Rare Adult & Pediatric Cancer Web Blog

Neuroendocrine tumors can grow anywhere in a person’s body.  They affect neuroendocrine cells, which can be found throughout the nervous and endocrine systems.  These cells manufacture and secrete regulatory hormones, necessary for several bodily functions.  Neuroendocrine tumors (NETs) can cause the overproduction of these hormones.

Carcinoid, a type of NET, are slow growing tumors.  The lack of patient symptoms make them hard to detect and diagnose.  Many patients with Carcinoid tumors acquire a condition called Carcinoid Syndrome from the over-production of serotonin. 

These conditions can create asthma-like wheezing, flushing of the face, severe diarrhea with resultant dehydration, and even cardiac disease.  Octreotide - brand name Sandostatin® - is a type of medicine called a somatostatin analogue.  It is used to treat NET patients who are experiencing these debillitating symptoms. 

Patients receiving injections of octreotide may experience difficulty in controlling their symptoms with monthly injections.  Your weight and the rate at which the drug is absorbed and metabolized, can effect the level of useful octreotide in the your system.  Often, your physician will need to supplement your treatment with additional daily injections.  This is referred to as ‘rescue’ treatment.  Having blood plasma checked for octreotide levels, to make sure that you are receiving and absorbing optimum levels of the drug, can help you to avoid this. You can read more about the testing here:
Octreotide Level Testing

Dr. Eugene Woltering is concerned that these optimal levels are not being achieved.  To help him research this, we have created a simple patient survey to compare octreotide use and drug activity.  This is an anonymous, volunteer survey.  All identifying information will remain secure and confidential.  The results of this survey may be important to you.

We are asking that all patients, using octreotide injections, consider participation in the survey.  To do this, you will need to gather octreotide blood level test results during your treatment period with octreotide injectible (Sandostatin®).  Contact your treating physician to get these reports.

If you would like to participate, click here:
Octreotide Survey

If you have any questions, or need help participating, please use this form and give me as much information as you can:
Contact Us

If you know of someone who might like to participate, but does not have a computer, please print this form off and have them fill it out:
Printable Survey Form

The survey is being conducted by:
Eugene A. Woltering MD FACS
James D. Rives Professor of Surgery & Neurosciences
Chief, Sections of Surgical Oncology & Endocrine Surgery
Director of Surgical Research
LSUHSC Department of Surgery
New Orleans LA 70112

In cooperation with:
The Rare Cancer Alliance
Sharon Lane, Administrator

Please pass this information on to other neuroendocrine and carcinoid survivors, you may know. Thank You

Intraperitoneal hyperthermic chemotherapy is being used, sometimes in conjunction with cytoreductive surgery, for selected peritoneal malignancies. Here is an excellent video presentation of intraperitoneal hyperthermic chemotherapy:
Wake Forest Video Presentation

You must have Real Player installed on your computer to view this video.  For a free download of Real Player, click here.

Types of cancer/conditions treated may include:

• Appendiceal Neoplasms with Peritoneal Dissemination
• Peritoneal Carcinomatosis
• Peritoneal Mesothelioma
• Peritoneal Sarcomatosis
• Pseudomyxoma Peritonei

Other types treated can be found here:
Dr. Sugarbaker - Current Indications For Use 

You can become a part of on of these clinical trials:

• Thalidomide in Treating Patients Who Have Undergone Surgery and Chemotherapy for Cancer That Has Spread Throughout the Abdomen Due to Colorectal Cancer or Appendix Cancer
• Continuous Hyperthermic Peritoneal Perfusion With Cisplatin Plus Intraperitoneal Paclitaxel and Fluorouracil Following Surgery in Treating Patients With Peritoneal Cancer
• Surgery Followed By Chemotherapy With or Without Intraperitoneal Chemotherapy in Treating Patients With Peritoneal Carcinomatosis From Gastrointestinal Cancer

So Is IPHC right for you? Blistering, burns, tissue swelling, blood clots, bleeding , and renal dysfunction were the possible side effects quoted in some of the abstracts I read. 

Morbidity is low if this treatment is administered in specialized centers with the necessary expertise and technical resources.  Keep in mind that this procedure is still considered relatively new.  Not all facilities/physicians are using it.  If you have chosen to do this, choose a physician who has extensive experience in this procedure. 

Take Care,  Sharon  www.rare-cancer.org 

I was reading an abstract from Endocrinologist today, written by physicians from MD Anderson, about the importance of long term follow up in differentiated thyroid cancers (a rare form of thyroid cancer).  The abstract discussed several cases that had recurrence after 10 years of NED (no evidence of disease).

