Tag: #NoChildShouldDieOfCancer

Treatment of High-Risk Neuroblastoma

Children with high-risk neuroblastoma is the most challenging group to treat. Current treatment strategy for this group consists of 3 treatment blocks:

  1. induction: chemotherapy and primary tumour resection;
  2. consolidation: high-dose chemotherapy with autologous stem-cell rescue and external-beam radiotherapy [XRT];
  3. post-consolidation: anti–ganglioside 2 immunotherapy with cytokines and cis-retinoic acid.
Adopted from: Pinto NR et al JCO  2015, 33, 3008-3017.
Up to 50% of children that do respond experience disease recurrence with tumour resistant to multiple drugs and more aggressive behaviour that all too frequently results in death.
For the majority of children who do survive cancer, the battle is never over. Over 60% of long‐term childhood cancer survivors have a chronic illness as a consequence of the treatment; over 25% have a severe or life‐ threatening illness.
Reference:

Pinto NR, Applebaum MA, Volchenboum SL, Matthay KK, London WB, Ambros PF, Nakagawara A, Berthold F, Schleiermacher G, Park JR, Valteau-Couanet D, Pearson AD, Cohn SL. Advances in Risk Classification and Treatment Strategies for Neuroblastoma.J Clin Oncol. 2015 Sep 20;33(27):3008-17.

 

 

What is the risk group classification system?

To be able to guide the treatment of neuroblastoma patients, doctors have developed a number of classification systems. Although sharing common features, they slightly vary by medical center, country and continents making direct comparisons of treatment results difficult. Doctors and scientists are trying to consolidate all systems in one in order to evaluate treatments in the past, currently ongoing and in the future.

Scientists have suggested a newer risk group classification system, the International Neuroblastoma Risk Group (INRG) classification that would incorporate the best knowledge gained and recent advancements in the disease imaging and neuroblastoma molecular diagnostics. This system is based on imaging criteria using the image-defined risk factors (IDRFs) and the prognostic factors such as:

  • The child’s age
  • Tumour histology (the tumour appearance under the microscope)
  • The presence or absence of MYCN gene amplification
  • Certain changes in chromosome 11 (known as an 11q aberration)
  • DNA ploidy (the total number of chromosomes in the tumour cells)
The table is adapted from Pinto NR J Clin Oncol. 2015

Using these factors the INRG classification put children into 16 different pre-treatment groups (lettered A through R). Each of these pretreatment groups is within 1 of 4 overall risk groups:

  1. Very low risk (A, B, C)
  2. Low risk (D, E, F)
  3. Intermediate risk (G, H, I, J)
  4. High risk (K, N, O, P, Q, R)

This system has not yet become common across all medical centers, but it is being researched in new treatment protocols.

Doctors and scientists are planning to improve the INRG classification system by incorporating other molecular diagnostics data such as profiles of the neuroblastoma genome (DNA), transcriptome (RNA), and epigenome* in order to make precise prognostication even better.

* The epigenome is made up of chemical compounds and proteins that can attach to DNA and direct such actions as turning genes on or off, controlling the production of proteins in particular cells

References:

Pinto NR, Applebaum MA, Volchenboum SL, Matthay KK, London WB, Ambros PF, Nakagawara A, Berthold F, Schleiermacher G, Park JR, Valteau-Couanet D, Pearson AD, Cohn SL. Advances in Risk Classification and Treatment Strategies for Neuroblastoma.J Clin Oncol. 2015 Sep 20;33(27):3008-17.

What is neuroblastoma?

Neuroblastoma is a childhood cancer. The word neuroblastoma consists of two words neuro and blastoma.The term neuro refers to nerves, blastoma  –  to a cancer of immature cells.

It starts in some types of nerve cells during embryo development.transforming immature nerve cells into cancerous cells. This type of cancer occurs most often in infants and young children mostly under the age of 5 years old.

Neuroblastoma cells

Neuroblastomas behave very differently:

 

The types of treatment used for neuroblastoma can include:

Children who survive have a high chance of developing long term side effects as a result of the treatment that saved their lives

More details about neuroblastoma can be found here:

American Cancer Society

Cancer Research UK

What is cancer?

Cancer is an umbrella term that covers a group of diseases sharing the common features but diseases vary by site of origin, tissue type, race, sex, and age. One of the main features is an uncontrollable growth of cells. These cells are capable of spreading to other parts of the body. This process is also known as invasion and metastasis.

