We got the results of the liver biopsy back today (about 10 minutes ago). These tests hold the final keys in staging Madelyn's disease and assigning her to a risk category. To catch everyone up, I wanted to review why these tests are so important.
The biology of her disease has had her staged as a 4S. Stages 1-3 are primarily localized tumors (in one place only), so we knew she was a 4 or 4S. The "S" in 4S means special, which is good. For more explanation read the following:
Stage 4: Any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver, skin, and/or other organs, except as defined for stage 4S.
Stage 4S: Localized primary tumor, as defined for stage 1, 2A, or 2B, with dissemination limited to skin, liver, and/or bone marrow (i.e., limited to infants younger than 1 year). Marrow involvement should be minimal (i.e., <10%>. More extensive bone marrow involvement would be considered to be stage 4 disease. The results of the MIBG (metaiodobenzylguanidine) scan, if performed, should be negative for disease in the bone marrow.
She is a stage 4S because
a. She is under 1 year of age
b. The disease has only spread to the liver
c. No bone or bone marrow involvement was observed
(no MIBG scan has been done and will probably not be indicated in Madelyn's case for now)
While being staged as a 4S is great (low risk groups are limited to stages 1, 2A/2B, or 4S), she still could be intermediate or high risk based upon the results of the biopsy.
The three tests that were performed on the tissue were
MYCN Status
Shimada Classification
DNA Ploidy
The values that we could have seen are:
MYCN Status: Amplified (BAD) versus NonAmplified (GOOD)
(If this is amplified, Madelyn is almost definitely high risk)
Shimada Classification: Favorable versus Unfavorable
This is more or less a test of the histology (the study of tissue sectioned as a thin slice).
DNA Ploidy: DNA Index (DI) >1 is favorable, = 1 is unfavorable.
DNA ploidy is basically a test that measures the DNA content within tumor cells. Neuroblastoma cells with increased amounts of DNA are termed hyperdiploid, and in infants, hyperdiploid cells are associated with earlier stages of disease, better response to therapy, and thus a generally better outcome than diploid cells.
For some of you this review has been pointless, but I wanted to make sure all of the information was here to better explain to you the significance of the results we got today.
Dr. Neuberg called today to let us know that:
Shimada Classification (cell histology) is FAVORABLE. Good news!
DNA Ploidy is 2.08. Anything greater than 1 is GOOD. And finally....
MYCN Status (we've been dreading this) is NOT AMPLIFIED!!!! FANTASTIC NEWS!!!!
(the primary source for this information can be found here in table 1)
All of our hopes and prayers so far have been answered. All 3 tests came back with the results we prayed we would get.
The plan going forward is to do a physical exam every three weeks (starting January 8th at 1:30PM) and an MRI every six weeks (around the beginning of February). This also means no chemotherapy or radiation at this time!!! The reason he wants to watch her so closely is because the tumor in her chest is close to the spinal cord and right now she can't tell us if she has issues due to that (numbness, motor problems, difficulty breathing, etc.) The good news is that she rolled over last night for the first time, so she's developing well.
Please check back because I have to run. I'll check and make sure I left nothing out, but to sum this posting up, we got some great news today and we thank you for all of your prayers.
We love you all,
The Bell Family
