Neuroblastoma Flashcards
(25 cards)
What is a Neuroblastoma
- paediatric cancer of the NS
- derived from the neural crest cells that would form the sympathetic ganglia & adrena medulla
Where do neuroblastoma’s typically develop?
- frequently starts from one of the adrenal glands
- can also develop in head, neck, chest, abdomen or spine
Describe neuroblastoma epidemiology
- incidence = 1/10,000 per year
- peak age = 2-3 y.o
- accounts for 10% of cancers under 15 y.o
- prognosis better for younger infants <18 months
What can cause neuroblastoma’s?
- cause isn;t well understood
- mostly sporadic & non-familail
- 1-2% of cases run in families
- Familial NB has been linked to specific gene mutations (gremlin mutations) = ALK gene
What are some clinical features of neuroblastoma’s?
- lymphatic & hematogenous spread
- metastases to bone, liver, bone marrow & skin
- most patients present with advanced disease = 40% of patients
What are the survival rates for neuroblastoma?
- 5-year survival rate = 7/10
What is the chance of survival for relapsed neuroblastoma?
1/0
How can neuroblastoma present?
- large abdominal masses ->may lead to shortness of breath
- increased catecholamines levels
- abnormal/excessive secretion of vasopressin intestinal peptide
What tests can help diagnose neuroblastoma?
- ultrasound, MRI & CT scans
- Histochemical analysis (staining)
- genetic testing may also be performed to determine cause
Describe Tumour Stage 1 of Neuroblastoma
= localised tumour that is completely surgically removed at diagnosis
Describe Tumour Stage 2 of Neuroblastoma
- localised tumour that cannot be completely surgically removed at diagnosis
- lymph nodes on same side at tumour may have tumour cells present
Describe Tumour Stage 3 of Neuroblastoma
- large tumour that has spread across middle of the body that cnanot be surgically removed at diagnosis
Describe Tumour Stage 4 of Neuroblastoma
- tumour of any size that has metastasized to distant lymph nodes, bone marrow, bone or liver
Describe Tumour Stage 4S of Neuroblastoma
- Special stage that only applies to a child <18 months
- small localised tumour that has metastasized to liver, skin and/or bone marrow
What is MYCN ?
- A transcription factor expressed predominantly in the developing nervous system
What does Amplification represent?
- represents one of the major molecular pathways through which the oncogenic potential of protocol-oncogenes is activaed during tumorigenesis
How does MYCN show amplification?
- extra-chromosomal amplification containing amplified gene
- intrachromosomal amplifications are areas on the native chromosome with multiple kB copies of the region containing the amplified gene
What percentage of neuroblastomas does MYCN Amplification account for ?
= 20-30%
What are some examples of MYCN targets?
- hTERT telomerase
- MDM2
- TrkA
Describe Cancer Stem Cell Theory
- hypothesis is that tumour growth is driven by a small number of stem cell like cells
- they’re capable of self-renewal & differentiation
- cancer stem cells/tumour initiating cells may be derived from stem cells/progenitor cells
Why do some tumour occur?
- chemo & radiotherapy target rapidly dividing cells = often missing slow dividing ‘stem’ cells
- tumour cells are also quite plastic-> meaning they could differentiate & may be able to evade specific treatment
What are some treatments for low/intermediate risk neuroblastoma?
- no treatment
- surgery to minimise tumour
- low intensity chemo can kill cancer cells
What are some treatments for high risk neuroblastoma?
- high dose chemo
- surgery
- radiation
- retinoic acid
- immunotherapy
- stem cell transplant
How does Cis-retinoic acid affect neuroblastoma ?
- induces differentiation of neuroblastoma cells in vitro & decreases proliferative capacity
- show in randomised clinical trials to improve outcome
