Oncology Flashcards
Leukaemia When does ALL normally present How does it present Are there any signs What should be looked at on bone marrow aspirate (4 things) What indicates high risk
Preschool- peaks 4 yrs
Quick 2-3 weeks pallor, fatigue, weight loss, low grade fevers, irritable
Enlarged testis, chloromas
Morphology- myeloid or lymphoid
Immunoflorescence-CD markers
Cytogenetics- translocations
Enzyme studies
Under 1 or over 10, Philadelphia chromosome, more mature phenotypes, high leukocyte count (>50)
What translocation gives rise to the Philadelphia chromosome?
Which other translocation gives poor prognosis in ALL
9: 22
11: 23
What syndromes are associated with leukaemias
Downs
Wiscott Aldrich
Ataxia teliengeictasia
Fanconi anaemia
How long is the standard treatment of ALL?
Outline the phases
What is MRD?
2 yrs girls, 3 boys
Induction- weekly vincristine and IT Methotrexate, daily steroids, once PEG
Consolodstion- clearing cns disease
Intensification
Maintenance- daily mcp and daily mtx
Minimal residual disease- do a bone marrow after 28 days treatment to see if <5% blasts after induction
What translocation gives good prognosis in ALL
Which trisomy “?
12:21
Trisomy 4 and 10
What are the features of tumour lysis syndrome
What are risks for it
Hyperuricaemia phosphataemia and kalaemia
Hypo ca and mg
Leukaemia and lymphomas
Hyper hydrate
Allopurinol
What is the association of Down’s syndrome and leukaemias (3 things)
Increased risk 20 times normal
Do better in AML
Can get myelodysplastic syndrome from infancy so high risk of aml in the future
How does AML differ from ALL?
How does treatment differ?
Sicker, older, more likely to have signs (gum hypertrophy and blueberry muffin lesions)
Need to look for a bone marrow donor sooner
What translocations show good prognosis in AML
Which monosomy is bad
8:21, 15:17
7
Which subtype of AML has a specific treatment?
What is that treatment?
Juvenile APML
Retinoic acid
What is juvenile myelomonocytic leukaemia
What is it associated with?
What is the prognosis
Is it associated with Philadelphia chromosome?
Juvenile cml
Noonans syndrome and nf1
Poor if not noonans
No!
Lymphoma
Hodgkin and non Hodgkin- what are the features regarding:
- number of nodes
- how they spread
- Extra nodal disease
Which subtype is more likely to involve mesenteric nodes
Hodgkin- single node, spread orderly, rare
NHL- multiple nodes, more sporadic spread, lots of extra nodal disease
NHL
Hodgkin lymphoma
How does it present?
What type of cells are seen on biopsy? Are they pathogonomic?
What staging system is used? What does it look for?
What gives a poor prognosis?
What is the most common histiological subtype ?
Slow onset, painless rubbery lymphadenopathy, B cell symptoms
Reed sternberg cells. No!
Ann arbour- LN involvement above or below diaphragm
B cell symptoms, Hugh tumour bulk, High stage
Nodular sclerosing
NHL
What is the most common subtype in children?
How do they present?
What abdominal pathology might make you think of it in an older child?
What is the classification staging?
What blood test monitors tumour burden?
Burkitts Faster, lymphadenopathy SVC obstruction Intussussecption St Jude LDH
Brain tumours
What is the tentorium? What are the most likely tumours -<1 -1-10 yrs -over 10
Dura mater that separates the hemispheres and cerebellum
Supratentorial
Infratentorial
Supratentorial
Brain tumours
What are signs of supra and infratentorial tumours?
What is diencephalic syndrome?
What might Torticollis suggest?
What is parinaud syndrome?
Signs of raised ICP- headache seizures weakness behaviour changes
Signs of brainstem- vomiting, incoordination, visual change, gait change, papilloedema
Tumours in thalamus or hypothalamus- increased appetite and irritable, cahexia and emaciated
Cerebellum herniating
Pineal tumour- paralysis of upward gaze
Brain tumours
What is the most common histiological subtype?
What syndrome can it be associated with?
What is the prognosis?
Where is it found and what does it look like
Pilocytic astrocytoma (low grade glioma)
NF 1
Good- 80-90%
Cerebellar- cystic appearing
Brain tumours
What is an ependynoma
What is a medulloblastoma Where are they normally found? When does they present What is seen on imaging and histology What are good signalling pathways
Tumour from the lining of the ventricles
Primitive Neuroendocrine tumour Cerebellum Primary school age Non calcified, contrast enhancing mass Homer Wright rosettes WNT sonic hedgehog
ATRT
What are they
How do they present
What is the prognosis
What is the youngest age you can have radiotherapy?
What genetic malformation are they associated with?
Germ cell tumours Rapid onset usually <2 years Poor 3 years IN11- complete peerage partial deletion of ch 22
Neuroblastoma
What is it?
What 2 syndromes is it associated with
What gene shows bad prognosis
Tumour of the peripheral nervous system- neural crest cells
Beckwith wiedemann NF1
NNYC
Neuroblastoma
What subtype can have a very good prognosis
How do they present
M4 s
Spontaneously regresses
Widespread nodes, massive liver
Neuroblastoma
How do they typically present What is unique about the mass (2 things) Where is the mass normally found What signs might be present How may a paraneoplastic syndrome present?
