Paeds Malignancy Flashcards
what is the most common childhood cancer?
acute lympoblastic leukaemia
what is are the top three most common childhood cancers?
leukaemia
brain tumours
lymphoma
The presentation of paediatric malignancy can be from 3 aspects.
- mass
- mass pressure local effects
- disseminated disease
A girl is on chemo. She has fever with neutropenia. This needs to be investigated and treated urgently. What 4 opportunistic infections are common?
pneumocystis jiroveccii
aspergillus
candida
coagulase -ve staph from cath
Staph aureus is coagulase positive. Coagulase negative staph often cause nosocomial infections. Give me 2 examples of coagulase …
staph epidermidis
staph saprophyticus
What is a PICC line?
peripherally inserted central catheter
What things might be involved in supportive care for child with cancer?
fertility preservation
central venous catheter
psychosocial support
Give me some side effects of chemo.
bone marrow suppression gut mucosal damage nausea + vomiting anorexia alopecia
Side effect of vincristine?
neuropathy
Side effect of cisplatin?
deafness
Side effect of cyclophosphamide?
haemorrhagic cystitis
What is the most common leukaemia for kids?
acute lymphoblastic leukaemia (80%)
80% of kids’ leukaemia is acute lymphoblastic leukaemia. Remainder are…
acute myeloid leukaemia
What are the myeloid cells?
granulocytes
megakaryocytes
RBCs
monocytes
What are the lymphoid cells?
T cells
B cells
What makes a leukaemia ‘acute’?
> 20% blasts in bone marrow
Crowding of blasts in acute leukaemia leads to marrow failure and infilitration. What are the consequences of marrow failure for the patient?
anaemia
bleeding
infection
BONE PAIN!
Crowding of blasts in acute leukaemia leads to marrow failure and infiltration. What are the consequences of infiltration for the patient?
CNS - headaches, vom, CN palsies
lymphadenopathy
hepato spleno megaly
(testes enlargement)
In acute leukaemis blasts build up because they’re not differentiating. They crowd and spill over into bloodstream and other parts of body, which is called?
infiltration
Give me two GENERAL symptoms of acute leukaemia.
malaise
anorexia
What would show up on the blood film in acute leukaemia?
blast cells
FBC sometimes abnormal
Give me two risk factors for acute leukaemia.
Down’s
Radiation
What is the key investigation for classifying acute leukaemia?
BONE MARROW ASPIRATE
What other investigations would you do once you’ve confirmed acute leukaemia from bone marrow aspirate?
clotting screen
LP
CXR
What might you be looking for on CXR in acute leukaemia?
mediastinal mass
Why do you use allopurinol as part of supportive care in acute leukaemia?
prevent hyperuricaemia
when therapy causes rapid lysis of cells
What might supportive care of acute leukaemia involve?
- transfuse for anaemia
- transfuse plt.s
- treat infection
- allopurinol
- hydration
Transfuse for anaemia Transfuse plts Treat infection Allopurinol Hydration
is all supportive care for
acute leukaemia
There are four steps of chemo for acute leukaemia. What are they?
- remission induction
- intensification
- intrathecal chemo
- maintenance chemo
What drugs are involved in remission induction for acute leukaemia?
combination chemo + steroids
What is the purpose of remission induction for acute leukaemia?
eradicate leukaemic blasts
+ restore marrow function
What is the purpose of intensification chemo for acute leukaemia?
to consolidate remission
What is the purpose of intrathecal chemo for acute leukaemia?
prevent CNS relapse
A 9 yr old girl has been through remission induction, intensification, and intrathecal chemo for acute lymphoblastic leukaemia. How long will she need maintenance chemo?
2 yrs
An 8 yr old boy has been through remission induction, intensification, and intrathecal chemo for acute lymphoblastic leukaemia. How long will he need maintenance chemo?
3 yrs
How long do boys and girls need maintenance chemo in acute lymphoblastic leukaemia?
girls - 2 yrs
boys - 3 yrs
What drug do you give for Pneumocystic jirovecii prophylaxis during chemo?
co-trimoxazole
Why would you give a child co-trimoxazole during chemo?
co-trimoxazole prophylaxis
How do you manage a relapse of acute leukaemia?
high dose chemo (+ radio)
then BONE MARROW TRANSPLANT
What are the two types of acute leukaemia?
acute lymphoblastic leukaemia (ALL)
acute myeloid leukaemia (AML)
Give me 5 indicators of prognosis of acute leukaemia?
- age
- tumour load (WCC)
- genetic abnormalities in malig cells
- speed of response to initial chemo
- “minimal residual disease assessment”
What is the “minimal residual disease assessment” in prognosis of acute leukaemia?
are there still tumour markers after remission induction?
