Paeds Oncology Flashcards
1
Q
ALL
Patho
A
- Malignant disorder of lymphoid progenitor cells
- Majority = B-cell origin
- Some are T-cell origin
- Lymphoid precursors proliferate –> replace normal bone marrow cells
2
Q
ALL
Epidemiology
A
- Affects boys slightly more than girls
- 2 - 5 years
- Most common cancer in children
3
Q
ALL
Presentation
A
- 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
4
Q
ALL
Associations
A
- Ionising radiation during pregnancy
- Downs syndrome
- Kleinfelters
- Noonans
- Fanconi’s anaemia
5
Q
ALL
Ix
A
- 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)
6
Q
ALL
Tx
A
- Radiotherapy
- CHEMO
o 5 stages: induction, consolidation, interim maintenance, delayed intensification, maintenance - Blood replacement therapy
7
Q
ALL
Complications
A
- neutropenic sepsis
- hyperuricaemia
- poor growth
- cancer elsewhere
8
Q
Neuroblastoma
Patho
A
- Arise from neural crest tissue in adrenal medulla + sympathetic nervous system
- Cells surrounding tumour produce chemokines (CXCL12)
9
Q
Neuroblastoma
Epidemiology
A
< 5 yrs
10
Q
Neuroblastoma
Features
A
- 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
11
Q
Neuroblastoma
Ix
A
- Urinalysis - RAISED vanillylmandelic acid (VMA) + homovanillic acid (HVA)
- Bloods
- CT - location + size
- BIOPSY
12
Q
Neuroblastoma
Tx
A
- Early + local = surgery
- Late + mets = CHEMO, radio, surgery
- May spontaneously resolve in YOUNG infants
13
Q
Wilm’s Tumour/Nephroblastoma
Patho
A
- originates from embryonal renal tissue
- Triphasic nephroblastoma = blastemal, stromal and epithelial cells
14
Q
Wilm’s Tumour/Nephroblastoma
Genetics and chromosome
+ Epidemiology
A
- Autosomal dominant
- Mostly idiopathic but sometimes genes WT1 / WT2
- Chromosome 11
- MOST COMMON renal tumour in childhood
- Most = < 5yrs
15
Q
Wilm’s Tumour/Nephroblastoma
Associated syndromes
A
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