It said “Factors known to influence risk of recurrence (age at diagnosis, initial tumor size, local invasion, metastatic disease at presentation, extent of initial surgery, and radioiodine treatment) were assessed in our patients. In each case, failure to continue close follow up led to delays in diagnosis of disease recurrence.”  The point here is that early detection of recurrence could increase a person’s ability to survive. 

Another article that I read today was discussing the mental mindset of the cancer clinician; where the primary emphasis of their practice is to detect and treat.  Long term follow up has not been a priority.  It was long believed that if you survived your cancer for over 5 years, you were ‘cured’.  Statistical data was only kept for 5 years.  I am not sure who came up with this time limit, but they obviously did not treat rare cancers.

In the rare cancer world, it is not uncommon to have a cancer that is indolent (slow growing) and resident in your body for many years prior to initial detection.  And, the same can happen with recurrence or metastasis.  I have been advocating for rare cancer patients for 8 years now and I find this to be true for several cell types. 

Let me bring this back into my own personal experience.  When I was diagnosed, I had an excellent medical oncologist who told me that he honestly did not know anything about my cancer, and could not spend time doing research on it.  He needed to spend his research time on cancers that would help the most number of patients.  But, he told me that he would read any research papers that I brought to him. 

We had a wonderful relationship, based on open and honest communication.  And, I felt that I was getting good care.  He told me that I would need to be followed by him, or someone else, for the rest of my life; because of the slow growth factor of my cancer and the unknowns left from the lack of long term follow up data.

I had to move.  I have seen 3 oncologist over the last 6 years in my new area.  Two of them blatantly lied to me during my followup appointment.  They both said that they had treated many, many women with my cancer.  One said he had treated over 50 (and he was a young man).  I highly doubt that, since in the year 1995, there were only 33 cases of my cancer diagnosed in the entire United States.  I never went back to either. For me, honesty is mandatory in a doctor.

The last one, I have stayed with for 3 years. On my first visit to his office, I saw him.  Last year, I was supposed to see him, but I saw the nurse practitioner.  She had absolutely zero information on my type of cancer. The only follow up test she order was a blood test (my cancer cannot be detected this way!).  She didn’t have a clue on what tests were approprate for my follow up.  I just called to make this year’s follow-up appointment and was told that I would need to see her again.  The office girls at this practice have been told that only new patients can see the oncologists. 

I have kept data on quite a few women with my cancer over the last 8 years.  Although metastasis is uncommon; in the women who had it happen, it did not show up until their 7th to 8th year.  So, I take this 8th year of NED seriously and I want to see a doctor, not a nurse practitioner.  I want appropriate follow up procedures ordered.  But, I can’t.  Who is responsible for this, I don’t know, and I don’t care.

Statistical data on many rare cancers is far too small to come up with any conclusive info on recurrence and metastasis.  In my humble opinion, all rare cancer patients should be followed up ‘over the long haul’.  If for nothing more than the pure study of that rare disease.

The most common body site for my cancer to return is the lungs.  So, I went to my primary care physician and asked him to order a CT lung scan, with contrast.  He did and I am awaiting the results.  I will not go back to that medical oncologist.  Maybe they don’t take my cancer seriously, but I do.  I know that I might have a fighting chance against it if it is rediagnosed at an early stage. I would like to give myself the ‘best chance’ of staying alive long enough to enjoy my grandkids!

One last note: Thank You! to the doctors at MD Anderson for taking the time and effort to take a long term view of rare cancers.  You get the Hero award of the week.

Take Care,  Sharon  www.rare-cancer.org

Today’s news articles are discussing her completion of treatments (radiotherapy and chemotherapy).  The thing that is bothering me about most of the articles is their desire to make this sound like it was a ‘cake walk’ for her. After listening to members of our Anal Cancer Group, I have to tell you that I don’t think this was an easy journey for her.  So, I want to take a moment out to tell her that she is one tough cookie and I am so glad that she got through her treatment phase.  I pray that she stays in remission and is able to get on with her life.  You Go Girl!

Take Care,  Sharon  www.rare-cancer.org

Dr. Matthew Anderson, Baylor College of Medicine in Houston, TX should be considered our knight in shining armor, ladies.  He and Dr. Diane Bodurka, from the M.D. Anderson Cancer Center, created a database of information for 400 patients diagnosed with this rare form of uterine cancer.  Compiling the patient information took Dr. Anderson over 5 years.  Data, such as tumor size, patient age, menopausal status, and treatment methods was documented. 