What ages are bad for acute leukaemia prognosis?
<1 or >10
What are the survival rates for acute lymphoblastic leukaemia?
> 90%
Most brain tumours in kids are….
primary and infratentorial
below tentorium cerebelli
What child brain tumour in cortex?
astrocytoma
What child brain tumour near pituitary tissue?
craniopharyngioma
What child brain tumour in brainstem?
brainstem glioma
What child brain tumours are cerebellar?
medulloblastoma
ependymoma
Remnant of Rathke’s pouch. Not malignant but invasive. ?
Craniopharyngioma
What is the leading cause of childhood cancer deaths in UK?
brain tumours
even tho acute leukaemia is most common cancer
Infant with developmental delay, increase in head circumference, and bulging fontanelle. Suspect?
brain tumour
Child/adolescent with recurrent early morn headache, blurred/double vision, worse school performance, poor growth and delayed puberty. Suspect?
brain tumour
Some symptoms of brain tumour specific to child / adolescent?
recurrent early morn headache
blurred / double vision
worse school performance
poor growth / delayed puberty
Some symptoms of brain tumour specific to infant?
developmental delay
increase in head circumference
bulging fontanelle
Afebrile seizures, vomiting, abnormal eye movements, behaviour change, lethargy, and problems with balance/coordination are all symptoms of…
brain tumour
Name some general brain tumour symptoms for all ages
Afebrile seizures vomiting abnormal eye movements lethargy behaviour change probs w balance / coordination
This tumour causes seizures, focal neuro deficit, and hemiplegia. It makes up 40% of child brain tumours and is found in the cortex.
astrocytoma
~~~~ glioblastoma multiforme
What are the 2 consequences of craniopharyngioma (benign but invasive)?
BITEMPORAL HEMIANOPIA
PITUITARY FAILURE
(growth failure,, weight gain, diabetes insipidus)
Why does craniopharyngioma cause bitemporal hemianopia?
presses on optic chiasm
Truncal ataxia and decreased coordination. What kind of brain tumour might this be?
cerbellar
medullloblastoma, ependymoma
Does persistent back pain in children warrant MRI?
yes
might be spinal tumour
Which brain tumour commonly metastasises to spine?
medulloblastoma (cerebellar)
lots have spinal mets at diagnosis
Cranial nerve defects. What kind of brain tumour might this be? Would you do a biopsy?
brainstem glioma
biopsy too hazardous
What is the prognosis like for brainstem glioma?
<10% survival.
palliative radiotherapy.
Back pain. Arm/leg weakness. Bladder/bowel dysfunction. What kind of tumour might this be?
spinal
can be primary astrocytoma in spine or mets from medulloblastoma
What are the investigations for a suspected brain tumour in a chid?
MRI head + spine
MR spectroscopy
(LP to check for CSF mets for staging but only carefully on neurosurgical advice)
tissue diagnosis from surgery - biopsy
When would you do LP for child with suspected brain tumour?
only on neurosurgical advice bc risky if raised ICP!
used to check for CSF mets for staging
What tends to be the treatment for brain tumour?
surgery
(biopsy, treat hydrocephalus, resection is poss)
+/- radio + chemo
(age + stage)
What warning signs are included in HeadSmart campaign for diagnosing childhood brain tumours?
- EYES new squint / nystagmus / papilloedema
- HEAD ache early morn / worse lying down
- VOM early morn effortless vom
What warning signs are included in HeadSmart campaign for diagnosing childhood brain tumours?
- EYES new squint /nystagmus /papil
- HEAD ache early morn /worse lying down
- VOM early morn effortless vom
The third commonest childhood cancer is lymphoma. What actually is lymphoma?
uncontrolled proliferation of lymphocytes (B + T cells)
Which lyphoma mainly affects ADOLESCENTS?
Hogkins.
also 50yr olds - bimodal
Which lymphoma mainly affects children (as opposed to adolescents)?
non-Hodgkins
Which is the commoner lymphoma?
NON hodgkins
Which cells distinguish Hodgkins from Non Hodgkins?
Reed-Sternberg
Painless cervical lymphadenopathy. What FLASHES across your mind?
Hodgkins lymphoma
hard rubbery big
What staging is used for Hodgkin’s lymphoma
ann arbor
I - one lymph node; IV- extranodal
What are the symptoms of Hodgkin’s lymphoma?
painless cervical lymphadenopathy.
SOB, chest pain, cough
(long Hx)
(B symps rare in kids - weight loss, fever, night sweats)
16 yr old presents with painless cervical lymphadenopathy and breathlessness. Hodgkin’s lymphoma should be on your list of differentials. What investigations for that?
