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Phase 3a > Paeds - oncology > Flashcards

Paeds - oncology Flashcards

1
Q

what is acute lymphoblastic leukaemia?

A

it is the most common malignancy in childhood.

It arises from malignant proliferation of pre-B (common ALL) or T-cell lymphoid precursors.

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2
Q

what age group is ALL common in?

A

the peak incidence is at around 2-5 years of age and boys are more commonly affected than girls

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3
Q

how does acute lymphoblastic anaemia present?

A

typically a short history (days or weeks) and with signs and symptoms reflecting pancytopenia, bone marrow expansion and lymphadenopathy

purpura, easy bruising, epistaxis due to thrombocytopenia

hepatosplenomegaly
pallor, ecchymoses or petechiae
fever
fatigue, dizziness, palpitations and dyspnoea
epistaxis, menorrhagia
bone pain - secondary to bone marrow infiltration

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4
Q

what investigations would you perform for ALL and what would you see?

A
  • bone marrow: hyper cellularity and infiltration by lymphoblast’s
  • FBC - anaemia, leucocytosis, neutropenia and/or thrombocytopenia
  • peripheral blood smear - leukaemic lymphoblast’s
  • serum electrolytes
  • chest XR may show evidence of a mediastinal mass, pleural effusion or LRTI
  • RFT - urea may be normal or elevated
  • LFTs - may be normal or elevated
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5
Q

what are the factors that lead to poor prognosis in ALL?

A 
age < 2 years or > 10 years
WBC > 20 * 109/l at diagnosis
T or B cell surface markers
non-Caucasian
male sex
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6
Q

what is acute myeloid leukaemia?

A

it is more common in adults than children but accounts for 5% of all childhood malignancies
it results from malignant proliferation of myeloid cell precursors in the bone marrow, peripheral blood or extramedullary tissues.

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7
Q

what are the features of acute myeloid leukaemia?

A 
anaemia - pallor, lethargy, weakness 
neutropenia - WCC may be very high however functioning neutrophil levels may be low leading to frequent infection 
thrombocytopenia - bleeding 
splenomegaly 
bone pain

*lymphadenopathy and intrathoracic extramedullary disease is less prominent than in ALL

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8
Q

what is the classification used for AML?

A 
MO - undifferentiated
M1 - without maturation
M2 - with granulocytic maturation
M3 - acute promyelocytic
M4 - granulocytic and monocytic maturation
M5 - monocytic
M6 - erythroleukaemia
M7 - megakaryoblastic
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9
Q

what conditions predispose to leukaemia ?

A

down’s syndrome increase risk of AML and ALL

Fanconi syndrome, bloom syndrome, ataxia telangiectasia, Kostmann’s syndrome, diamond blackfan syndrome, klinefelters, turners, neurofibromatosis, incontinentia pigmenti all increase the risk of AML.

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10
Q

what is Hodgkin’s lymphoma?

A

Hodgkin’s lymphoma (HL), also referred to as Hodgkin’s disease, is an uncommon haematological malignancy arising from mature B cells. It is characterised by the presence of Hodgkin’s cells and Reed-Sternberg cells.

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11
Q

what is Hodgkin’s lymphoma characterised by?

A

the presence of Reed-Sternberg cells
it is characterised by lymphadenopathy - painless, non tender, asymmetrical
Systemic - B symptoms - weight loss greater than 10%, night sweats, fever (Pel-Ebstein)

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12
Q

what are the investigations for AML?

A

FBC - anaemia, microcytosis, leucocytosis, neutropenia and thrombocytopenia

peripheral blood smear - blasts on blood film, presence of Auer rods

coagulation panel
serum electrolytes  
renal function 
LFTs
bone marrow biopsy - will show bone marrow hypercellularity and infiltration by blasts, blasts >20%, Auer rods
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13
Q

what are the risk factors for Hodgkin’s lymphoma?

A

history of EBV infection

fam history of Hodgkin’s lymphoma

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14
Q

what are the different types of Hodgkin’s lymphoma ?

A

Nodular sclerosing Hodgkin lymphoma (NSHL) - most common in children and adolescents
Mixed-cellularity Hodgkin lymphoma (MCHL)
Lymphocyte-depleted Hodgkin lymphoma (LDHL)
Lymphocyte-rich classical Hodgkin lymphoma (LRHL)

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15
Q

what are the common sites for hodgkins lymphoma?

A

most common site is cervical and mediastinal

dissemination to extra-nodal sites is uncommon

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16
Q

what is the staging for hodgkins lymphoma?

A

Ann Arbor system
I -Single lymph node region
II- Two or more regions on the same side of the diaphragm
III- Involvement of lymph node regions on both sides of the diaphragm
IV - Involvement of extra nodal sites

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17
Q

what investigations would you perform for hodgkins lymphoma?

A 
FBC 
metabolic panel 
ESR 
CT neck chest and abdomen/pelvis
PET scan 
Bone marrow biopsy - if radiological evidence of at least stage 3)  - the presence of Hodgkin's cells

EBV serology

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18
Q

what is a lymphoma?

A

Lymphoma is the malignant proliferation of lymphocytes which accumulate in lymph nodes or other organs. Lymphoma may be classified as either Hodgkin’s lymphoma (a specific type of lymphoma characterized by the presence of Reed-Sternberg cells) or non-Hodgkin’s lymphoma (every other type of lymphoma that is not Hodgkin’s lymphoma).

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19
Q

what are the clinical features of non-Hodgkin’s lymphoma?

A 
peripheral lymphadenopathy 
splenomegaly 
night sweats 
weight loss 
fatigue 
malaise 
fever 
extra nodal disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)
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20
Q

how can you differentiate hodgkins from non-Hodgkin’s?

