Oncology Paeds Flashcards
most common solid tumour in children
brain tumour
top 2 most common cancers in children
- leukaemia
- brain tumour
common location of brain tumours
50% posterior fossa
50% glioma
3 main paeds CNS tumours
astrocytoma
ependymoma
medulloblastoma
2 main branches of CNS tumours
glial - astrocytoma and ependymoma
neuroectodermal
astrocytoma grading
1 - 4
1 = pilocytic (essentially benign)
4 = glioblastoma
definition: ependymoma
glial but originate from ependymal lining of vernticles (lateral or 4th)
defintion: medulloblastomas
primitive neuroectodermal tumour
found in medulla oblongata usually
most common brain tumour
medulloblastoma
type of tumour in cerebellum (3)
astrocytoma, ependymoma, PNET
type of tumour in brainstem (1)
astrocytoma
type of tumour in hypothalamus/pineal
astrocytoma, PNEY, NGGCT< teratoma, dysgerminoma
type of supratentorial tumour
astrocytoma, PNET, ependymoma
syndromes increasing risk of brain tumour
neurofiromatosis type 1
tuberous sclerosis
von-hippel lindau
Li Fraumeni syndrome
Turcot syndrome
tumour associated with neurofibromatosis type 1
optic nerve glioma
tumour associated with tuberous sclerosis
subependymal giant cell astrocytoma
associations with brain tumours
ionising radiation
HIV, ataxic telangectasia
clinical presentation of brain tumours (8)
vomiting
headache
changes in personality
squint
reduced school performance
growth failure
seizures
developmental delay
aetiology: PNT tumours
arise from primitive neuroepithelial cells
Metastasis PNT
invade locally
metastasise in subarachnoid space
PNT treatment (3)
surgery
post-op craniospinal radiothetrapy
post-radio chemotherapy
Radiotherapy side effects in <4y/o
severe hearing loss
visual problems
ambulatory problems
tx: ependymoma
surgery
radiotherapy
(no chemo if >3y/o)
grading of glial tumours
pilocytic (low)
low grade (low)
anaplastic (intermediate)
glioblastoma multiforme (high)
sites of glial tumours
optic nerve
hypothalamic
thalamic
cerebellar
brain stem
tx: low grade glioma
watch adn wnd
surgery
watch and wait
if not able to operate - ?chemo and radio
tx: high grade glioma
palliative
temozolamide to reduce progression
effects of cancer therapy in children
endocrine - growth, puberty, thyroid, fertility
cognitive - learning and psychosocial
neurological
hearing - radiotherpy and cisplatin
2ary melignant neoplasms
cardiac
leukaemia
most common leukaemia in children
acute lymphoblastic leukaemia (ALL)
leukaemia
other types of leukaemia common in children
AML - acute myeloid leukaemia
ANLL - acute non-lymphocytic leukaemia
peak ages of leukaemia in children
ALL - peaks 2-5 y/o
AML - <2 y/o
pathyphysiology of leukaemia
bone marrow cancer
genetic mutation in one of precursor cells in bone marrow causes excessive production of single type of abnormal WBC
excessive production of one causes suppression of other lines - PANCYTOPENIA (low RBC, WBS and platelets - anaemia, luekopenia and thrombocytopenia)
investigations: leukaemia
FBC
bone marrow biopsy
coagulation screen (DIC - disseminated intravascular coagulation)
lumbar puncture (disease in CSF)
CXR (mediastinal mass - characteristic of T-cell sdisease)
risk factors: leukaemia
radiation exposure (AXR in prepgnancy)
Down’s syndrome
Kleinfelter’s
Noonan
Fanconi’s anaemia
Presentation: leukaemia (12)
persistent fatigue
unexplained fever
failure to thrive
weight loss
night sweates
petechiae and abnormal brusing
unexplained bleeding
lymphadenopathy
bone or joint pain (unexplained and persistent)
hepatosplenomegaly
abdominal pain
pallor
diagnosis: leukaemia
immediate referral to specialist
FBC within 48 hours
blood film - can show blast cells
bone marrow biopsy
lymph node biopsy
staging investigations: leukaemia
CXR
CT scan
lumbar puncture
genetics analysis and immunophenotypung