FBC, ESR
lymph node biopsy - diagnostic
CT chest abdo pelvis
bone marrow biopsy - staging
What is it that causes the SOB, chest pain and cough in Hodgkin’s lymphoma?
mediastinal lymphadenopathy
17 yr old presents with painless cervical lymphadenopathy and cough. After FBC + ESR to exlcude other causes, you do lymph node biopsy. If it’s Hodgkin’s lymphoma, what would you expect to see on histology?
Reed-Sternberg cells
multinucleated giant cells
A malignant tumour of the lymphatic system that is characterised histologically by the presence of multinucleated giant cells (Reed-Sternberg cells). What’s this?
Hodgkin’s
name some risk factors for Hodgkin’s lymphoma
EBV
immunosuppression, HIV
name some risk factors for Hodgkin’s lymphoma
EBV
immunosuppression, HIV
What kind of scan is good to monitor response to treatment in Hodgkin’s lymphoma?
PET scan
Investigations into a teenager with Hogkins lymphoma show that the malignancy remains in one lymph node only. What Ann Arbor stage is this?
stage I
What % of kids with Hogkin’s lymphoma are cured?
80%
What is the treatment for Hodgkin’s lymphoma?
COMBINATION CHEMO (+/- radio)
What % of kids with Non Hogkins lymphoma survive?
80%
Tell me about the long term toxicity of radiotherapy for Hodgkins lymphoma.
leukaemia risk
Breast, lung and thyroid Ca risk
What is primary lymphoid tissue?
bone marrow
What is secondary lymphoid tissue? (x4)
spleen
blood
lymph nodes
MALT
What are B lymphocytes and where are they.
plasma cells
memory cells
lymph noes
What are T lymphocytes and where are they.
T helper
cytotoxic T
thymus
Non-Hogkins lymphoma is the commoner lymphoma. It’s uncontrolled proliferation of which lymphocytes mainly?
B lymphocytes
B cell non hogkin lymphoma can cause lymphadenopathy in head neck and abdo. How can this manifest as symptoms?
bowel obstruction (palpable mass, intussusception)
T cell non hodgkin lymphoma can cause lymphadenopathy in MEDIASTINUM. What symptoms can this lead to?
SVC obstruction
SOB
facial swelling
acute leukaemia starts off in the marrow and infiltrates the nodes. Whereas non hogkins lymphoma starts off in the nodes and infiltrates the marrow. yes?
yes
What is the treatment for non hodgkin lymphoma?
MULTIAGENT CHEMO
6yr old presents with lymphadenopathy in head and neck, and bowel obstruction with palpable mass. You suspect non hodgkin lymphoma. What investigations do you do?
lymph node biopsy
CT chest abdo pelvis
bone marrow biopsy
Apart from ‘endemic’ Burkitt’s lymphoma (in areas of Africa with high EBV and chronic malaria), what other types of Burkitt lymphoma are there?
sporadic
imm def associated
Which part of body does advanced Burkitt’s lymphoma affect?
jaw
What is treatment for Burkitt’s lymphoma?
multiagent chemo
What cells uncontrolled proliferation in Burkitts lymphoma?
B lymphocytes
its NHL
What is neuroblastoma?
primary tumour anywhere along sympathetic chain, esp neural crest tissue in adrenal medulla
affects <5s
Why is neuroblastoma unsusual?
it can spontaneously regress?!
What is most common symp of neuroblastoma?
abdo mass
adrenal in origin
(if paravertebral - cord compression)
What is the key investigation for suspected neuroblastoma?
high VMA / HVA
catecholamine metabolites
Which radioactive isotope injection scan maps metastases in neuroblastoma?
MIBG scan
What’s Wilm’s tumour?
nephroblastoma
<5yrs
How does nephroblastoma present?
abdo mass
haematuria
otherwise welll
Ix and Rx for Wilm’s?
US , CT/MRI - renal mass
chemo then nephrectomy
Ewing and osteosarcoma affect bones, whereas rhabdomyosarcoma affects…
muscle
Which vaccines should be given to children receving treatment for Hodgkin’s lymmphoma?
penumococcal vaccine
flu jab
Child <3yrs with squint and white pupilllary reflex (absentt red reflex). Which cancer do you suspect?
retinoblastoma.
laser therapy
Langerhans cell histiocytosis a.k.a
eosinophilic granuloma
histiocytes are dendritic antigen presenting cells
Abnormal prolif of histiocytes, causing bone lysis and diabetes insipidus. Rare AF. What’s this?
Langerhans cell histiocytosis
What age kids does acute lymphoblastic leukaemia affect?
ALL (all)
List 6 long term effects of paediatric malignancy / chemo / radio / surgery.
specific organ dysfunction poor growth infertility second cancer neuropsychological social / educational