Paeds malignancy, haem, derm 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?
Posterior fossa
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 and enhancing
What mutation is most common in pilocystic astrocytoma?
BRAF
Recall the 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 MRI, normal exam, papilloedema, 14y/o, high BMI
Which investigation is most appropriate in investigating an astrocytoma?
MRI
What is the specialised member of the MDT in childhood cancer cases?
CLIC Sargent (cancer and leukaemia in children social worker)
What is the first-line management for astrocytoma?
Surgery
Which CNS tumours can be treated with radiotherapy?
Gliomas and 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 will be seen on CXR in ALL?
Enlarged thymus
What results of BM biopsy would be diagnostic of ALL?
> 20% blasts in BM/ peripheral blood
Immunological and cytological characteristics
What should be the immediate management of a high WCC in ALL?
TLS needs to be reduced: allopurinol and 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 (if LP is negative initially)
Molecular treatment
Transplantation