management: leukaemia
chemotherapy mainstay
radio, bone marrow transplant or sugery are other options)
complications of chemotherapy
endocrine - failure to thrive, puberty, growth, fertility
immunodeficiency
secondary malignancy
cadiotoxicity
neurotoxicity
infections and immunodeficiency
3 core symptoms ALL
recurrent inffections (neutropenia)
anaemia (pallor and fatigue)
thrombocytopena (purpuric/bruising)
supratentorial tumour
astrocytoma
midline brain tumour
cranipharygioma
cerebellar brain tumour
medulloblastoma, astrocytoma or ependymoma
brainstem brain tumour
brainstem glioms
spinal cord tumour
astrocytoma, ependymoma
clinical features with cupratentorial (cortex tumours) (3)
seizures
hemiplegia
focal neurological signs
clinical features of midline tumours
visual field loss (bitemporal hemianopia)
pituitary failure (growth failure, diabetes insipud, weight gain)
clinical features with cerebellar and IVth ventricle tumour
truncal ataxia
co-oordination difficulties
abnormal eye movements
clinical features of brainstem tumours
cranial nerve defects
pyramidal tract signs
cerebellar signs (ataxia)
no raised ICP
4 year old refusing to walk, unable to climb stairs, qsquint and facial asymmetry with drooling
brainstem glioma
10 y/o headaches, vomiting, poor groth, struggling to see board at school
craniopharygioma
3 year old vomiting in mornings, unsteady on feet and new onset convergent squint
medulloblastoma
14 y/o aggressive behavious, headaches, seizures
astrocytoma - glioblastoma multiforme
investigations for brain tumours
MRI scan
management of brain tumours
surgery usually 1st lune
aetiology: neuroblastoma
neural crest tissue in adrenal medulla and aympathetic nervous system
common age of neuroblastoma
<5y/o
clinical features: neuroblastoma
abdominal mass
primary tumour usually in sympathetic chain between neck and pelvis
tumour mass often large and croses midline - affecting major blood vessels and lymph nodes
spinal cord compression
clinical features metatstatic neuroblastoma
bone pain
bone morrow suppression
weight loss
malaise
symptoms of neuroblastoma
palloe
weight loss
abdominal mass
hepatomegaly
bone painlimp
investigations: neuroblastoma
raised urinary catecholamine metabolite levels
confirmatory biopsy
oncogene predictive of neuroblastoma prognosis
MYCN
management: neuroblastoma
surgery alone if no mets
chemotherapy if metastatic, stem cell, surgery and rasiotherapy
def: Wilm’s tumour
nephroblastoma
originates from embryonal renal tissue and is most common renal cancer of childhood
age of presentation with Wilm’s
<5y/o
(v rare after 10 y/o)
clinical feat: Wilm’s tumour
large abdominal mass
haematuria
otherwise well
RF: nephroblastoma/Wilm’s tumour
Beckwith-Wiedemann syndrome
investigations: Wilm’s
USS
and/or CT/MRI - intrinsic renal mass
management: wilm’s tumour
chemotherapu and delayed nephrectomy
def: retinoblastoma
malignant tumour of retinal cells
aetiology of retinoblastoma
bilateral = all hereditary (chromosome 13)
dominant pattern of inheritance but incomplete penetrance
common age of presentation retinoblastoma
<3 y/o
clinical features: retinoblastoma
white puilary reflex
squint
investigations: retinoblastoma
MRI and examination under anaesthetic
treatment: retinoblastoma
chemotherapy (esp bilateral) to shrink tumour
laser
2 main types of bone cancer
osteosarcoma
Ewing sarcoma
clinical features: bone cancer
limbs most common site
persistent localised bone pain
mass on limbs
mainly otherwise well
investigation: bonce cancer
plain X-ray - destruction and variable periosteal new bone formation
MRI
bone scan
CT chest for lung mets
bone marrow sampling
what is often seen on imaging of Ewing sarcoma
substantial soft tissue mass
management: bone tumour
chemotherapy before surgery
radiotherapy in Eqing sarcoma
