Interesting Powerpoint on Sarcomas.
If you don't have time to read all hundred of slides :) You could read slides 6, 102 and 103.
Bottom line:
Sarcomas are rare tumors of the uterus
Adenosarcoma is the rarest type of uterine sarcomas
It seems to be lower grade than carcinosarcoma (eg won't spread as fast which is probably because the carcinosarcoma must have some of the "carcinoma" feature, eg one tumor in one site and spread fast from there)
And a much simpler powerpoint (pretty clear and nicely laid out, with pictures!!)
Although a bit dated
General note for all types of uterine sarcomas:
Limited data
Rapidly growing (doubling time is 4 weeks)
tend to be increasing
"
Specifically for adenosarcomas:
"- both malignant stromal and benign epithelial components
- a significantly increased occurence of this tumor (Seidman et al, 1999)
- present as polypoid masses"
"Because of their rarity, uterine sarcomas are not suitable for screening."
"Surgery is the only curative therapy for uterine sarcomas (2003)"
(with or without adjuvant therapy)
"five year survivals (2001) across all types of uterine sarcomas
Surgery Alone (46%)
Surgery and Radiotherapy (62%)
Surgery and Chemotherapy (43%)
Radiation Alone (8%)"
Surgery + Chemo + Radiation seems to be working better for other types of tumor.
Author notes that there isn't enough sample to really use these numbers (esp across different types, grades, etc...)
"Three-year local recurrent rates
- No adjuvant treatment: 62%
- Whole pelvis external beam radiation therapy: 31%
- Chemotherapy alone: 71%"
But people don't agree. Massachusset General thinks that adjuvant therapy has no impact on recurrence rate, other studies showed that cure rate was similar for those treated with surgery alone or followed by radiation, regardless of the stage of the disease.
"Recurrent Disease:
- Most relapses occur in the pelvis, followed by lung and abdomen
- Currently no standard therapy for patients with recurrent disease"
Only for uterine sarcomas (post-aggressive treatment):
"The 5-year survival rate was 33% for LMS, 38% for uterine MMMT, 57% for ESS, 69% for adenosarcoma and 30% for ovarian MMMT."
http://www.diva-portal.org/liu/abstract.xsql?dbid=4990
But from 1999...I am told that for adenosarcoma, it probably holds since I am told that there was no research, no progress and virtually no additional data between 1974 (year of discovery of adenosarcoma) and now...
And an article about an adenosarcoma survivor
http://www.wweek.com/editorial/3046/5515/
In this case it started in her abdomen, but looks like it's the same type.
and highlights below:
"June: During a follow-up exam, her gynecologist finds a hard mass. A CT scan shows a rare aggressive cancer, called adenosarcoma, in her abdomen spreading to her lungs. She begins chemotherapy in mid-June. Doctors discover her esophagus is bleeding (attributed, in part, to heavy use of anti-inflammatory drugs to combat back pain) and her kidneys are failing (from the chemo)."
"
Now you didn't go through chemo with the breast cancer, just radiation.
With the breast--right. Because the cancer hadn't burst out. But what I have now is a sarcoma, which is very, very different, because it's very aggressive, very invasive. It can spread very easily. And when they discovered it, they thought they took it all out, but then they found out that it had actually grown.
JS: And this is a pretty rare form of cancer, right?
Yeah. You go on the Web, well, there's hardly anything on it. In books, they don't even discuss it. There's maybe 12 cases in the country. There are a couple of clinical trials--very, very small ones. And the prognosis is God-awful. Chemotherapy doesn't help. Radiation doesn't help.
You say the prognosis is awful. How awful?
Some people live two years. Some people die before the two-year period. So it's really not terribly good. The oncologist asked, "What's your goal?" I said my goal is to finish my term. I said to her, 'You keep me alive until Dec. 31."
Brilliant!
Had a really nice weekend. Most of Saturday was spent doing housework and various sailing-related shopping. Ironing, laundry, cleaning up cat mess, mail/bills, reading, etc...I find this routine very soothing. At other times, it would probably annoy me and I would want to expedite it as quickly as possible. Now it's a solid, repetitive set of activities that just looks like normality...Life as usual! I also went out for a run. I hadn't run in a long time (last surgery + IVF cycle kept me off exercise for a little while) so I went out for only 30 minutes but up three hills :) Very sore legs the day after :) but I am definitely going back tonight. This is one of the things that I miss most! (not the sore legs, the exercise!)
