Paeds malignancy Flashcards
What type of tumour are the majority of CNS tumours in children?
Astrocytoma (pilocystic astrocytoma = most common)
Where are astrocytomas found?
Cerebellum
Where are medulloblastomas found?
Cerebellum
Where are ependymomas found?
4th Ventricle
What is the WHO grade for pilocystic astrocytoma?
Grade I
What syndrome is associated with pilocystic astrocytoma?
Neurofibromatosis 1
How will pilocystic astrocytoma appear on MRI?
Cerebellar
Well-circumscribed
Cystic
Enhancing
What mutation is most common in pilocystic astrocytoma?
BRAF
Recall the 7 signs and symptoms of pilocystic astrocytoma
Headaches (worst in morning)
Gait problems, co-ordination problems
Visual changes
Vomiting on waking
Failure to thrive
Behaviour/ personality change
Later sign = papilloedema due to raised ICP
What is the typical picture of benign intracranial HTN (not malignant cause)?
Normal exam
14y/o
High BMI
Normal MRI
Papilloedema
Which investigation is most appropriate in investigating an astrocytoma?
MRI
What histopathological feature characterises pilocystic astrocytomas?
Rosenthal fibres: corkscrew eosinophilic fibre bundles
What is the specialised member of the MDT in childhood cancer cases?
CLIC Sargent (cancer + leukaemia in children social worker)
What is the most common malignant brain tumour in children?
Medulloblastoma
What is the first-line management for astrocytoma?
Surgery
Which CNS tumours can be treated with radiotherapy?
Gliomas + metastases
Which CNS tumours can be treated with chemotherapy?
High grade gliomas
What % of ALL is B lineage vs T lineage?
85% B
15% T
What % of leukaemia is ALL vs AML in children?
80% ALL
20% AML
Recall the signs and symptoms of ALL
BM failure (anaemia, thrombocytopaenia, neutropaenia)
Local infiltration: lymphadenopathy, hepatosplenomegaly, bone pain, testes/ CNS
Leukaemia cutis = petechial rash on face + trunk
What is raised in tumour lysis syndrome?
Potassium
LDH
Phosphate
Uric acid
What may be seen on CXR in ALL?
Enlarged thymus (Mediastinal mass)
What investigations are used for ALL?
FBC: Thrombocytopenia, Anaemia, abnormal WCC
CRP
Coagulation profile
U+Es: TLS
Peripheral blood smear: >20% blasts
BMA: >20% blasts
Immunophenotyping using flow cytology
What results of BM biopsy would be diagnostic of ALL?
> 20% blasts in BM/ peripheral blood
Immunological + cytological characteristics
What should be the immediate management of a high WCC in ALL?
TLS needs to be reduced: allopurinol + hyperhydration
How many years of chemotherapy are necessary in ALL?
2-3 years
Boys are treated for longer because testes are a site of accumulation of lymphoblasts
What therapies can be given alongside chemotherapy in ALL?
CNS-directed therapy
Molecular tx
Transplantation
What types of molecular treatment are available for ALL?
Imatinib (TK inhibitor) for Ph +ve cases
Rituximab (monoclonal antibodies against CD20 for B cell depletion)
What age group are NHL/HL most common in?
NHL = childhood
HL = adolescence
Which of NHL/HL is more likely to be localised to one nodal site?
Hodgkin’s lymphoma
Compare the spread of HL vs NHL
HL = spreads contiguously to adjacent LNs
NHL involves multiple sites + spreads sporadically
Recall the signs and symptoms of HL
Painless cervical lymphadenopathy
Painful on drinking alcohol (in 10%)
Mediastinal mass
B Sx uncommon, even in advanced disease
Recall the investigations and results that are standard for HL
LN biopsy: Reed Sternberg cells
PDG-PET/ CT staging: Ann Arbor
Bloods: FBC, ESR, LFTs, LDH, Alb (prognostic markers)
Immunophenotyping (CD30, CD15 - diagnostic markers)