Meanwhile, Neva’s bravery and ability to handle this new turn in her life are remarkable. She knows doctor visits are coming hard and fast, she knows some of them won’t be fun, she knows that surgery is in the near future. She seems to meet each challenge incredibly well. At times she prepares herself by pretending to be the doctor or by describing how her imaginary friend (named Munchie…really…) has to have surgery and why. At other times she puts on her patented game face, shown here. She’s smart, funny and tough — all attributes that will help carry her through.