Sunday was a double-handed practice on the boat. Well, it was supposed to be a race but we lost the gas cap for the engine and couldn't beat the ebb to go to the start line. It was a lot of fun. Much more active than with a full crew since two people have to do the job of five but so cool! I managed to do a quadruple wrap around three different lines (forestay, topping lift and pole cables) on my second spinnaker gybe. No one got hurt and nothing got broken :) When I am on the boat, I have to pay attention to really short term items, whatever is happening at that time so it takes my mind off any other problems! I find it very relaxing...even though it is physically and mentally demanding! At work, I am constantly tempted to check out some new articles, visit some sarcoma-related message boards, follow a link that some friend might send...So I can't concentrate on much else than sarcoma...
The other problem with work is that I really can't do as much as I used to, and even focusing on top top level priorities does not seem to be enough. On the one hand, it is useful to meet people and I really enjoy doing my job. On the other hand, it is frustrating because I can't really get excited about a project which competes in my mind with other worries. And I see all these cool developments without realistically being a part of them.
***********************
OK...I wanted to continue a dialogue here. I am wondering if there's anyone else who thinks like me or if I am hearing things that no one else can hear. I will still go ahead with the trachelectomy, there really seems to be no downside in doing this and it is the most urgent thing that needs to happen.
It really seems to me that this isn't such a big deal given that it was found early and that the most aggressive tumor has already been taken out. Otherwise I guess that I don't understand why doctors would even think about option #2.
It sounds like all doctors agree that ultimately, I will need a hysterectomy. There also seems to be some sort of consensus around recommendations, in that option #2 may be a viable option, and will allow me to go through a pregnancy.
When challenged with a more aggressive treatment view, Oncologist #2 replied "I am quite willing to be more aggressive but it may not be necessary at this time." It sounds to me as if she was worried that I would do something definitive that is not warranted (yet). She also changed her opinion from "hysterectomy ASAP and probably radiation" to Option #2 after she discussed my case at tumor board.
What am I missing?
Any other oncologist you know who would have a different opinion?
What is your opinion? Am I being over-optimistic?
I have shared all the information I have gathered and gave as accurate a transcript of my conversations with doctors as my memory allows me to.
Here are some pros and cons that I have picked up through research/chats with oncologists.
Let me know if I am missing anything.
Cons
- Sarcomas are tricky to treat because they spread without doctors noticing.
- It's a rare disease and there is no case history so treatment is experimental
- Chemotherapy/radiation is not proven to work (because there isn't enough data to find out what works)
- there is no standard therapy for recurrent disease (eg, officially recurrent adenosarcoma is incurable)
- Mine is listed as "atypical" sarcoma. Not all pathology labs agree on the diagnosis and type of cancer
- Seems that risk cannot be quantified
- Final staging diagnosis/prognosis can only be established after biopsy of the uterus (and removal of the uterus)
Pros
- Out of all the uterine sarcomas I have read about, adenosarcomas have the fairest prognosis. (LMS has a poor prognosis because it starts deeper in the uterine tissues and is closer to the extra-uterine region, and it spreads faster to that area. MMMT are really bad because they have both carcinoma and sarcoma features. ESS high grade spread so fast that chemo can't keep up with it (and human bodies need time to recover from each chemo cycle). Granted, there is little data (some 12 people over 20 years, or some 3 people in Scandinavia) and I also sometimes bump across an article that talks about how aggressive the disease is.
If it was that aggressive, I don't understand why doctors would support the "wait" option
- All doctors I spoke to entertain option #2 (trachelectomy now, pregnancy, then back to option 1 of hysterectomy with maybe adjuvant treatment) with close monitoring so they can manage the disease
- Most sarcomas start as low grade (eg low mitotic count, hehe, this means that the growth rate or number of new cells is lower than for high grade tumors. Low grade tumors have a mitotic count that is already erratic, eg, they fall in the malignant categories because this growth rate is higher than what it should be, but it is not as aggressive as it could be. The grade increases with the size of the tumor as it is an exponential growth curve) so don't grow as fast as for example carcinoma in early stages (apart from some even more abnormal ones)
- Most aggressive part is removed - the cervix where many other cancer cells have been confirmed will be removed on Thursday
- Scans have shown no other visible growth in the uterus/ovaries after polypectomy and main tumor removal.
- If after the removal of the main tumor, this was such an aggressive cancer, I don't think that even the IVF cycle would have been considered.
