My Long Journey with LMS & ESS
My story started 26 ½ years ago (June 24, 1981) when I had a hysterectomy to remove a fibroid the size of a 5-month pregnancy (12 cm.) after a year of heavy bleeding. The gyn and I were both surprised it was cancer. He told me I was cured. Sure….I went on my merry way never getting chest X-rays or CT scans, never getting checked out except for pelvic exams and pap smears every year. Eleven years later, I had to have bilateral breast surgery and during pre-op tests they discovered 5 nodes in my lungs. I had the breast surgery and 6 weeks later I found myself in MSKCC having lung surgery. (I had breast surgery 3 more times for lobular carcinoma in situ.) The pulmonologist I was sent to ordered a lung biopsy. He told me after the biopsy to go home and pray for the best and expect the worst. He did not offer me any hope. They did not get enough cells to make a diagnosis. They wanted me to have another biopsy but I was afraid that cells would spread from taking it again and I refused.
My breast surgeon recommended that I go to Memorial Sloan-Kettering Cancer Center in NYC. I did and the thoracic surgeon knew immediately it was metastases from the uterus. I had a definite diagnosis of myxoid LMS, low grade. No one made a fuss over it being LMS. I did not know it was so rare and hard to treat. I asked questions; no one knew much so many years ago. I read books and all I found out was that .01% of women with uterine cancer get LMS. My thoracic surgeon did both lungs at the same time with a bilateral clamshell thoracotomy, cutting down from the armpit under the breasts and across the chest up the other armpit. The spindle cells that were in my lungs hung down like gelatinous icicles. Almost 3 years later, I had to have the same operation for 4 more nodules in both lungs again. This is a very painful surgery because it hurts to breathe in the beginning and the sternum hurts from being sawed open. The narcotic drugs hyped me up so badly, I never slept and couldn’t rest in bed. The second time, I asked to see someone who could help and an Anesthesiologist from the Pain Clinic was sent to see me. He suggested an epidural and a PCA pump (personal computerized anesthesia pump). When the pain gets bad, you push a button for more of the drug but it is programmed so you don’t overdose. I don’t know why I wasn’t told of the Pain Management Clinic the first time so I wouldn’t have suffered the way I did.
I have never had chemo or radiation. Low grade LMS would not respond to chemo. I was very lucky that I could be a candidate for surgery. I have other health problems that do not allow me options like some people. I am lucky to be alive. I say it is luck because I have been neglected and not had scans, and I am still here. I had another node that grew back 4 months after my second lung surgery. How? No answer but: “It happens.” The nodules had not changed and the surgeon said it is too difficult to keep opening up a person to get to the lungs, so we waited and watched with scans. My last lung surgery was 11 years ago and the first was 14 years ago. The tension, surgical trauma and arthritis that has set in from the upper body operations after all these years is finally getting to me. I have a lot of nerve pain in the lung area whenever I use my arms too much. After 10 surgeries I am not as active as I would like to be. In my last operation my obturator nerve had to be cut and a lymph node taken out to get clear margins. This left me with a long period in which I was not able to walk well, another obstacle to deal with during recovery.
Dealing with my other health problems is a challenge too. I have an autoimmune condition called Celiac Disease. It requires a special diet. If I don’t stay on it, it could cause an intestinal lymphoma over a long period of time. Celiac Disease damages or destroys the lining of the intestines (villi) in reaction to gluten, a protein found in wheat, rye, barley and possibly oats. It may cause other intolerances which it has with me, restricting my diet still further. I also have an IgA deficiency which complicates treatment options too. This causes me lots of infections and leaky gut syndrome (malabsorption) which allows many allergens to pass into my system causing multiple food and drug allergies. I have developed anxieties over the years and relive my bad experiences. My mind does not turn off at night and I find it hard to sleep. I guess you would say I suffer from post-traumatic stress disorder. I do take something for it at night.
My fight is ongoing. I had surgery to remove a 6.9 cm. retroperitoneal tumor on March 8, 2000. It was encapsulated and taken out with clear margins. No other tumors. It was reclassified as endometrial stromal sarcoma. After almost 19 years, it returned to the scene of the original crime in the pelvic area. No two cases are alike and the doctor said he’d never heard of it happening after so many years. I went back to MSKCC again. I had a lot of complications this time. The tumor was in a difficult place to reach. The retroperitoneal area is under the intestines in the pelvis. I had to have 1” of my obturator nerve taken out because the tumor was growing into it along with one lymph node. This surgery left me with a disability. I have trouble walking to this day. I also had my remaining ovary removed. My path report said I was progesterone receptor positive. I had never been tested for that before. I was on Tamoxifen for 5 years when the lobular carcinoma in situ was discovered. No one seems to know it if helped slow down my LMS or didn’t, because there have been no studies on anything but breast cancer. It did lower my risk of developing invasive breast cancer. They have found out that Tamoxifen is a no-no for uterine sarcomas since then. It has been linked to causing uterine cancer and sarcomas now.
