Sarcoma Treatments

Sarcoma - Metastases to the Heart

2010-10-30T19:10:00.000-07:00

Although heart metastases are identified in 25% of people who die of cancer, detection and diagnoses of heart metastases in living patients are surprisingly low (1% of pediatric sarcoma patients). We wanted to share this information because although most cancer patients feel as if they are having frequent tests and imaging to look for new signs of cancer, cardiac metastases are commonly missed when standard studies only include conventional CT, MRI, and PET scans.

Tumors in the heart can come from neighboring lung metastases that travel up through the lung vessels in to the heart, direct extension from metastases in the lung, or from mets that arrive from the bloodstream.

If you have a met to the heart, you want to have it surgically removed as soon as safely possible because it can become deadly whether your cancer responds well to treatment or not. If the tumor invades the heart, then it can cause the heart to stop pumping well; but other causes of mischief include obstructing blood flow into or out of the heart, or breaking off and forming metastases to other parts of the body like the brain.

We wanted to post about heart metastases because we found out about them only because our dd was on a Phase I / II trial that required an ECHO. The mass was seen on ECHO - and although it was about the size of a golf ball, it didn't block any of the valves - and there were no changes in EKG or abnormal sounds that the cardiologist could hear. The important point is your doctor should have a high index of suspicion to find these - and the screening study is likely to be an ECHO. We had been watching our dd's lung mets - and thought these were still fairly far (1-2 cm) from the main pulmonary vessels by chest CT - but when we didn't know is that they can sneak into small pulmonary veins that aren't well seen on chest CTs (the resolution is too small) and travel into the heart. On Chest CT the heart is bright white - so intracardiac tumors can't be seen.

Recently we heard that young woman with cardiac tumors seemed to be responding to a promising clinical trials medication, but she unexpectedly died. The problem with medical treatments and cardiac tumors is that even when a cancer responds to treatment, a tumor in the heart can be just as deadly.

Our dd had open heart surgery a few weeks ago and the tumor was removed. The surgery was surprisingly quick (13 minutes on cardiac bypass) and her recovery was quick too (discharged on the 3rd day of after the operation). She was able to be back at school in less than 2 weeks and required no pain medication by then. Her recovery was much quicker than when she had a thoracotomy. As tough as it is to face difficult news such as a heart metastasis, the earlier diagnosis the better - and surgery can be effective.

Metastases to the heart
Management of intraatrial lung tumors
Cardiac metastases in soft tissue sarcomas

Surviving the Financial Costs of a Rare Cancer - Sarcoma

2010-09-16T08:58:00.000-07:00

I thought I'd write a post to share tips.

Everyone knows this disease is expensive. Here are tips we've used to deal with insurance companies and try to avoid going bankrupt over this cancer.

1.Case Manager.Get an insurance case manager. They're free and they'll help expedite insurance appeals, getting approval etc. if you need one. If you've been overbilled, you can also call 1 person every time and not have to start over with "I have this rare cancer.."

2.Insurance Appeals If you've been denied for a medication or procedure, you call them. Insurance companies usually give in on an appeal (I've heard 75%) - you just have to do it. They don't want you to die. They don't want to be sued either.

3. Ask to Pay in Installments Almost every hospital will agree to let you pay off your bill in installments. We pay some off over 6 months - and other over one year (we have a high deductible plan). Sometimes you need to ask to have all the bills combined into one installment plan (e.g. if the doctors, radiologists, etc. are on different payment / billings systems). If you have a very high fee from a doctor who is out of network, call their office and ask if they're willing to accept a lower fee. Many are.

4.Fundraisers When a big event is coming up, think about a fundraiser and think about opening a charity account. People can donate into your account - it just makes it easier for them to do it. We set ours up at Bank of America. Because we give talks (learning issues, dyslexia), sometimes we waive our fee and donate speakers fees or products fees to our daughter's health fund. The advantage is it goes to the health bills and it is not taxed as income (it adds up).

