(The majority of Wilms at Home articles can be found in our newsletters. These include articles written by families directly affected, survivors of Wilms and experts in the area of Wilms Tumour diagnosis, treatment and aftercare.)
This page features articles and interviews written by doctors and other experts who care for patients with Wilm's Tumour. This page includes two interviews, one with Professor Giulio D'Angio who is an expert in this area and has been involved in Wilms Tumour treatment in the US since the 1940s and a further interview with Dr Margarita Belogurova specialist in paediatric oncology from St Petersburg in Russia. We have also added an article by Professor Pritchard-Jones on 'Stem Cell Transplantation'. (Please see below)
Article by Professor Kathy Pritchard-Jones on Stem Cell Transplantation
Professor of Paediatric Oncology
Royal Marsden NHS Foundation Trust &
Institute of Cancer Research, Sutton, Surrey
April 2006
‘Stem Cell Transplantation' means the use of stem cells to re-populate a patient's bone marrow after they have received high-dose chemotherapy. When used in the treatment of Wilms' tumour, the stem cells will have been harvested from the patient's own bone marrow earlier in their treatment. This is called autologous stem cell transplantation or high-dose therapy with stem cell rescue. Most children with Wilms tumour do not need this type of therapy. However, for those children whose Wilms tumour relapses, a substantial proportion need intensive re-treatment. In the UK, there is currently a national clinical trial or study called UKWR . In this trial, children with relapsed Wilms tumour are allocated to one of three treatment arms, according to the amount of treatment already given and the time interval between diagnosis and relapse. Children who have previously received at least three chemotherapy drugs (ie including doxorubicin), with or without radiotherapy, or for any relapse occurring within six months from initial diagnosis, or for any patient progressing on first-line therapy, are allocated to Treatment arm C . This comprises six courses of fairly intensive chemotherapy followed by high-dose chemotherapy with a drug called melphalan . Following this final course of ‘high dose' melphalan, the patient's stored stem cells are re-infused in order to allow the blood count to recover. As the high-dose chemotherapy wipes out the patient's own bone marrow, they need to stay in hospital until the re-infused stem cells have had time to make new blood. This usually means a 3 – 4 week stay in hospital, during which time the child will require antibiotics, nutritional support, and possibly other supportive care. There is a very small risk of a serious, life-threatening infection during a high-dose therapy procedure. Usually, however, it is well tolerated. Following the high-dose therapy, the child usually receives radiotherapy to any sites of relapse, unless they have been previously irradiated. They may also have needed surgery to any remaining tumour lumps. There is a general consensus among international experts in Wilms tumour that intensive chemotherapy is of benefit in treating children with relapsed Wilms tumour. What is less clear is whether high-dose therapy is any better than simply continuing more blocks of intensive chemotherapy. This question will be addressed by a new international clinical trial, to be run jointly between North America and the European groups. We hope that this trial will open some time in 2006/2007.
Thank you very much Professor Pritchard-Jones for this very helpful and informative article on Stem Cell Transplantation.
Interview with Professor Giulio D'Angio
Department of Radiation Oncology/2 Donner
Hospital of the University of Pennsylvania
3400 Spruce Street
Philadelphia, PA 19104
5th
November 2003
1. We are aware that you have a tremendous amount of experience and knowledge in the treatment of Wilms Tumour over the years.When were you first involved in the treatment of Wilms and how was it treated then?
"
I first become involved with the management of children with Wilms tumor as
a surgical intern in 1945. It was then treated by nephrectomy and routine
postoperative radiation therapy at the Boston Children's Hospital where much
of the early research in the management of these children was performed."
2. How did treatment change over the years?
"
There has been a steady progression of management changes over the years.
These have to do with very careful
calibration of the intensity of treatment according to well-defined risks. For
example, the need for routine postoperative radiation therapy was shown to be
unnecessary in early stage, so-called favorable histology Wilms tumors.
