Paeds Oncology Flashcards
ALL
Patho
- Malignant disorder of lymphoid progenitor cells
- Majority = B-cell origin
- Some are T-cell origin
- Lymphoid precursors proliferate –> replace normal bone marrow cells
ALL
Epidemiology
- Affects boys slightly more than girls
- 2 - 5 years
- Most common cancer in children
ALL
Presentation
- PANCYTOPAENIA = PALLOR, INFECTION, BLEEDING
- Fever (w/o obvious cause)
- Fatigue, dizziness, palps
- Severe/unusual joint pain
- Recurrent/severe infections
- Early satiety (hepato/splenomegaly)
- MEDIASTINAL LYMPHADENOPATHY (painless lumps in neck/axilla/groin
- Dyspnoea
- Headache/irritability/altered mental state
- Bleeding
- Pallor
- Tachycardia/flow murmur
- Petechiae
- Abdo distension
- Testicular enlargement
- Gum hypertrophy
- Cranial nerve palsys
- Leukaemia cutis
ALL
Associations
- Ionising radiation during pregnancy
- Downs syndrome
- Kleinfelters
- Noonans
- Fanconi’s anaemia
ALL
Ix
- FBC - anaemia, thrombocytopaenia, neutropenia
- Blood film = BLAST cells
- Clotting: DIC
- Raised lactic dehydrogenase levels
- CXR: pneumonia/mediastinal mass
- Liver/renal function
- Testicular US - large
- Bone marrow aspirate/biopsy - CONFIRMS DIAGNOSIS –> BLAST CELLS
- LP to assess CNS involvement
- genetic analysis and immunophenotyping (tDT)
ALL
Tx
- Radiotherapy
- CHEMO
o 5 stages: induction, consolidation, interim maintenance, delayed intensification, maintenance - Blood replacement therapy
ALL
Complications
- neutropenic sepsis
- hyperuricaemia
- poor growth
- cancer elsewhere
Neuroblastoma
Patho
- Arise from neural crest tissue in adrenal medulla + sympathetic nervous system
- Cells surrounding tumour produce chemokines (CXCL12)
Neuroblastoma
Epidemiology
< 5 yrs
Neuroblastoma
Features
- Chemokine release: fatigue, fever, weight loss, sweating
- Location-dependent:
o Thoracic - breathing problems
o Neck - Horner’s syndrome
o Spine - Neuro symptoms
o Abdominal mass
o Bones - base of skull –> periorbital ecchymosis (RACOON EYES)
o Bone marrow - fatigue, easy bruising, freq infections
Neuroblastoma
Ix
- Urinalysis - RAISED vanillylmandelic acid (VMA) + homovanillic acid (HVA)
- Bloods
- CT - location + size
- BIOPSY
Neuroblastoma
Tx
- Early + local = surgery
- Late + mets = CHEMO, radio, surgery
- May spontaneously resolve in YOUNG infants
Wilm’s Tumour/Nephroblastoma
Patho
- originates from embryonal renal tissue
- Triphasic nephroblastoma = blastemal, stromal and epithelial cells
Wilm’s Tumour/Nephroblastoma
Genetics and chromosome
+ Epidemiology
- Autosomal dominant
- Mostly idiopathic but sometimes genes WT1 / WT2
- Chromosome 11
- MOST COMMON renal tumour in childhood
- Most = < 5yrs
Wilm’s Tumour/Nephroblastoma
Associated syndromes
WAGR: WT1 and PAX6
- Wilms, Aniridia (absence of iris), Gonadoblastoma (GU malformation), mental Retardation
Dennys Drash syndrome: WT1
+ nephrotic syndrome
+ male pseudohermaphrotism
- Edward’s
- Blooms
Wilm’s Tumour/Nephroblastoma
Presentation
- First 5 years of life
- 95% = unilateral
- Asymptomatic abdominal mass - incidental finding!
- Haematuria
- Hypertension
Wilm’s Tumour/Nephroblastoma
Ix
- USS
- CT/MRI
- FBC, U&Es
- Urinalysis
- AVOID RENAL BIOPSY - MAY MAKE WORSE
Wilm’s Tumour/Nephroblastoma
Tx
- Nephrectomy preceded by chemo
- Radiotherapy for advanced
- 90% = long term survival
CNS tumours
Presentation
- Raised ICP
- Headache - worse lying down
- Vomiting - early morn
- Papilloedema
- Squint
- Nystagmus
- Ataxia
- Personality/behavioural change
CNS tumours
Examples
- Medulloblastoma
- Pilocytic astrocytoma
- Pontine glioma
CNS tumours
Tx
- Surgery: resection, VP shunt
- Chemo: single/combination, many don’t pass BBB so not as effective
- Radio: malignant tumours in older children, post-surgery, in combo with chemo
CNS tumours
ix
- MRI
Retinoblastoma
Genetics + chromosome
- Autosomal dominant
- Chromosome 13
- Average age of diagnosis = 18 months
Retinoblastoma
Features
- Absence of red reflex
- Replaced by a white pupil
- Visual problems
- Strabismus
Retinoblastoma
Tx
- Enucleation is not the only option
- External beam radiation therapy
- Chemotherapy
- Photocoagulation
Retinoblastoma
Prognosis
- Excellent, 90% survive into adulthood