5. Medical Tax Deduction - Used to be if medical bills add up to 7.5% of your income, this year it's 10% of your income (boo) - you can take this as a tax deduction. Do it! The costs really add up. Look at all that are allowable. This is the IRS page for 2009: http://www.irs.gov/publications/p502/ar02.html
We add up mileage driving to doctors appointments (this may be airfare), up to $100 per night when away for medical treatment. Driving to the pharmacy, etc. Every year the reimbursement rate per mile for medical changes, so you have to look up how much you can deduct. Save all the costs and itemize in case you're ever audited. I keep track of everything with a very iPhone App - iExpense it and FYI Mileage. I keep track of both business expenses and medical expenses. I love these apps - it allows you to take a picture of the receipt and have the report emailed to you - so you don't have to keep everything in a shoebox.

6. Self-Employed We are self-employed which can be another killer for medical costs - but we tried to help by buying our insurance through a broker who combines small groups like ours to get reasonable insurance rates through companies like Blue Cross. That's really helped. Another big tip I discovered only a few months ago was MERP and switching to a C corporation. We had been an S corporation, but switching to a C corporation helps out a lot. Because we have only 2 full time employees (my husband and I), we created a MERP (Medical Expense Reimbursement Plan) that covered full time employees. As a result, now medical and dental expenses not covered by insurance (like copays, deductibles, over-the-counter symptom medications etc) can be picked up by our business. The advantage to us is that the money we spend on medical is not part of our income tax. Not everything is deductible, so read the tax info carefully and consult with a CPA if necessary. We borrowed a Nolo book from the library - Home Business Tax Deductions - it's great.

Hope this helps. If anything else has tips on this, please share!!! Fernette

CureASPS.org - Resources and Discussion Forum for Alveolar Soft Part Sarcoma

2010-08-17T18:27:00.000-07:00

CureASPS.org is the definite site for information about Alveolar Soft Part Sarcoma on the Web. Look their library of resources for recent articles about new clinical trials drugs and new surgical and minimally invasive treatments for this cancer.

Alveolar soft part sarcoma is an early metastasizing cancer that affects mostly children and young adults. No definite cure is known, but many promising therapies have been discovered and some 15+ year survivors are members of the CureASPS.org community.

Additional Minimally-Invasive Options for Sarcoma - Cryoablation and RFA

2010-05-22T08:12:00.000-07:00

Another quick update for those of you with metastatic sarcoma.

Our daughter is 2 years out from laser surgery on on the right, and 1 year out from laser surgery on the left. Some small nodules have appeared in the intervening times - this is a difficult aspect of determining the timing of metastasectomy and probably why so many people have to have repeated lung surgeries. Because metastasectomies seem to slow the progress of disease there is always a difficulty determining when to operate - you want to operate to slow the disease, but you don't want to operate too early because new mets appear and then you have to operate again. A second surgery is always more difficult than the first - because of scarring from the previous surgery that may make the procedure longer (need to release the adhesions) and also the greater difficulty feeling for the mets. Usually the mets will have to be larger for a second surgery, but if you wait too long, the mets may also become inoperable.

We are currently considering cryoablation at least on one side. There was a small nodule missed on our daughter's surgery on the left, and that nodule has been slowly increasing over the past year. The main danger of these tumors is if they press on a major airway. Also it adds to the overall tumor burden which may make it more difficult for patients to respond to medications. One of the world's experts is Dr. Peter Littrup: http://www.karmanos.org/app.asp?id=1117

The best candidates for cryoablation are those with fewer rather than many lung mets. It is best for the mets to be smaller than 3 cm for a complete ablation, although potentially if something regrows, it can be ablated again. We were told the reason multiple tumors aren't done is that when the lung recovers from the ablation, revascularization in the area could make the other tumors grow more quickly. It seems that it makes more sense that metastatsectomy comes first to reduce the numbers of multiple tumors, and then for additional tumors that either got missed or appear, they can be removed by cryoablation. At least one sarcoma doc told us that because lung blood vessels are terminal - they don't tend to form distal metastases...that means they are unlikely to 'break off' and go to other parts of the body.

Usually these procedures are covered by insurance, but they may require pre-authorization. Radiofrequency ablation or RFA can also be performed for lung, but from what I've read, it can be harder to perform because the burn zone can't be easily visualized by CT (by cryoablation you can see the 'ice ball') and the great vessels can pull away the heat. RFA is a great option for liver mets though (http://www.medicinenet.com/radiofrequency_ablation/article.htm).

