The title of this journal entry comes from my Uncle Matt, who once shared a helpful phrase when making a medical diagnosis.  “Common things happen commonly,” is an elegant way of saying, “If it walks like a duck, quacks like a duck, etc…. then you’ve got yourself a duck.”

Last week, Vera underwent a series of scans to learn about this tumor in her leg.  Each step of the way, they were concerned by its characteristics—by the end of the week, our pediatric oncologist and orthopedic surgeon explained that this looked like a textbook rhabdomyosarcoma tumor.  Given the tumor’s distinct characteristics in each scan, the “no-brainer” diagnosis was rhabdomyosarcoma because they knew that “common things happen commonly.”  That’s why we stayed for a three hour clinic visit and discussed options for surgery, chemotherapy, and open clinical studies we could utilize for other treatments; we met with numerous social workers and oncology support staff who assured us they would be with us throughout Vera’s journey; we cried in the parking lot for 10 minutes before driving away; we got home and ordered matching yellow bracelets (yellow for sarcoma) so we could think of Vera wherever we went, in addition to bulk masks and hand sanitizer so we could “chemo-patient-proof” our home; and we shared our unfortunate news with our family and friends so we wouldn’t face this alone.  And the resulting flood of prayers, words of encouragement, meals delivered, and donations toward Vera’s treatment assured us that we have the best support network imaginable, full stop.

Yesterday we received some unexpected news… and I believe it’s safe to say that our team of doctors have never been so happy to be WRONG.  Vera’s tumor is NOT cancer!  Instead, it’s an exceedingly rare benign tumor called myofibroma.  If you’re not scientifically inclined, feel free to skip the next paragraph… But the short story is that this is the BEST possible news we could have gotten from the biopsy results! 

Our team of doctors were divided on whether to do a biopsy of the tumor first, or rather, just take the whole thing out right away.  The idea behind doing a biopsy first was that in the extremely rare case that the tumor was benign, we could hold off on removing it until Vera was older.  Performing a resection of the tumor at this age would mean a massive scar on Vera’s leg (nearly hip to knee) and the potential for loss of healthy muscle tissue.  In hindsight, of course, this was the right decision!  The most important immunohistochemical result from the biopsy is that the tumor tissue was negative for desmin and myogenin.  These are both well-studied markers for rhabdomyosarcoma, and the absence of both is a decisive step in ruling out that particular form of cancer.  Under a microscope, they noted “hyalinized, basophilic, myoid nodules” and “spindle cell proliferation.”  Together with the immunohistochemical finding of smooth muscle actin (SMA-positivity), the pathologists concluded that this is a textbook myofibroma.  In other words, their diagnosis comes with an overwhelming degree of confidence, since despite being an incredibly rare tumor, myofibroma is well-documented to have all of these characteristics.  The other factor that adds to our confidence is that the Sanford pathology department, as well as the Mayo Clinic pathology department, came to the same conclusion.  Myofibromas are often misdiagnosed as sarcomas, so we are fortunate to have gotten this right.  We finally have a definitive answer for what’s in Vera’s leg, and it’s safe to say that this could have been a LOT worse!

Now the question is, as we’ve been hearing on repeat while listening to Taylor Swift on vinyl all week, “Are we out of the woods yet?  Are we in the clear?”  There are a couple quirks of myofibroma to consider, starting with the fact that its rarity is estimated to be about 1 out of 150,000 (we knew you were special, Vera, but you’ve outdone yourself).  That simply means that treatment is done on a case-by-case basis, since there’s no consensus in the medical community.  And despite being a benign tumor, there can be significant concerns about local damage to tissues and organs if it continues to grow in the wrong places.  It is an arguably more complicated diagnosis, depending on many factors to know how it will play out.

Fortunately, Vera’s prognosis is about as good as you can get, since the tumor isn’t located next to any “important” things—just the femur bone and a handful of blood vessels and nerves.  And it would be more concerning if there were multiple tumors (elevating the diagnosis to myofibromatosis), but there’s thankfully only one. That means our treatment plan will be about as conservative as you’ll find among myofibroma patients.

For the next few years, Vera will go in for an ultrasound to monitor the size and location of this tumor over time.  As long as it behaves itself (that is, doesn’t grow too fast or into neighboring tissues too aggressively), nothing else will need to be done.  The hope is that the tumor stops growing, or even better, starts to regress on its own!  If, however, any ultrasound indicates a problem, we’ll need a follow-up MRI to see what’s happening more closely.  If at any point the doctors are concerned about it, it’ll be surgically removed; fortunately, the surgery would be tolerated much better once Vera’s a bit older.  There is also a risk of reoccurrence even after removal, but that’s irrelevant for now.

We’ll still get to know the Sanford Children’s castle pretty well over the coming years, but not nearly as much as we were preparing ourselves for.  Vera will still require some extra health care from the whole ordeal, but not even close to the extent we feared.  Considering the dark place we found ourselves in a week ago…. we feel blessed beyond measure to be where we’re at now.

We want to thank all of you who reached out to us with encouraging words last week, brought us food, prayed for us, donated to our GoFundMe, and all the other ways you offered support and will continue to offer support for our family!  The road ahead is so much brighter than we could have ever imagined, and Vera is blessed to have all of you in her life.  There’s nothing “common” about Vera’s life so far, but that’s to be expected for a girl like her—to adapt our phrase from earlier, dare I say: “Uncommon things happen uncommonly.”

The overwhelming feeling we have now?  Relief, because there are zero upcoming appointments or pending test results on Vera’s chart.

Much love from the Mahowalds,
Jake, Becca, Leland & Vera