- Because it is so difficult to detect, a lot of people find out about it only in more advances stages (it could still be a stage I but an already bigger tumor, hence a more aggressive sarcoma). So the dire stats could well apply to these people but not to my case.
- If the margins are not clear after the trachelectomy, there will be no more Option #2
- I did not come up with option 2 in the first place; Oncologist #1 brought it up first as an option. If he had said, hysterectomy and no discussion, then I would have accepted it. So Option 2 is a physician's suggestion!
Here's a friend'ss opinion, substantiated by arguments, which he has agreed to share on this blog.
When I forwarded some positive stats (fair prognosis) from a recent article (2007)
"The numbers are "tentative" because of the large number of relevant factors compared to the sample size. They are not "true" or "untrue".
"This excerpt is clearly taken out of context. They say that their study examines THREE cases. Based on that, you can't call distant metastasis very rare. So either you've taken something out of context or they're stating invalid conclusions. I'm not saying that you've deliberately pulled out a selective quote, but I assume that this is an article abstract or something."
(article available at:
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=17292949&query_hl=1&itool=pubmed_docsum)
When I say that I think that this whole thing is "no biggies"
"Your last sentence is one of a few lately that I am certain ALL of the doctors you have spoken with would disagree with you about. They have used words like "reasonable" which take your own preferences (and their own lack of certainty as to what is going to happen) into account. There's a reason that there would be NO doubt as to the appropriate treatment if you were 50, or even 35 with 3 kids and no desire for more.
You are considering a very serious gamble, and no one knows the odds.
Remember the phrases like "patient understands the risks"? They may not know exactly how high the risks are, but they are NOT saying that the situation is "no big deal".
[...]
"Your doctors are still arguing about exactly which disease it is-doesn't that scare you?"
Debating whether or not, it has spread to the uterus (or started there which I found hard to believe)
"A polyp starts bleeding in your uterus. It is cut out. Some labs identify it as cancerous.
A few months later, you have a definite diagnosis of cancer in your cervix. The type is open to a bit of debate, but the most likely candidate is a cancer which in known medicine has always arisen first in the uterus." (documented as having started in the cervix in only 2% of the uterine adenosarcoma cases...given that there are some 12 cases overall, it's not much :))
On my arguing that there is time because growth rate seems to be pretty low
"Timing is linked to growth rate and metastasis, but we don't know which clock we're syncronized to. I also don't know whether the new seeds start out the least malignant level again or whether they are more dangerous. The very low probability of survival if the cancer recurs even once sounds to me as if the seeds are quite dangerous to miss in the first surgery."
Describing option 2 Vs option 1:
"they want your uterus out ASAP, and are negotiating on whether it gets used once first."
"It's not the growth that scares me. It's the hopping/metastasis, and the fact that it likely already has done so. If they didn't think it was spread around yet, then why would they be negotiating only timing and not whether a hysteresctomy was necessary at all? To beat the recurrence, it sounds like the only way to win is to cut one step ahead of the tumor."
And his last comment on the subject of my positive filtering:
"I'm not saying that option 1 makes the situation "no big deal" from a risk point of view either.
From what I've seen, I believe that there's significnt danger either way, and that option 2 is considerably more dangerous than option 1."
Another friend made a very interesting remark in her e-mail to me. She is also a cancer survivor so this may be something that she's experienced.
She says that she can understand a portion of my agony, and that my desire to have a baby is linked to the standard "human" reasons (birthing experience, genetic perpetuation, biological need, etc...). However, she wonders if by deciding to have a baby, despite all the health issues, I am not trying to feel/exert control over the situation.
She believes that cancer is all about loss of control. Cells behave abnormally. Our body cannot dictate the pace at which they multiply, our body cannot kill them (or more exactly, cancer cells do not commit suicide like well-disciplined cells). Our body is not in control. We are not in control physically. By making decision and by feeling in control of those decisions, we may regain a sense of mastery over the disease, even if it is an illusion.
This friend recommends to surrender to this reality, somehow trust that whatever happens happens for a good reason and believe that I will be made stronger through this experience.
I will ponder over this.
I am still struggling because Option 2 was a physician's suggestion so it appears to be OK to me, as if it was a reality I could accept. In a way, I think that I want to go through the trachelectomy to see if the decision will be made for me. If the margins are not clear, Option 2 disappears and there is no more struggle. If the margins are not clear - which may mean absolutely nothing in the case of a sarcoma, then I will challenge again this approach.