Whether a met or group of mets can be treated by cryoablation completely depends upon the appearance of the scan (the doctor will look at size and location etc.), so scans need to be sent to the doctor who is considering ablation. Usually the procedure takes about an hour or less, no ICU time afterward, small incision, and home the next day. No flying for 1 week due to the risk of pneumothorax. The most common complication is a pneumothorax, especially if a patient hasn't had a prior surgery before.

Hope that helps!

Molecular Profiling to Select Promising Cancer Drugs

2009-05-19T10:21:00.000-07:00

If you or your loved one is dealing with stage IV or a metastatic rare cancer like sarcoma, one of the rudest shocks is discovering that your doctor may have no specific recommendations for systemic / chemotherapy. The reason for this is several-fold, among them - the rareness of the cancer (large scale studies may be impossible), the heterogeneity or molecular variability among different patients and even among different tumors within a single patient. As a result - cancer patients must face the possibility that they could take a toxic medication that not only could not have any benefit, but that even could make the cancer worse.

Not surprisingly, with all the recent advances in molecular biology, some researchers and clinicians want this to change. The result are options such as molecular profiling - recently proven to show some benefit on the outcomes of refractory metastatic cancer patients (Molecular Profiling Improves Cancer Treatment. The science is very cutting-edge though, and while many oncologists may feel the technology is not well-studied enough to be used in the clinic on a regular basis, if patient has a rare cancer that has metastasized, profiling may be a powerful weapon in the arsenal to fight the cancer.

In our daughter's case, we found that molecular profiling helped us select "more helpful" and "less helpful" options for investigational agents (there were no known curative agents available), information about how long to stay on an agent, and information about whether the current regimen was beneficial.

There are many different laboratories now offering molecular profiling of cancer tissues, but we can only share our experience. We had the benefit of a charitable organization helping pay for costs that were not covered by insurance. Currently I have heard that one of the labs we used (Clarient) has been covered under traditional health insurance plans. We requested most of the tests on the Immunohistochemistry form off this page). Because of the rareness of our daughter's cancer, we asked all of her doctors (in 3 states) for their opinions, as well as a friend active in biotechnology - and

Because of what was known about the particular sarcoma we were dealing with, we also sent for some outside tests through Targeted Molecular Diagnostics. Our tests from Clarient included: TS, Ki67, Cox 2, PTEN, VEGF, Tau, ERCC1, PDGF alpha and beta, GSTpi, myc, topoII-alpha. From TMD, we were able to check for K-ras, c-met, and IGFR. In addition, we looked at some phosphorylated receptors because we knew we wanted to test the tissue before going to surgery and we were able to have tissue fixed in TMD's Phosphoguard. This may not be necessary, but if results are conflicting, having the phospho-receptor data can be helpful.

Our Experience with Laser Lung Resection of Sarcoma Metastases in Germany (Dr. Rolle)

2009-03-15T11:40:00.001-07:00

For certain solid tumors, lung metastases are the main sites for tumor spread. If the primary or first tumor is removed, then long term or even complete remissions can occur if all of visible metastases are completely resected.

The lung is a common site of tumor spread for many cancers including many sarcomas, breast cancer, lung cancer, and colon cancer. If tumor growth in the lungs proceeds unchecked, it can cause death by overwhelming the lung, blocking main airways (bronchi), or invading vital structures (heart, great vessels leading into the heart).

Conventional resection of lung metastases can remove metastases, but only if not all lobes are involved, and even then - only if the surgery would not involve so much lung removal that it would be too debilitating. Conventional surgery removes whole lobes and segments for a single metastasis...so the number of people who qualify for conventional metastasectomy are even fewer than those who qualify for laser resection.

A very detailed website of the laser resection process in Germany is available here:(http://www.geocities.com/laserlungsurgery/). Dr. Rolle is an awesome human being and world class surgeon. We are very thankful he could be our doctor.

We have also added a few details based on our experiences below:

1. Can my lung metastases be removed by laser?
The first step is to send a brief history and copy / CD of a recent chest CT to Dr Rolle at:

Dr. Axel Rolle, phone 0049 352365102
Fackkrankenhaus Coswig GMbH, Neucoswiger StraBe 21, Coswig / Dresden D-01640 Germany.

We have heard that Dr. Rolle accepts only 10% or so of patients because of various reasons. Possible reasons may include widely disseminated (involvement beyond the lung) or too extensive disease (not completely resectable), unresected primary tumor, or frailty of the patient.