On the other hand, the addition of doxorubicin was found to be beneficial in
children with more advanced stages of disease. An additional important
point is the separation of "Wilms tumors" into its component and quite separate
parts. Two of the cancers are now known to be unrelated to classic Wilms
tumors. They are the so-called "clear cell sarcoma", and the "rhabdoid
tumor of the kidney". A subset of Wilms tumor called the "anaplastic"
form was also identified and makes up approximately 8% of the total. The
remaining proportion is called "favorable histology". The importance of
all this is that the amount of treatment and the kind of therapy varies according
to the risks inherent in each of the different subsets."
3. We understand that you are an expert in the use of radiotherapy
in Wilms Tumour treatment. Can you expalin to us how radiotherapy works?
" X-ray therapy works because it affects the ability of dividing cells to reproduce. This eventually leads to their death. The mechanisms involved in producing this result are very complex."
4. One family in our network is especially interested
in hairgrowth after treatment. Is it quite common that hair grows quite thin
after chemotherapy and radiotherapy for Wilms? Is there anything parents can
do about this?
"There
is nothing the parents can do about the thinning of hair that occurs after chemotherapy.
Radiation therapy does
not affect hair growth unless the scalp itself is irradiated, of course."
5. Another family is interested in finding out about the
possibility of stenghtening the immune system during and after treatment? Is
there any evidence that nutrition or vitamins can discourage Wilms Tumour growth?
"There is no diet or "tonic" or other mechanisms by which the immune system can be "strengthened". Also, there is no evidence that nutrition, vitamins, tonics or any other dietary modification or additive can affect the re-growth of Wilms tumor."
6. What role do you see complimentary medicine take either
during or after treatment?
"There is no known role for complementary medicine in the management of Wilms tumor patients."
7. Do you believe that certain psychosocial factors such
as effective communication, decision making with health professionals and support
for the whole family in the form of rehabilitation is important in Wilms?
"Psychosocial Factors-These cannot be stressed enough. The concept of "total care" implies exactly that. That is, a family with a sick child is a sick family, and all members of the family, particularly the siblings, must be remembered in the support and rehabilitative efforts that need to be undertaken. Too often, the focus is only on the sick child. All the members of the family are affected including the fathers. They are supposed to be strong and brave, and it is often forgotten that they suffer as much as the mothers from what is a devastating illness and psychosocial economic problem."
8. What is your opinion on the different methods used to
treat Wilms Tumour in Europe and the US?
"Europe
vs. the USA. - It is forgotten that preoperative treatment is adopted in the
United States when the surgeon is faced with a huge tumor that makes the operation
risky. Equally forgotten is the fact that young children in Europe have
primary surgery rather than preoperative therapy. Each has its place.
The results are equally good given 100 children. We in the United States
feel that the treatment can best be tailored to the facts gathered by primary
surgery. It fulfills one of the cardinal rules in managing children or
adults with cancer; that is, assure the diagnosis before starting treatment.
I have recently analyzed the advantages and disadvantages of the two systems
and it will appear in the pages of Medical and Pediatric Oncology very soon.
To summarize the results of those analyses, I can paraphrase the concluding
remarks. They read something like this, "What would the author (myself)
do if a member of the family developed a renal mass? If near a primary
pediatric hospital or institute of top quality, surgery first. If not
near such an institution, preoperative chemotherapy." I think that summarizes
the main issues that are involved."
9. Do you have any opinions on the causes/development of Wilms Tumour? If Wilms Tumour is purely seen as a genetic condition how can the particular genetic mutation be explained? Do you believe that certain chemicals can encourage Wilms tumour growth?
"Wilms
tumor causation. - To date, no "familial Wilms tumor gene" has been identified.
It is therefore not possible to predict with accuracy whether a specific child
is at risk through genetic testing. Rather, the family history is the
best indicator of such an increased risk. Simply put, if more than one
near family member has or has had a Wilms tumor, the children in that family
have an increased risk. I do not believe there is any chemical that encourages
Wilms tumor growth."
