Paediatric, teenage and young adult Flashcards
Causative factors
No definite and known causes
Genetic causative factors
Very rare - possibly linked to retinoblastoma
Some children born with genetic changes that may increase risk of developing a malignancy
Down’s syndrome (10-20% increased risk of acute leukaemia’s but lessened risk of developing solid tumours)
Neurofibromatosis
Environmental causative factors
Radiation
Pelvic imaging during pregnancy linked to ~30% increased risk of leukaemia/ solid tumour in childhood
No evidence that environmental radiation increases risk
EMF (no evidence)
In the 0-14 age group what percentage of cancers are leukaemia?
1/3 of diagnoses
In the 0-14 age group what percentage of cancers are leukaemia, brain and lymphoma?
2/3 of diagnoses
Childhood cancers examples
Retinoblastoma, rhabdomyosarcoma, neuroblastoma, wilm’s tumour, medulloblastoma, DIPG
Retinoblastoma
Optic nerve invaded
Children under 5
Genetic - RB1 mutation
Leukocoria - white glow/refelction
Ophthalomoscopy
Intraocular/extraocular
Chemo/RT/surgery
Rhabdomyosarcoma
Muscle tissue cancer
Children under 10 - boys
H&N, bladder, testes, uterus
Embryonal/Alveolar/Pleomorphic
Rhabdomyosarcoma - Embryonal location and treatment
Head and neck, bladder or genital area
Surgery and chemo
Rhabdomyosarcoma - Alveolar location and treatment
Arms and legs of older children, can also occur in trunk
Surgery, chemo, RT
Rhabdomyosarcoma - Pleomorphic location and treatment
Adults
Surgery and RT (does not respond well to chemo)
Neuroblastoma
Develops in neuroblasts - early nerve cells
Commonly abdomen - adrenal glands
Age 5
Blood tests, US abdomen, chest, MRI, PET-CT
Surgery, chemo, RT, immunotherapy (GD2 MAB used- dinutuximab)
Wilm’s tumour
Nephroblastoma, kidney tumour
Under 5s - girls
Usually 1 kidney but 10% cases 2
Blood test, US, Chest imaging, MRI, biopsy
Chemo, surgery (nephrectomy), RT
Medulloblastoma
15-20% of childhood brain tumours
More common in ages 3- 8 years
(2nd most common brain tumour, 1st most common malignant brain tumour - astrocytoma most common)
Fast growing
4 subgroups:
WNT - regulate cell growth, motility and differentiation during embryonic development
SHH - TP53 status
Group 3 - aggressive, worst prognosis
Group 4 - most common
Headaches, morning vomiting, vision issues, bowel/bladder control
Surgery (reduce pressure), RT whole brain/spinal cord/whole CNS, chemo
70-80% survival at 5 years + (subtype dependant)
What does DIPG stand for?
Diffuse Intrinsic Pontine Glioma
What is DIPG and where does it originate?
A very high grade Glioma
Develops in the Pons
The Pons is the area of the brain stem responsible for critical functions; breathing, sleeping, blood pressure
DIPG symtoms
Abnormal eye alignment
Weakness of facial muscles/ facial asymmetry
Arm/ leg weakness
Unstable balance
DIPG management
No cure! RT
Surgery not always an option due to position
Chemotherapy drugs are not shown to be effective, no increase in overall survival than RT alone.
TRIALS - ACVR1 gene
DIPG prognosis
90% mortality within 18 months of diagnosis
2 year survival is extremely rare
Most common 0-4
Acute Lymphoblastic Leukaemia
Retinoblastoma
Neuroblastoma
Wilm’s tumor (seldom present at birth)
Hepatoblastoma
Embryonal rhabdomyosarcoma
Most common 5-9
Acute Lymphoblastic Leukaemia
Retinoblastoma
Medulloblastoma
Ewing’s sarcoma
Juvenile pilocytic astrocytoma (infratentorial)
Most common 10-14
Hodgkin’s disease
Osteosarcoma
Alveolar rhabdomyosarcoma
Papillary carcinoma of the thyroid