2. When will I hear from Dr. Rolle? After fedexing scans, we heard from Dr. Rolle within 1-2 weeks by email. Once accepted, you may proceed to surgery quickly (e.g. "Come to Germany this weekend").

3. How will I get to Germany? If you are coming from the US, you will need a passport. If you need a passport quickly, there is a process for getting a passport in 24 hours. Look for discount airlines. From Vancouver Canada, Condor Air (discount airlines associated with Lufthansa) was recommended to us. We found cheapoair.com to be more convenient from Seattle. They are a travel agency and were sometimes $1000 cheaper than published fares. We flew on Lufthansa and United. Both had changeable airfares (with penalty) if we could not make our return flights because of delays in the hospital. For our daughter's first surgery, our departures were delayed by one week because she had exercise-induced asthma diagnosed and her lung condition needed to be optimized before surgery.

4. Paying for the surgery. Frau Conni Meissner from Administration contacted by email and told us that surgery needed to be prepaid (11,000 Euros) before admission. This was arranged via a transfer through our bank. About one week after the surgery, expect another email from Ms. Meissner that tells you what additional money you will need to transfer. If the side has not been operated on before, it may be about 2500 euros. If it has been operated on before (even non-laser), it may be closer to 5000 euros because of the increased complexity of the surgery.

5. Arrival in Coswig. We took a flight with a stopover in Frankfurt, then arrival at the Dresden airport. Print out the address of the hotel where you will be staying and catch a cab after you have collected your bags. We stayed at Alt Coswiger Hof because it could be booked online in advance and we would not have to go looking for a place to stay at night. There is a little light that come on once you get close to the door. If you come after hours, there is a bell to ring and Mr. or Mrs. Striegler will come down to let you in. If you arrive on a Sunday, we aware that many stores will be closed. At Alt Coswiger Hof a good breakfast is included with the accommodations. Also there is a bakery right next door.

6. Marketing, Banking in Coswig. Electronic Adapters for Germany. Internet Access. In Coswig, we often went to Kaufland (supermarket) for bottled water and snacks. If we really didn't like the meal, we could always make a peanut butter and jelly sandwich. Their strawberry jam seemed better than ours in the States. They also have misc. items such as batteries and housewares. There is an ATM machine at Kaufland.

Our DD took her Nintendo DS and this definitely helped occupy her time. Our friends from Sarcoma Alliance also gifted her with a wonderful scrapbook kit and she worked on making a beautiful bookmark when she was on the mend.

There is also a bank in downtown Coswig near the tram tracks (e.g. we forgot to bring our ATM card found ourselves short of cash because the hotel's credit card machine wasn't working!), but the bank may not be willing to give you an advance without a pin. Thankfully, we were able to obtain a small advance from Conni Meissner.

During the day there are food wagons that sell Chinese food, roasted chicken, etc. if you do not like hospital offerings. There is also a reasonable good Chinese restaurant (Take out OK) located 25 Johannesstrasse called Tsing Tao Haus.

If you have some advance notice and like electronic gadgets (like a laptop with DVD player), buy a electric converter from Amazon (SIMRAN plu adapter). There is no Wifi in Coswig (and no free Wifi at German airports). There is one computer on the groundfloor of the hospital that provides Internet Access (2 Euros per hour). Friends we made through Sarcoma Alliance were much more tech-saavy...they managed to get 2 bars of Wifi in their hospital room using a Satellite card.

7. Admissions. What to Expect Day 1. When you first arrive go to the front desk reception and they can hold your luggage while you wait to meet with Frau Korbel from Admissions. Many of the forms will not have been translated into English. She will ask you whether you want options like the staff anesthesiologist or the chief of Anesthesiology (pay more). The front desk can hold your bags while you speak with her. You will go up to C1 where you will find a small closet and lock box (will fit a laptop).

If you go with a family member, Frau Meissner may be able to arrange for your husband / wife etc. to stay in the hospital pension (top floor of the Administration building). If the patient is a child, one parent can stay in the hospital room or even in the ICU after surgery.