10. A few weeks after this years SIOP's conference in Cairo, where does Wilms Tumour research and treatment go from here?
"The future of Wilms tumor research and treatment. - There continues to be extensive work at the molecular genetic level to try to identify the individual child with a Wilms tumor who may be at increased risk of relapse. Meanwhile, refinements of treatment are going on on both sides of the Atlantic. The objective always is not only to increase survival rates, but also to obtain the best possible quality of life of the survivor. Those have been goals from the very beginning of the coordinated, group-wide studies of Wilms tumor, and they remain paramount."
Questions were prepared by Monica with contributions from family members affected. We would like to thank Dr D'Angio for taking the time to answer our questions. top
Interview with Dr Margarita Belogurova
Hospital N31 Dinamo
St Petersburg 197110
Russia
t. + 7 812 235 07 21
f. + 7 812 235 21 11
1. We are interested to find out how Wilm's Tumour is treated in St Petersburg and also in other parts of Russia. Is the SIOP's protocol followed in your hospital?
Since 1993 in our hospital the patients with Wilms tumors have been treated according to SIOP` s protocol.
In my city there is the other children`s department in the Research Institute named after Petrov, where the children with solid tumors can be treated. We have not seen articles from this hospital, concerning these patients. Unfortunately, in our city there is not a united program for the treatment of the patients with malignant diseases.
There are several children`s oncology departments in other parts of Russia, where the children are treated according to SIOP`s protocol.
2. What is your role in the hospital?
I
am a head of department of paediatric oncology and haematology.
3. How many children
with Wilms are treated in your hospital each year?
In our hospital we usually have from two to five primary patients with Wilms tumors in a year.
4. We are aware of some health funding difficulties where you are. Can you tell us about the particular challenges you are facing when treating Wilm's Tumour in the hospital you are based? For example, do you have enough specialists, medicines, equipment etc?
We have everything, that is necessary for the modern treatment of this group of patients. But we would like to have more modern CT scans, because the quality of CT scans can be not high enough and does not always satisfy us.
5. How can the right diagnosis be ensured if there is a lack of essential equipment such as MRI and CT scans?
In our hospital we have CT and MRI scans.
6. How was Wilms treated prior to the Gorbachov era?
Prior to the Gorbachov`s era the overall survival of the patients with Wilms tumors was about 80%, now in our hospital the overall survival is 90%.
The treatment`s results between 1990 and 2002 differ insignificantly, but protocol SIOP allowed us to
7. What research is being carried out in St Petersburg and other parts of Russia in the area of Wilm's tumour?
Now in our city any research is not being carried out in the area of Wilms tumor In our hospital we have a very good genetic lab, we would like to begin genetic investigations, concerning Wilms tumors.
We would like to define telomerase activity in Wilms tumors, because we know that high expression of telomerase in Wilms tumor is associated with adverse clinical outcome.
8. Is your hospital involved in any experimental treatment possibly using potentially new medicines in Wilm’s tumour?
Our hospital is not involved in any experimental treatment`s protocol concerning Wilms tumor.
9. What is your opinion on the causes of Wilms?
We think that Wilms tumor has genetic nature. Wilms tumor is one of the diseases of civilization. Industrial and nuclear waste, acid rains and others can be reasons inducing this disease.
10. We are about to start a charity benefiting families affected by Wilms across Europe. Do you have any suggestions as to the help we could provide to some of your families affected by Wilms?
Most of our patients are from young and unprovided families. They are in need of any help / good nutrition, rehabilitation. Some patients suffer from anemia. In my hospital we have not enough erythro-poetin, which is necessary for any of them.
Questions
were prepared by Monica with contributions from family members affected. We
would like to thank Dr Belogurova for taking the time to answer our questions.
top
contact:
monica@wilmsineurope.net
Last Updated: June 2007