I had another recurrence, my fourth of LMS. My CT scan in January 2001 showed I had 5 nodules in my lungs again. I started hormone therapy, Megace, because my last tumor tested hormone receptor positive for progesterone, negative for estrogen. The nodules are very small, 1cm. and the rest in the mm range. I started taking Femara (Letrozole) and it kept me STABLE since I have been on the hormone therapy, which is in pill form. (I had to go off the Megace because it bothered my stomach and I had other bad side effects.) None of the nodules grew. I was reclassified as having endometrial stromal sarcoma because they are learning new things about sarcomas now. My last tumor carried stromal cells. ESS is considered a subclassification of LMS. It is thought that my cells mutated into ESS from LMS because I had myxoid LMS which has mixed cells.
I always have lots of questions to ask my doctors and write them down before a visit. Whenever I greet the doctors, I have a smile on my face. I think they have a hard time dealing with cancer patients all day long. I think it brightens their day and their attitude changes towards me. Sometimes I have to show them that I have a positive attitude and a sense of humor when they seem so serious. I have even had to reassure my surgeon that I would come through the last surgery with flying colors and gave him a pat on the arm. I tell my doctors I have no intentions of going anywhere real soon and I’ll be there to see them again. My family is proud of my attitude and I have become their role model just as my mother was my role model, surviving breast cancer for 35 years. I know that if something bad happens to any of them, they will show the courage, bravery and positive attitude that I have shown them. I have been lucky to have my husband walking by my side every step of the way. Others who do not have mates should find support from friends and family who really want to be there for them. Quite a few of my friends and family backed away because they no longer knew what to say to me. I found out that it was not me that had a problem. It was them. I felt better realizing this. They simply could not cope with my cancer. People tend to forget that even strong people need support.
It seems that I have lived with LMS half of my married life. I learned to cope and survive by joining a gyn-cancer group when I had my first recurrence. It was a wonderful group at the Brooklyn Cancer Institute and the therapist was a dedicated and caring woman who did her job well. I think all doctors should suggest counseling to their patients. There were 8 women but only I had LMS in a fibroid. For years, I listened to Dr. Bernie Siegel’s self-hypnosis tapes, Dr. Simonton’s relaxation tapes, and read all sorts of self-help books. I think they really helped me to cope. I got over the hump of living in fear and learned to live day by day. I am a survivor and I have dedicated myself to helping others like myself with education and knowledge. If I can help one person with my long journey, so that they learn about their options and that they MUST be monitored by scans (because I wasn’t and my recurrence was discovered by accident) I will be happy. If we started out as pussy cats, we can toughen up and learn to live like lions.
On 5/2004, My scans showed no growth after not taking Femara/Letrozole for 5 months. I had to stop taking it because it raised my cholesterol. I could not take statins so I went off it to rest my body. I remained stable and was monitored closely. No treatment until further growth.
On 12/1/04, My scans showed small growth in lung mets. Oncologist and I decided to wait longer before doing anything. We make decisions together.
On 4/05 Scans showed mm growth of one lung met, insignificant, may be just the slice in the scan. Check in 3 mos. No drugs started yet.
On 1/06 Scans showed 4 of 5 mets grew by mms. One was 1.8 cm, now 2.8 cm.
Oncologist and I decided it was time to start taking Femara/Letrozole again. Checking in 3 mos with ct scans.
On 4/06 Scans showed no growth in lung mets, no growth anywhere else. It is still working. Taking 1 month off to rest the body, 2 or 3 months on due to side effects of high cholesterol and bone pains. Decided to stay on a schedule of 2 months on, 1 month off. Will be monitored as oncologist sees fit. Always discusses options with me. Ultimately, the decision is always yours on what to do, it is your body and your life.
I started Aromasin. It is reported that the side effects are less bone pain. If it works, and the ct scan shows stability or shrinkage (that is possible too) I will go back to the 2 month schedule and rest the body one month.
I am now the co-moderator of the Endometrial Stromal Sarcoma online support group.
I am dedicating myself to educate other women who are not lucky enough to live in big cities and have doctors that only see sarcomas once or twice in a lifetime. I am the longest living survivor of the LMS list and ESS list and the oldest living survivor. It is my time to give back.
2007 Took Aromasin for 3 months, took scans, growth noted in 3 of my 5 lung mets. It didn’t work for me and promoted growth. One of my nodules doubled in size! Back to Femara. Scan in 3 months. Going to discuss other drugs for down the road.
6/07 Stable once again. Femara is still working. I did not become resistant.
Have to figure out new schedule with other substances to stay stable and go on and off the drug to avoid high cholesterol and osteoporosis and bone pain.
8/07 Taking a 6 week break from Femara hoping to reduce the bone pain and lower my cholesterol naturally. Will scan chest in Oct, discuss another strategy of going on and off the Femara to extend the time taking it…….
10/07 Scans showed growth in 3 nodules, back to Femara. The largest met is now 5.5 cm. New schedule: Femara 2 weeks on, 1 week off. Scans in January to see if it is holding stable.
LMS uterine fibroid primary
Rosalie lost her battle with sarcoma in 2011. She was a wonderful person and worked hard on behalf of the sarcoma community. We will all miss her.