Paeds malignancy Flashcards by Georgia Leggett

What type of tumour are the majority of CNS tumours in children?

Astrocytoma (pilocystic astrocytoma = most common)

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Where are astrocytomas found?

Cerebellum

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Where are medulloblastomas found?

Cerebellum

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Where are ependymomas found?

4th Ventricle

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What is the WHO grade for pilocystic astrocytoma?

Grade I

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What syndrome is associated with pilocystic astrocytoma?

Neurofibromatosis 1

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How will pilocystic astrocytoma appear on MRI?

Cerebellar
Well-circumscribed
Cystic
Enhancing

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What mutation is most common in pilocystic astrocytoma?

BRAF

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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

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What is the typical picture of benign intracranial HTN (not malignant cause)?

Normal exam
14y/o
High BMI
Normal MRI
Papilloedema

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Which investigation is most appropriate in investigating an astrocytoma?

MRI

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What histopathological feature characterises pilocystic astrocytomas?

Rosenthal fibres: corkscrew eosinophilic fibre bundles

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What is the specialised member of the MDT in childhood cancer cases?

CLIC Sargent (cancer + leukaemia in children social worker)

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What is the most common malignant brain tumour in children?

Medulloblastoma

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What is the first-line management for astrocytoma?

Surgery

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Which CNS tumours can be treated with radiotherapy?

Gliomas + metastases

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Which CNS tumours can be treated with chemotherapy?

High grade gliomas

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What % of ALL is B lineage vs T lineage?

85% B
15% T

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What % of leukaemia is ALL vs AML in children?

80% ALL
20% AML

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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

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What is raised in tumour lysis syndrome?

Potassium
LDH
Phosphate
Uric acid

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What may be seen on CXR in ALL?

Enlarged thymus (Mediastinal mass)

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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

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What results of BM biopsy would be diagnostic of ALL?

> 20% blasts in BM/ peripheral blood
Immunological + cytological characteristics

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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)

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 Extra-nodal involvement B Sx indicate worse prognosis: 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

1. Urgent chemo 2. Monitor only 3. 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

What is the most common primary bone cancer in adolescents?

Osteosarcoma

Where does osteosarcoma tend to occur?

Long bones: 60-75% in the knee

Recall 3 signs and symptoms of osteosarcoma

Pain: worse at night Mass/ swelling- tender Restricted movement/ limp

Give 2 genetic abnormalities give predisposition to osteosarcoma

RB1 abnormality (Retinoblastoma) Li-Fraumeni syndrome (TP53 tumour suppressor gene mutation)

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 Normal XR: does NOT r/o osteosarcoma

What investigations should be done following XR in suspected osteosarcoma?

MRI Biopsy: definitive dx

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% 5y survival

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?

Femur Tibia Fibula Humerus Ileum of pelvis

What translocation is associated with Ewing's sarcoma?

t(11:22)

What are the signs and symptoms of Ewing's sarcoma?

Bone pain: often worse at night Mass Restricted movement of joint Constitutional Sx: fatigue, fever, weight loss

What investigations are appropriate to investigate Ewing's sarcoma?

XR CT/PET/MRI Biopsy: definitive dx

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

How does retinoblastoma often present?

Leukocoria <3y

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 RB1: Tumour suppressor gene inactivation

Recall the 2 key signs and symptoms of retinoblastoma

Negative red reflex: white pupillary reflex (Leukocoria) Strabismus (Squint)

Recall 2 investigations in retinoblastoma

MRI + EUA (examination under anaesthetic)

How should retinoblastoma be treated?

Minimal/ no seeding present: systemic chemo + focal therapy Laser therapy External beam radiotherapy Gross seeding (tumour cells floating in vitreous cavity): Enucleation (eye removal) + Chemo

What is the average age of diagnosis of retinoblastoma?

18 months

What is the prognosis in retinoblastoma?

90% cured + survive into adulthood Many will be visually imparted Significant risk of 2nd malignancy (esp. sarcoma) among survivors of hereditary retinoblastoma

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 5 symptoms of neuroblastoma

Abdo distension Fatigue WL Sweating, agitation Bone pain (mets)

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