He is currently working to create a specialized clinic for uterine leiomyosarcoma at the Baylor Clinic in Houston.  Hopefully, those of you who have uterine leiomyosarcoma will have a ‘champion’ to help your through it.  This is a rare gift in the world of rare cancers.  I only wish that more physicians would follow in Dr. Anderson’s footsteps and take an interest in some of the many rare cancers who have no champion.  We sure could use the help.

Dr. Matthew Anderson, I Salute You!

Take Care,  Sharon  www.rare-cancer.org

Ewing’s sarcoma, peripheral primitive neuroectodermal tumor (PPNET), and Askin’s tumor are classified as Ewing’s tumors.  It is most often found in children, adolescents, and young adults.

Chromosomal translocation and fusion between chromosomes is the basic cause of this cancer. These tumors can occur anywhere in the body, but most commonly present in the pelvis, proximal long tubular bones (such as the ribs, femur, and humerus), and/or soft tissues. 

Prognosis, based on age, has led to controversy.  Some studies show that the older a person is at diagnosis, the poorer the outconme of treatment. Others studies show no age-related survival differences. This article examines whether age at diagnosis affects prognosis:

How to Treat the Ewing’s Family of Sarcomas in Adult Patients

Take Care,  Sharon  http://www.rare-cancer.org
This email or the contents of our website should not be misconstrued as medical advice.  Please review all information with your medical professionals.

I am working on a rare cancer symbol and ‘ribbon’.  I will have that work done this week and (hopefully) will be drafting up some bumper stickers, window clings, decals, pins, and pendants for rare cancer survivors.

At the same time, I will be writing up the story behind this symbol and why I chose to design it this way.  I hope that it will let others see how we do not necessarily benefit from research done for more common cancers.  If anyone would like to participate by sharing your story of ‘difference’, please let me know.

Take Care,  Sharon - www.rare-cancer.org

Adult Soft Tissue/Bone Sarcoma -  

Soft tissue/bone sarcomas are malignant tumors that may arise in any of the connective tissues (muscles, tendons, vessels that carry blood or lymph, joints, bones, nerves and fat). The peak age incidence is around 50 years. Sarcomas are a diverse range of tumors, they are named after the type of soft tissue/bone cell they arise from. Types of soft tissue/bone sarcomas include; alveolar soft-part sarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, liposarcoma, malignant fibrous histiocytoma (MFH)*, hemangiopericytoma, mesenchymoma, schwannoma, peripheral neuroectodermal tumours, rhabdomyosarcoma, synovial sarcoma, osteosarcoma and other types.

Kaposi’s sarcoma is a type of cancer where malignant cells are found in the tissues under the skin, lining of the mouth, nose, and anus. Symptoms may include red or purple patches (lesions) on the skin and other parts of the body. Many (though not all) Kaposi’s sarcomas are AIDS related.

August 5, 2006

I have metastatic MFH* since 2000: (Eight Occurrences – as of 8-5-06)
(all surgery path reports were notated  MFHs).

All the following were tumors:

1. Right shoulder/arm interface – limb-sparing surgery, with adjuvant external radiation.

2. Anterior right thigh – surgery, with adjuvant external radiation.

3. Left para-spinal muscles – surgery.

4. Anterior left thigh, on the femor bone – chemo trip (only Adriamycin) to see if it can kill the MFH. Chemo didn’t.  5. Recur – left para-spinal muscles – surgery, with adjuvant external radiation. (Recur because of no radiation – in the first instance - #3).

6. Upper lobe of my left lung – surgery (thorocoscopy/thorocotomy).

7. “Satellite” of the left para-spinal muscle – surgery, with adjuvant external radiation. Was due to collection of MFH semi-solid sac that appeared ~ 1 month later from #5.

8. (Just last month) – (3-month growth of) 2 cm. “presumably” MFH on the anterior left chest wall, projecting into the pleural space; this was a  “left-over” piece from the thorocotomy (in #6. above). Radio-Frequency Ablation (RFA) performed for this tumor – to be imaged for effect – (CT) on/about Sep. 20, 2006. The RFA was a “piece-of-cake”, and I feel “great” since then.

If other tumors show up in percutaneous locations, I will explore the RFA procedure to apply to these. I’m tired of the “cut-and-take” jobs on my body.

So, you see that I am a sarcoma “survivor”, and at 75!

I am of the opinion (unfortunately), that rapid spread and recurs of sarcomas are more prevalent in people of age 50 or younger.

So, “youngsters”, if you have ANY symptoms (lumps, pain, etc. – anywhere on your body), go to a sarcoma center-of-excellence ASAP – to get the RIGHT diagnosis the FIRST time, and to start treatment – as per protocols.

With Hope & Life For All 
Stan