If available, a nurse that speaks English will ask you for your menu preferences. The large meal in Germany is lunch. If you cannot figure out what to order, you can order pizza, hamburger, or chicken salad for the lunchtime meal. We often ordered Brotchen, ham, and cheese for the morning and evening meal. Our daughter did not like fizzy mineral water, so we ordered Oppacher Still Water - and this was the best bottled water like we are used to in the US. If you are able, after you are finished eating, return the tray to the cart by the nurses station. This is another task that in the US is done by the nutrition service - in Germany there is a lot more you are expected to do-yourself. They also collect empty bottles afterwards for recycling - so put empty water bottles in the crates in the TeeKuchen.

The first day usually involves lung function testing, possible bicycle stress test with EKG, and brief meeting with a PT (maybe Sebastian who speaks excellent English) who encourages you to practice with the spirometer every hour and with the breathing machine in the hall (you receive your own connector tube package). The techs in the stress lab unfortunately don't speak much English. If you are female, you may be disconcerted that they do not provide dressing gowns for the stress test - you are expected to bicycle at maximal power without a top on (this is not the usual US way). If you have a blood gas, they may may wipe your ear with something that makes it hurt and feels as if it is burning. If you are a young person, you may not need this - it is especially for older people who may not have good blood circulation in their ears. We had them try it without the creme for our daughter and it was just fine. Other activities - surgeons will come by, internal medicine doctor, chest xray, and possibly you will be told to go to Gymnastik class (there is a preop and postop class) and inhalation therapy (e.g. for asthma inhalers). The class will be conducted in German, but just follow along.

Often you will be told where and when you should go for tests or classes and then you just show up at the right place and time (different from US hospitals where wheelchair transport is required to go anywhere). You are also given a thermometer that you are to use to take your own temperature. If you don't speak German, it is confusing at first - but you will catch on.

The nurses on C1 don't seem to do as many medical things as in US hospitals (e.g. they will offer you tea) - it is more like a rehab hospital. The floors are much quieter than US hospital wards because there are no constantly beeping IV machines and taking blood pressure every 4 hours, and patients are encouraged to come and go when they are not scheduled to be in their rooms (to get exercise). In the summer months, they encouraged us to go outside to the parks on hospital grounds, walk, and breathe the "fresh air". Very different from US urban hospitals!

There is a button in your room by the door that controls the blinds outside. At times custodians or nurses? will come in and dust or sweep. They are very clean there.

You can keep small items in a refrigerator in the Teekuchen (Tea Kitchen). Also there is coffee, hot water, mik, sugar, tea bags, crackers, etc. in there for your use.

The second day before surgery, expect a blood draw in the morning, collect a urine sample, and preop instruction (shaving, given surgical hose, etc.) from the nurse. You are not to eat after lunchtime. They will give a soup for dinner. Afterwards you will have a tap water enema.

You will also meet with the anesthesiologist.

8. Surgery Day: I was allowed to accompany my daughter into the pre-surgical area (change into gown etc.). She had received a sedative pill, received something more from her IV, and then sat up to receive her epidural. It was a bit difficult, so too a while, but I was with her until she fell asleep.

We waited in the general waiting on C1 and they were kind enough to let us be in the operating room when she woke up (she had been disoriented other times waking up from anesthesia). After waking up, she was moved to the ICU (very close - almost an adjacent room) where she was connected to a sliding wall set-up that managed her various infusions. Remarkably she had no pain immediately post-op, but she was still very sleepy and puffy because of the fluids received during surgery. She slept a lot.

We tried to keep track of some of the medications she was on - not always easy because there are some differences in the drugs in the US vs. Germany. K's epidural delivered Naropin or Ropivacaine (great stuff). She also received Keatril or gransitron for nausea, sutanol or salbutamol which was albuterol, Dynastat or Parecoxib (an IV Cox2 inhibitor for 2-3 days post op), Dipidolor (opiate), and Paracetomol which is our Tylenol. For her first surgery they gave her Targin which is a formulation that combines oxycodone with naloxone..supposed to be less constipating, but this really wasn't as good a medication for K...she preferred plain Oxycodone...and we brought from Colace / Docusate from home (now you can buy over-the-counter).

9. Recovering: The first few days are spent in the ICU. There are many nurses that speak English in the ICU, and our anesthesiologist was Dr. KraBler who was chief of service (you may extra if you want the head of the dept) who was mainly responsible for us there. Post-op day (POD) 1, you begin with the physiotherapists...easy things first (moving hands, arms, legs), then over the next few days it will progress to sitting on the side of the bed, then standing, using the commode, and lastly walk to the bathroom. Your appetite might take a while to come back.