Another chat with the East Coast Oncologist - here are notes from that conversation (third-hand notes so hopefully not too distorted)
Oncologists #2 doesn't agree with the other labs on the cancer type. has come up with an alternate diagnosis.
Here's what this oncologist has to say about them.
peripheral nerve sheath: without a lot more data, it seems unlikely as such a tumor is pure sarcoma. Adeno means that it starts in the glands (endometrium, colon, prostate, lung, etc...). Peripheral nerve sheath tumor would be starting in supporting tissue (mesenchymal) and not be expected to demonstrate adeno characteristics.
spindle cell tumor = this is also sarcoma so same recommendations as with adenosarcoma should hold.
In short, both of these diagnosis are more sarcoma-like than the adenosarcoma. In the adenosarcoma, it's the sarcoma part that's evil. The adeno portion is the benign portion. This oncologist doesn't believe that classification is as important as the fact that there are sarcoma features present. This is what makes it mean! Adenosarcoma is typically hard to diagnose because it is heterogenous/a mix of benign and malignant and has this hopping habit, hence creates perfectly healthy areas. So if pathologists look at an ambiguous sample, they might find different diagnoses and recommended treatments.
If there's any questions about the pathology, best pathology referral agency for someone your age (military age) is the Armed Forces Institute of Pathology (AFIP). They have an absolutely
incredible reference file on people late teens to early 40's. Little doubt in his mind that they're the best in the world. So I could send some new tumor tissue sample to them.
About the "Big Deal" question
Everyone will agree that "this isn't good". Some might be pessimistic, some more optimistic but everyone should agree that it is serious.
He still maintains that in his opinion, the best chance of survival is aggressive surgery. The more variations on the sarcoma theme he hears, the more convinced he is of the aggressive surgery.
About "Why would doctors recommend an unsafe treatment?"
He thinks that doctors can be scared of making the conservative (safe) recommendation and having an angry patient later. When situation is less clear, the doctor is less likely to really come down solidly on one side.
He can't say that six weeks is an incremental risk but he thinks that six months certainly is.
In his case, he prefers to give an initial diagnosis and options when the patient is accompanied by a relative/friend/social worker/etc, because the patient never hears it all. The bigger the group the better. He feels that it is hard for both patient and doctor to be neutral as doctors are influenced by patient's desires which detract them from being objective.
He believes that I am having a substantial influence on my doctors' recommendations. From where he stands (granted far away), he doesn't think there's a whole lot of objectivity going on here.
Also, in his opinion, doctors have signalled to me that they disapprove.
About the trachelectomy approach
Removing the cervix = a good start but not enough (especially after he saw the recommendations and various diagnosis from the tumor boards). It may be possible to preserve the ovaries and physicians should try to do that. Other than that, he would really recommend the big surgery, ASAP. If I were a close relative, we wouldn't be having this discussion (eg, hysterectomy would not be questioned).
He stated that life _WAS_ being jeopardized by delaying surgery, that even with definitive surgery, survival was not guaranteed. There is no doubt in his mind that this is the best shot at survival I will ever get.
He believes that I still have a chance to be cured and survive (past surgery and potential adjuvant therapy, there is no known cure for this type of cancer) and he wants me to have the best chance.
He added that if I wanted to sacrifice some of that chance to have a baby that I might not be around for, that's my prerogative. I should know that a serious risk to my life is involved. It sounds like no one can say what kind of deal, but "no big deal" is definitely not applicable.
Unfortunately, this is a statistics game where there's no way to plot me as a specific point on the graph because of the lack of history. To give me an idea of this "unquantifiable risk", he used another cancer for which there is a lot of data available. With a melanoma (most deadly cancer type), a wait of 2 years would double the chances of being dead (which probably don't start very low in the first place). Sarcoma is worse than most other types of cancer so he's not very optimistic about the number. Adeno would give wiggle room, but sarcoma does not.
When both features (adeno and sarcoma) can be seen, it probably is a relatively primitive cell that turned malignant. Apparently (and there - this is Nat talking - I don't think that I understand the following sentence...If it were in Chinese, it might be easier) Mullerian duct is primitive, undifferentiated tissue that develops into the genital tract, which is why we see the adenosarcoma characteristic.
His Bottom Line:
1) I should never go to one of these appointments alone. There should ALWAYS be a second set of ears.
2) Any kind of stalling is a mistake.
My Next Steps
So, I will still go through the trachelectomy because I believe that there is no downside, potentially upside (negative margins so no more struggle) or more information as more sample.
