Paeds CANCER 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
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 exam
14y/o
High BMI
Normal MRI
Papilloedema
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 + leukaemia in children social worker)
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 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 + 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 (if LP is negative initially)
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 lymph nodes
NHL involves multiple sites + spreads sporadically
Recall the signs and symptoms of HL
Painless cervical lymphadenopathy
B symptoms are uncommon, even in advanced disease
Painful on drinking alcohol (in 10%)
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)
How should HL be managed?
Combination chemotherapy (ABVD) + radiotherapy
Recall the chemotherapy regime used in paediatric HL
ABVD
Adriamycin
Bleomycin
Vincristine
Dacarbazine
Recall the signs and symptoms of NHL
Painless lymphadenopathy +/- compression syndrome
B symptoms: fever, weight loss, night sweats
Recall the useful investigations and their results in NHL
LN/BM biopsy for cytology, histology + immunophenotyping
PDG-PET/ CT staging: Ann Arbor
Bloods: FBC, ESR, LFTs, LDH, Alb (prognostic)
Recall the management approach dependent on the type of NHL
- Urgent chemo
- Monitor only
- Abx eradication: H pylori gastric MALToma
What chemotherapy regime is used in NHL?
R-CHOP
Rituximab
Cyclophosphamide
H: Doxorubicin
O: Vincristine
Prednisolone
What is Burkitt’s lymphoma a type of?
B cell NHL
What are the different variants of burkitt’s lymphoma - and what are their different causes?
Endemic (EBV)
Sporadic (EBV)
Immunodeficiency (HIV)
Why is endemic Burkitt’s so common in Africa?
Chronic malaria reduced EBV resistance
What part of the body does Burkitt’s lymphoma affect?
JAW/ facial bones
What type of cell does burkitt’s lymphoma arise from?
Germinal centre cells
What is the histopathological appearance of burkitt’s lymphoma?
Starry sky
What translocation is most common in burkitt’s lymphoma?
t(8;14)
What is the prognosis for burkitt’s lymphoma?
Really bad as fastest growing human tumour known
Where does osteosarcoma tend to occur?
Long bones: 60-75% in the knee
Recall the signs and symptoms of osteosarcoma
Relatively painless
Mass/ swelling
Restricted movement
Where does osteosarcoma tend to metastasise to?
Lung
What will be shows on XR in osteosarcoma?
Soft tissue calcification - sunburst appearance
Elevated periosteum = Codman’s triangle
What investigations should be done following XR in suspected osteosarcoma?
Biopsy
CT/MRI/PET
How should osteosarcoma be managed?
Specialised sarcoma team
Surgery + chemo
Post-treatment OT/PT/ orthotics etc
What is the surgical management of osteosarcoma?
Limb-sparing surgery +/- amputation
What is the prognosis for osteosarcoma?
Poor: 60% 5 year survival
What is the difference between osteosarcoma and Ewing’s sarcoma?
Osteosarcoma forms bone
Ewing’s sarcoma forms mesenchymal tissue
How does Ewing’s sarcoma appear under the microscope?
Small round blue cell tumour
What is the median age of Ewing’s sarcoma development?
15y
Where does Ewing’s sarcoma develop?
Long bones: arms, chest etc
What translocation is associated with Ewing’s sarcoma?
t(11:22)
What are the signs and symptoms of Ewing’s sarcoma?
Mass/ swelling + bone pain (no pain in osteosarcoma)
Malaise, fever, paralysis (may precipitate osteomyelitis)
What investigations are appropriate to investigate Ewing’s sarcoma?
XR
Biopsy
CT/PET/MRI
What would be seen on XR in Ewing’s sarcoma?
Bone destruction with overlying onion-skin layers of periosteal bone formation
What is the main management of Ewing sarcoma?
Surgery + VIDE chemotherapy + radiotherapy
What management should be given post-treatment for osteosarcoma?
OT, PT, dietician, orthotics/ prosthetics
Which cells have undergone malignant transformation in retinoblastoma?
Retinal cells
What is the main difference in presentation between hereditary and spontaneous retinoblastoma?
Hereditary is nearly always bilateral
Spontaneous is unilateral
Recall the pattern of inheritance of retinoblastoma
Autosomal dominant mutation on chr13
Recall the 2 key signs and symptoms of retinoblastoma
Negative red reflex
Squint
Recall the 2 key investigations in retinoblastoma
MRI + EUA (examination under anaesthetic)
How should retinoblastoma be treated?
Enucleation (eye removal)
Chemotherapy (BL) + laser tx to retina
What is the average age of diagnosis of retinoblastoma?
18 months
What type of tissue and where does neuroblastoma arise?
Neural crest tissue in adrenal medulla + SNS
What is a benign tumour of the same tissue as neuroblastoma originates from called?
Ganglioneuroma
Which gene is associated with a poor prognosis for neuroblastoma?
N-MYC
Where is the mass most likely to be found in neuroblastoma?
Abdomen
Recall the symptoms of neuroblastoma
Systemic:
WL
Pallor
Hepatosplenomegaly
Bone pain
Limp
What additional symptoms of neuroblastoma are likely in people under 2 years?
Sx of metastases (poorer prognosis in under 1s)
What urinary abnormality may be seen in neuroblastoma?
Urinary catecholamine metabolites eg VMA/HVA
How should neuroblastoma be managed?
In very young infants spontaneous regression can occur
If localised primaries with no mets: surgery alone
If metastatic: chemotherapy + radio (with autologous stem cell rescue) + surgery
What are 3 common oncological diagnoses in children?
ALL
Neuroblastoma
Wilms tumour (RCC)
What is tumour lysis syndrome?
An oncological emergency whereby massive tumour cell lysis results in dangerous electrolyte imbalance
What is the management of tumour lysis syndrome?
Allopurinol
Fluid resuscitation (0.9% NaCl + 5% dex) - to preserve renal function as uric acid will crystallise in kidney otherwise
Cross-match for platelet transfusion (small volume)
Chemotherapy
Why is a small volume of platelets given in TLS?
The blood is already quite viscous due to hyperleucocytosis so only a small amount of platelets are given
What are the thresholds for febrile neutropenia?
Over 38 degrees
Less than 0.5 x 10^9 neutrophils
How would febrile neutropenia be treated in children?
Broad spectrum antibiotics with gram negative cover
e.g. piptazobactam and gentamicin
Why would a leukaemia patient be prescribed septrin?
Given a prophylaxis in leukaemia patients to prevent PCP
What is another name for Septrin?
Co-trimoxazole
What is another name for augmentin?
Co-amoxiclav