Large abdominal mass, weight loss, night sweats and fevers
Crosses the midline, hard craggy
Adrenal
Raccoon eyes and Horner syndrome, urinary or bowel obstruction
Opsomyoclonus, ataxia, diarrhoea and hypertension
Neuroblastoma What is the best screening test What bloods monitor prognosis What is seen on ct What specialised imaging technique is used. What is needed to be given too
Vmas&HVAs
Ferritin and ldh
Calcification of the mass
MIBG scanning. Idodine
Neuroblastoma
What immune therapy is given
Anti GD 2 (glycoprotein)
Neuroblastoma
Does it cause renal calyceal dilatation
No!
Wilms
What genetic mutation will outline a bad prognosis
What is Denys Dash syndrome
What gene is associated with Denys drash
P53
Wilms plus abnormal male genitalia and nephropathy
WT1
Wilms How does it normally present Are they sick at diagnosis normally What is the best initial investigation. Where does it metastasise you Which renal tumours are more likely to met to the brain
Single smooth abdominal mass.
Hypertensive
No!
Abdominal ultrasound
Lungs and liver
Clear cell and rhabdoid
Retinoblastoma What gene is mostly involved? which chromosome is it coded on? What is the pattern of inheritance? What is the difference between inherited and sporadic forms?
RET 1 13 AD Sporadic- usually unilateral Hereditary- bilateral- one gene hit from birth, second sporadic mutation
Retinoblastoma
How does it present in the developed world
How else can it present?
What type of tumour is it?
Squint
Leukocoria
Pain, unequal pupils, mass, fevers, visual loss
Small blue round cell
Rhabdomyosarcoma Where is the most common site? What are three other common sites? What type of tumour is it? Which syndrome is it associated with? How can it present What two translocations are common
Head and neck, trunk, limbs, GU tract Small round blue cell Li Frauneni syndrome Painful mass, longer than expected healing after an injury, bleeding or mass from vagina. Stridor and cough 1:13 2:13
Bone tumours When are they most likely to happen Ewing’s and osteo: - location -X-ray findings - symptoms -genetics - Radiotherapy?
Adolescents
Ewing’s- diaphysis- pelvis chest wallBones and soft tissues. Lytic. Systemic upset. Ch 22 translocations. Yes of large or not fully ressected
Osteo- metaphysic, bone only- femur and tibia. Sunburst pattern. Pathological fractures or painful, slow to heal fractures. Retinoblastoma & li fraumeni. No
Osteosarcoma
Where does it met to
What gives the biggest prognostic indicator?
Chest
Level of necrosis with pre op chemo
Liver tumours
What is the most common type in children?
Which lobe do they commonly involve?
How do they present?
Which blood test will show it? How might that test also be useful?
Do they typically need chemo? Why?
Hepatoblastomas R lobe Large asymptomatic abdominal mass AFP. monitors response to chemo No >85% liver can be ressected with no complications
Germ cell tumours Are most gonadal or extra gonadal Where is the most common site What is the most likely histiological type? What will raised alpha FP show What type of chemo is used
Extra (gonadal in teens) Sacrococcygeal Teratomas If it’s malignant Platinum based
What are the three most common cancers to have secondary malignancies?
Hereditary retinoblastoma
Hodgkin lymphoma
Soft tissue sarcoma
What are 5 small blue round cell to tumours?
Ewing’s Rhabdomyosarcoma PNET Neuroblastoma NHL
What are the 5 points of the cell cycle?
Where does the p53 gene work
G0 G1 S G2 M
G1
Which drugs work in M phase
Where in the cell cycle does asparginase work?
What works in the S phase
What acts in the G phase
Vincristine and vinblastane
G1
Methotrexate and 6MCP. Ara C
Bleomycin and etoposide
Chemo side effects
Methotrexate
Bone marrow suppression
Nephro/hepato toxic
Chemo side effects
Cytarabine
Cns side effects and fevers
Chemo side effects
Vincristine
Peripheral neuropathy
Constipation
Fatal if given IT
Chemo side effects
Asparginase
Anaphylaxis
Chemo side effects
Cyclo/ifosfamide
What might help prevent it?
Renal tubulopathy ifos also encephalopathy
MENSA
Chemo side effects
Carboplatin/ cisplatin
Hearing and neuro
Renal impairment
Chemo side effects
Bleomycin
Pulmonary fibrosis
What is a common side effect of etoposide
Allergy
Chemo side effects
Cisplatin
Vomiting ++
What stain on blood film may help identify blasts
MPO- myeloperoxidase
Which chemo has high risk for secondary malignancies
What is the cancer type
Etoposide
AML
How is Philadelphia chromosome positive leukaemia treated
Imatinib or dasatinib- tyrosine kinase inhibitors
When will screening for beckwith wiedemann abdo tumours stop?
When is the highest risk period?
1st decade
1st year of life
Phenotypically what is seen in Bloom syndrome
What tumours are likely
Short, photosensitive rash and T2dm
Leukaemia and lymphoma
What tumours do inactivated p16 and 19 cause
T cell and pre B ALL
How does rasburicase work
Enzyme oxidation to HPO and allantoin
How does carT therapy work
What is the main side effect
What is it used for currently
Engineered T cells to kill cd19 cells
Cytokine storm
Relapsed ALL or primary refractory ALL
Febrile neutropenia
How low do the neuts have to be
What is given for fungal cover
What is given for AML
<0.5
Ambisome
Taz and vanc
Which 2 chemos cause mucositis
What is used to treat it
Doxyrubicin and methotrexate
Fluids and leukovorin rescue
In tumour lysis syndrome, what precipitates in the tubules
“ if urine is alkaline
Uric acid
Calcium phosphate
What is the most common cause of secondary HLH
Ebv
