We're done!

I'll start with an understatement: this has been a very good week.

On Wednesday morning, after conferring with the sarcoma group at Dana Farber, my medical oncologist at Hopkins gave us some great news. Just to make sure that nothing was going to change (we were a bit wary...), we've kept quiet for a couple of days.

Based on my clinical course and examination of my tumor specimen, I've been given a diagnosis of angiomatoid malignant fibrous histiocytoma. Despite the long and frightening name, this is REALLY good. The bottom line: I'm likely to do very well (i.e., the cancer is not likely to come back) and I don't need adjuvant chemotherapy.

I assume you might have a question or two...

What is an angiomatoid malignant fibrous histiocytoma?
As we're getting used to these days, angiomatoid malignant fibrous histiocytoma is a rare cancer. It was first described in 1979 and, originally, was thought to be a type of malignant fibrous histiocytoma (often abbreviated MFH). MFH is a type of sarcoma (i.e., cancer of connective and supportive tissue) that is typically seen in older adults and has a significant risk of local and distant recurrence.

Since 1979, several studies have found that angiomatoid MFH tends to behave very differently than a more typical MFH. For example, it's seen in young patients and is most likely to be located in the skin. Importantly for us, it has a far better prognosis than MFH. For this reason, some pathologists place the term malignant in quotes and some have dropped it all together.

Is this still cancer?
Yes - absolutely. This tumor has come back in some patients, both locally and/or distantly. However, compared with my previous diagnosis (Ewing's family sarcoma), the chance of this is low. It still could happen, and we'll be watching closely in case it does.

Why don't I need adjuvant chemotherapy?
Chemotherapy can have side effects, both during treatment (e.g., severe infections due to a weakened immune system) and later in life (e.g., heart, liver, or kidney damage). If the risk of cancer coming back is higher than the risk of these side effects, then - generally speaking - it's worth receiving chemotherapy. However, if the risk of cancer coming back is smaller than these risks, then it's not a good idea. I now fall into the latter group.

What kind of follow up will I have?
For the first two years, I'll have a clinical exam, CT scan of my lungs, and an MRI of my left hip/butt once every three months. Then, until five years, I'll do this every six months. Finally, until ten years from now, we'll repeat once a year.

What's next?
My Hickman catheter comes out Friday (good riddance), I'll keep shaving my head (we've grown to like it), and I get to start changing diapers again (yes, even cancer had it's perks). Especially now, we are so very appreciative of everyone's support and prayers - it's been truly overwhelming. Thank you.

What's Next?

Now that surgery is done, we need to decide what treatments to pursue next. As all of the detectable cancer has been removed, further chemotherapy is termed "adjuvant" therapy (i.e., treatment intended to reduce the risk of relapse). This decision is heavily influence by the pathology results from my surgery.

The first issue for the pathologists was to determine the adequacy of the resection. They examined the tumor and found that the radial skin margins were very good (i.e., the tumor was no where close to the cut skin edges). The deep margin was closer, but still good. Importantly, the tumor did not appear to involve the fascia covering the gluteous muscle, which is thought to act as a strong anatomic boundary against the local spread of cancer cells.

The next issue was to determine how well the chemotherapy worked at killing tumor cells. This was a bit less clear. When comparing my pre-chemotherapy MRI to my pre-surgery MRI, the tumor volume had reduced by at least 50% (i.e., it was half as big as it used to be). This would suggest that the chemotherapy did a pretty good job of killing the cancer cells. However, when the pathologists looked at the tumor under the microscope, they saw very few dead cells mixed in with the living tumor cells. This suggests that the chemotherapy was not working very well. There was not a clear consensus among my oncologists as to how these two facts should be reconciled.

So, where do we go from here? Should I get more chemotherapy in case any cells were left behind (locally or elsewhere)? Could I just be done with treatment now? There are three options:

• Continue with the same chemotherapy for another 9 months. The fact that my tumor got smaller suggests that the chemotherapy was working, so we could continue with the same drugs. But, there is concern given that very few dead cells were seen under the microscope.
• Continue with chemotherapy, but use different agents. If we don't think that my current chemotherapy is very effective at killing this tumor, we could always use different agents (there are other chemotherapy combinations that, in general, are known to work well with this kind of tumor). However, as my tumor is now gone, we won't be able to tell if the new chemotherapy is effective or not.
  
• Don't do any more chemotherapy. There are case series which suggest that patients with Ewing's family sarcomas of the skin do very well and have a low chance of cancer recurrence. Therefore, it's possible that I don't need any more chemotherapy. However, as this kind of cancer is rarely seen in the skin, these reports involve only a small numbers of patients (around five to fifteen). So, it's hard to know if this information is reliable and generalizable to me.
  

My cancer doctors met last week and, while there was some inclination to recommend the second option (more chemotherapy, but with different agents), there was indecision. Therefore, the group recommended that I get another, independent evaluation. As some of the sarcoma doctors at MD Anderson in Houston already know about my case, we decided that it would be best to see the sarcoma group at the Dana Farber Cancer Institute in Boston. We have an appointment to be seen on July 16th.

There's no great rush to start more chemotherapy immediately; it will be good to step back and have a fresh look at where we are. As always, we are so grateful for everyone's continuing prayers and support.

Surgery

We're very happy to report that I'm now 3 weeks post-surgery and feeling great!

My operation was on June 14th and took around 2 hours. The procedure started with my oncologic surgeon removing my tumor, some surrounding skin, the underlying fat, the underlying fascia (a fibrous covering over muscle), and a thin rind of gluteous muscle. All in all, I ended up with about a 4 inch diameter "hole" over my left hip/butt. Then, my plastic surgeon rotated a flap of skin from next to the resection site to cover the defect. The technique is called a "rhomboid flap" and is explained here (with not-too-graphic pictures).

I had general anesthesia for the procedure, so I was asleep and intubated (i.e., using a breathing tube) for the whole thing. In fact, as I received a couple milligrams of Versed in the preop area, I have no recollection of ever being in the operating room at all. I "woke up" in the recovery area with a sore throat and 37 stitches in my back side.

I was in the post-op recovery area for a couple hours and did need some narcotic pain medicine. However, from then on, I was surprised at how little pain I had; I only needed Tylenol for a couple days, then nothing. I stayed on the inpatient surgery floor Monday and Tuesday night, mainly for observation (i.e., to make sure the surgery site was not bleeding and healing properly), and then went home Wednesday morning.

I was up and walking the evening of my surgery. However, I was instructed not to flex my left hip or sit down for one week, as this would put tension on the sutures. To help "remind" me not to bend the hip, my surgeons put me in a knee brace. Interestingly, if you can't bend your knee, you tend not to flex your hip (unless you're a Rockette...). So, I spent the week at home either standing, reclined on my right side, or lying on my belly. This got old very quickly.

Once I was out of the knee brace, things were pretty much back to normal. I gradually increased my hip flexion and now have enough range-of-motion for walking and sitting, although the skin is still a bit tight (it may take several months for this to go back to normal).

Who would have thought I'd already have my first butt-lift at age 33??