Though cancer in kids is not the same as in adults, childhood cancer cells behave in the same way. They grow uncontrollably and can travel to new destinations in the body.

This video ‘Cancer: from a healthy cell to a cancer cell’ nicely explains this transformation.

This video ‘How does cancer spread through the body?’ gives a perspective on the ways cancer cells travel in the body.

September is Childhood Cancer Awareness Month

September is Childhood Cancer Awareness Month!
Facts about childhood cancer

Please watch this video created by St. Baldrick’s Foundation | The Childhood Cancer Ripple Effect

References:
Gatta G, Botta L, Rossi S, Aareleid T, Bielska-Lasota M, Clavel J, et al. Childhood cancer survival in Europe 1999-2007: Results of EUROCARE-5-a population-based study. Lancet Oncol. 2014;15(1):35–47.
Ward E, Desantis C, Robbins A, Kohler B, Jemal A. Childhood and Adolescent Cancer Statistics, 2014. Ca Cancer J Clin. 2014;64(2):83–103.
Dolgin MJ, Jay SM. Childhood cancer. 1989;327–40.
Miller RW, Young Jr. JL, Novakovic B. Childhood cancer. Cancer [Internet]. 1995;75(1 Suppl):395–405.
Raab CP, Gartner JC. Diagnosis of Childhood Cancer. Primary Care – Clinics in Office Practice. 2009. p. 671–84.
Howlader N, Noone A, Krapcho M, Garshell J, Miller D, Altekruse S, et al. SEER Cancer Statistics Review, 1975-2011 [Internet]. National Cancer Institute. 2014.
Ries L a. G, Smith M a., Gurney JG, Linet M, Tamra T, Young JL, et al. Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995. NIH Pub No 99-4649. 1999;179 pp
Warren KE. Diffuse intrinsic pontine glioma: poised for progress. Front Oncol [Internet]. 2012;2(December):205.
Lackner H, Benesch M, Schagerl S, Kerbl R, Schwinger W, Urban C. Prospective evaluation of late effects after childhood cancer therapy with a follow-up over 9 years. Eur J Pediatr. 2000;159(10):750–8.

 

SIOPEN Meeting 2017

The dates for the SIOPEN AGM and Neuroblastoma Research Symposium were announced. This meeting will be hosted by the German So­cie­ty for Pa­ed­ia­tric On­co­lo­gy and Hae­ma­to­lo­gy and will take place @ Langenbeck Virchow Haus, Berlin, Germany on October 25-27, 2017.

Mission
To increase the understanding of neuroblastoma pathogenesis,
progression and treatment failure and to improve survival
and quality of life for children with neuroblastoma.

Main Objectives

  • To consolidate a platform for global collaboration
  • To establish networks of multidisciplinary caregivers
  • To develop new trial protocols
  • To develop standards for radiotherapy and surgery
  • To develop SOPs for biomaterial collection, handling and storage
  • To develop SOPs for application of major research technologies
  • To identify leaders for specific topics

Scientific Topics/Plenary Sessions

  1. Molecular Risk Stratification
  2. Liquid Biopsies
  3. Tumor Heterogeneity+Tumor Microenvironment
  4.  New Preclinical Models: PDX, GEMM, zebrafish
  5. New Immunotherapy Approaches
  6. New Drug Targets/Early Clinical Trials
  7. Neuroblastoma Pathogenesis/Genetics
  8. Targeting MYC
  9. Targeting ALK
  10. Targeting RAS/MAPK
  11. SIOPEN HR-NBL2 Clinical Trial Strategy
  12. Update ongoing SIOPEN trials: HR-NBL-1, LINES, VERITAS, OMS
  13. Update SIOPEN Bioportal
  14. Concept Biology+Relapse Umbrella Trial

Why do we need fundraising for cancer research?

There is no short answer. Research is a slow, meticulous process of testing theories and finding out which ones work.It is exactly the same for both curiosity- and disease- driven questions. Long years of ground research full of ups and downs are critical for any breakthrough or progress. Very often with more downs than ups. Importantly, all researchers build on the work of their predecessors. This is the nature of science.

To understand the world around us, we have to do be curious and do “blue sky or curiosity-driven” research. It is a long shot, but this type of research can lead to practical applications down the road. One of the most recent examples is a drug Vismodegib (Erivedse) to treat basal cell carcinoma (the most common type of skin cancer) approved by the FDA in 2012. This drug targets genes of a hedgehog-associated signalling pathway. Defects in this pathway were found to drive many cases of skin cancer. But, how this relationship was found? Blue sky research!