The most uncomfortable thing in the first few days is the chest tube. Our daughter said it wasn't really pain, but felt like a cramp in her neck. The only thing that makes it better is their pulling back the tube, or even better - removing it entirely. They will be able to remove that first one usually within the first 2 days...but depends how much it is draining and perhaps what the chest XR shows.

We did tell our DD to put her best face forward when the attendings come around...even if you have been walking long distances and doing a decathalon, if you happen to look tired or sleepy when the attending comes around, they may be reluctant to

K was able to walk to the ICU nurses station by POD 2 or 3...main difficulty was feeling lightheaded.

10. Back to the Floor: Our daughter was able to move to back to C1 on POD 3. She went back with just her IV (antibiotics), 1 chest tube (and a wheeled gizmo that we nicknamed Fido), and epidural catheter. The second chest tube came out on day 5 or 6. And epidural catheter shortly after that.

The rest of your time will be spent working with the spirometer to expand your lungs, going to the post-op physiotherapy classes, and trying to walk more. If you are crossing the Atlantic ocean, it is not recommended that you leave any sooner than 3 weeks after surgery. The flight is long and because it is an airplane, there will be less oxygen at the higher altitude. By 2 weeks postop, you may feel pretty good though, and K was able to take fairly long walks around the full perimeter of the hospital.

That's it! Soon you will be home. We were able to arrange for the pathology slides to be sent to UCLA for additional reading as well as we had molecular profiling done. It may be helpful to make sure you get the pathology report if you have been on some therapy before this (look for necrotic response).

We found the jet lag much worse going back home, but our daughter was essentially be able to go back to school the next week. She was able able to make a low key tour of Dresden (took the train) the last day she was there.

Surgery and Laser Resection for Sarcoma Lung Metastases

2009-03-03T07:28:00.000-08:00

Because sarcoma preferentially spread to the lungs, surgical resection of lung metastases can be curative or significant extend life (years) for sarcoma patients. The most common options facing patients are VATS, open thoracotomy (surgical wedge resection), and radiofrequency ablation. The gold standard is open thoracotomy in terms of try to remove all gross disease. If resections are complete, there is the possibility of long term (decades) remission or "NED" (no evidence of disease). But there are significant risks and irreversible consequences of conventional thoracotomy, and in addition, many more patients will be told they are not operative candidates at all because there are too many lung nodules, there are central lung nodules (deep in the lung lobe) and / or they involve all lobes.

We wanted to post about the option for laser resection of sarcoma lung metastases because this approach offers hope to patients who are thought to be unresectable by conventional surgery, and less disabling surgery for those who are contemplating conventional thoracotomy.

The lung metastases in sarcoma are a common cause of death. Death can be caused by a number of factors -but most often due to cumulative tumor burden on the lungs, or compression of main airways or bronchi, or less commonly invasion of the heart or great vessels. The clinical rationale for removing lung metastases is not only the removal of tumors that may invade critical structures, but also there seems to be a therapeutic effect of thoracotomy on sarcomas that might be an immune response to the actual surgery itself.

The survival statistics for redo thoracotomy are pretty impressive (see below), although a surgeon friend of our said to be wary of "sampling error" - the more healthy people may be candidates for redo thoracotomy.

See entire Powerpoint presentation here. There are many other supporting studies that support repeated resection or metastasectomy when the primary soft tissue sarcoma is removed and the lungs are the only known site of metastases, but for instance in our daughter's rare alveolar soft part sarcoma, Judson's group reported that of 9 patients who underwent metastectomy, 7 achieved complete clearance and 44% were disease-free at last following (followup ranging from 2-22 years).

The purpose of this blog is to share what information and experience we have gained in our daughter's battle with metastatic sarcoma. Patients and their families should research promising therapies and investigational drugs because great progress is being made into the biology of these rare cancers and no single doctor will be able to keep up with all the research advances.

Recently we have been contacted by numbers of patients seeking more information about our experiences with laser resection in Germany with Dr. Rolle. In order to make it easier for others to learn from our personal experiences and sharing of the literature, we decided to post the information to a blog. We realize this may be a big step for you to take, and if you have any questions, please feel and contact by email or through the comments on this blog.