Researchers studied hedgehog signalling in fruit flies and mice. One of the researchers had a strong interest in a fruit fly gene called hedgehog. If this gene is defective, then fly embryos look stubby and hairy aka a hedgehog. Further research brought more interesting facts and relationships leading to the identification of a drug that can stop the function of this faulty gene. Decades later with the advancement of genome sequencing, the defect in hedgehog signalling pathway genes was identified in patients with locally advanced and metastatic basal cell carcinoma.

What would happen if there were no research in fruit flies and mice? There would have been no rationale to create a drug like Vismodegib!

The best discovery research is unrestricted. It is driven by intellectual curiosity and conceptual advancement. More such curiosity- driven research is needed. For every medical breakthrough, for every Vismodegib, there were hundreds of blind alleys and failed ideas.

The research is a long-term investment. This contradicts to the short-term life of the politicians and governments who give the money. They do not take the risks. So, the discovery research becomes critically underfunded.

Fundraising creates opportunities for blue sky research and developing cancer treatments.

Thank you all who support cancer research charities!

 

The IACR Meeting 2017 is targeting childhood cancer challenges and advancements

This week Newpark Hotel Kilkenny is hosting the Irish Association for Cancer Research annual meeting 2017. This meeting is the biggest event for Irish cancer researchers.

This is the first time in the history of the IACR meetings when an entire plenary session is solely dedicated to challenges and advancements in childhood cancer.

This session will unite Internationally recognised leaders in childhood cancer research. They will speak about what we know about origin and evolution of childhood cancers (Prof. Tariq Enver), how blood biomarkers can help in stratification and treatment of children (Prof. Sue Burchill), what impact Down syndrome has in the white blood cell cancer development and progression (Prof. Irene Roberts), how epigenetic changes affect tumour pathogenesis and future of therapeutics targeting theses changes (Prof Raymond Stallings).

International Childhood Cancer Day: 15 February 2017

 

Today, we are celebrating International Childhood Cancer Day to raise awareness and to express support for children and adolescents with cancer, survivors and their families.

Childhood cancer is an umbrella term for a great variety of malignancies which vary by site of disease origin, tissue type, race, sex, and age.

The cause of childhood cancers is believed to be due to faulty genes in embryonic cells that happen before birth and develop later. In contrast to many adult’s cancers, there is no evidence that links lifestyle or environmental risk factors to the development of childhood cancer.

Every 100th patient diagnosed with cancer is a child.

In the last 40 years the survival of children with most types of cancer has radically improved owing to the advances in diagnosis, treatment, and supportive care. Now, more than 80% of children with cancer in the same age gap survive at least 5 years when compared to 50% of children with cancer survived in 1970s-80s.

Childhood cancer is the second most common cause of death among children between the ages of 1 and 14 years after accidents.


Unfortunately, no progress has been made in survival of children with tumours that have the worst prognosis (brain tumours, neuroblastoma and sarcomas, cancers developing in certain age groups and/or located within certain sites in the body), along with acute myeloid leukaemia (blood cancer). Children with a rare brain cancer – diffuse intrinsic pontine glioma survive less than 1 year from diagnosis. Children with soft tissue tumours have 5-year survival rates ranging from 64% (rhabdomyosarcoma) to 72% (Ewing sarcoma).


For majority of children who do survive cancer, the battle is never over. Over 60% of long‐term childhood cancer survivors have a chronic illness as a consequence of the treatment; over 25% have a severe or life‐ threatening illness.

 

References:
Gatta G, Botta L, Rossi S, Aareleid T, Bielska-Lasota M, Clavel J, et al. Childhood cancer survival in Europe 1999-2007: Results of EUROCARE-5-a population-based study. Lancet Oncol. 2014.
Howlader N, Noone A, Krapcho M, Garshell J, Miller D, Altekruse S, et al. SEER Cancer Statistics Review, 1975-2011. National Cancer Institute.
Lackner H, Benesch M, Schagerl S, Kerbl R, Schwinger W, Urban C. Prospective evaluation of late effects after childhood cancer therapy with a follow-up over 9 years. Eur J Pediatr. 2000.
Ries L a. G, Smith M a., Gurney JG, Linet M, Tamra T, Young JL, et al. Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995. NIH Pub No 99-4649. 1999;179 pp
Ward E, Desantis C, Robbins A, Kohler B, Jemal A. Childhood and Adolescent Cancer Statistics , 2014. CA: Cancer J Clin. 2014.