A

Lymphadenopathy in Hodgkin’s lymphoma can experience alcohol-induced pain in the node
‘B’ symptoms typically occur earlier in Hodgkin’s lymphoma and later in non-Hodgkin’s lymphoma
Extra-nodal disease is much more common in non-Hodgkin’s lymphoma than in Hodgkin’s lymphoma

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21
Q

what investigations would you perform for non-Hodgkin’s lymphoma

A

excisional node biopsy
CT chest abdomen and pelvis - to assess staging
HIV test
FBC - thrombocytopenia, pancytopenia, lymphocytosis
blood smear - nucleated red blood cells, left shift
LDH - an indirect indication of the proliferative rate of the lymphoma - an important diagnostic and prognostic factor

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22
Q

what are the risk factors for non-Hodgkin’s lymphoma?

A 
males 
immunocompromised 
EBV infection 
human T-lymphocytotrophic virus 1 (HYLV-1)
human herpesvirus-8
H.pylori 
coeliac disease
HIV
Hep C
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23
Q

what is the staging used for non-hodgkins lymphoma

A

same as HL - ann arbor system

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24
Q

how is non-Hodgkin’s lymphoma managed?

A

Management is dependent on the specific sub-type of non-Hodgkin’s lymphoma and will typically take the form of watchful waiting, chemotherapy or radiotherapy.
All patients will receive flu/pneumococcal vaccines
Patients with neutropenia may require antibiotic prophylaxis

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25
Q

what are the complications of Hodgkin’s lymphoma?

A

Bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia
Superior vena cava obstruction
Metastasis
Spinal cord compression
Complications related to treatment e.g. Side effects of chemotherapy

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26
Q

what are the different types of ALL?

A

common - 75%
T cell only - 20%
B cell only - 5%

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27
Q

what are the complications of ALL?

A

neutropenic sepsis - give tazocin
hyperuricaemia - give allopurinol
secondary cancer
stunted growth

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28
Q

what are the different types of brain tumours?

A

Astrocytoma - the most common - varies from benign to highly malignant
Medulloblastoma - arises in the midline of the posterior fossa
ependymoma
brainstem glioma
craniopharyngioma

29
Q

what are the classifications of brain tumours?

A

supratentorial - cortex - astrocytoma

Infratentorial - cerebellar - medulloblastoma, astrocytoma, ependymoma. - brainstem, - brainstem glioma

spinal cord - astrocytoma, ependymoma

Midline - craniopharyngioma

30
Q

what are the clinical features of a supratentorial tumour?

A

seizures
hemiplegia
focal neurological signs

31
Q

what are the symptoms of a midline tumour?

A

visual field loss - bitemporal hemianopia

pituitary failure - growth failure, diabetes insipidus, weight gain

32
Q

what are the symptoms of cerebellar and IVth ventricle tumour?

A

truncal ataxia
coordination difficulties
abnormal eye movements

33
Q

what are the symptoms of a brainstem tumour?

A

cranial nerve defects
pyramidal tract signs
cerebellar signs - ataxia

*prognosis is poor
diffuse brainstem gliomas are inoperable

34
Q

what are the most common sites for a low grade glioma?

A

cerebellum and optic pathway

35
Q

what is the most common malignant brain tumour in children?

A

primitive neuroectodermal tumours - majority occur in the cerebellum (similar histologically to medulloblastoma however worse prognosis) they usually arise in the midline and invade the fourth ventricle and cerebral hemispheres

36
Q

how do children with an ependymoma present?

A

they usually present with obstructive hydrocephalus

37
Q

how do brainstem gliomas present?

A

cranial nerve palsies, ataxia and pyramidal tract signs

38
Q

how do primitive neuroectodermal tumours present?

A

headache, vomiting and ataxia

39
Q

what does a craniopharyngioma present with?

A

visual field loss due to compression of the optic chiasm

pituitary dysfunction - growth failure and diabetes insipidus

40
Q

what is a craniopharyngioma?

A

slow growing midline epithelial tumours arising from the remnant Rathke’s pouch

41
Q

what are the symptoms of a retinoblastoma?

A

absent or abnormal light reflex, squint or visual deterioration

42
Q

what is a Wilm’s tumour?

A

it is a nephroblastoma - the most common form of renal malignancy in childhood.
it arises from embryonic renal tissue

43
Q

what age group does a Wilm’s tumour present in?

A

2-5 years

44
Q

what are the clinical features of a Wilms’ tumour?

A

abdominal mass (most common presenting feature)
painless haematuria
flank pain
other features: anorexia, fever
unilateral in 95% of cases
metastases are found in 20% of patients (most commonly lung)

45
Q

what are Wilms’ tumours associated with ?

A

genitourinary abnormalities e.g. horseshoe kidney, hypospadias
hemihypertrophy syndrome

beckwith-wiedemann syndrome

around one-third of cases are associated with a loss-of-function mutation in the WT1 gene on chromosome 11

associated with aniridia (absent iris)

46
Q

what investigations would you perform for a wilms’ tumour ?

A

FBC
Renal function - decreased clearance creatinine/raised serum creatinine
LFT
urinalysis
abdominal US
CT scan of abdomen - claw signed in involved kidney
biopsy

47
Q

how do you treat a wilm’s tumour?

A

chemotherapy
nephrectomy
radiotherapy if advanced disease

48
Q

what is a neuroblastoma?

A

Neuroblastoma is one of the top five causes of cancer in children, accounting for around 7-8% of childhood malignancies. The tumour arises from neural crest tissue of the adrenal medulla (the most common site) and sympathetic nervous system.

49
Q

what are the common sites for neuroblastoma involvement?

A

they can occur anywhere in the sympathetic nervous system

commonly occur in the adrenals
the sympathetic chain - abdomen, thorax, pelvis, neck

50
Q

what are the clinical features of a neuroblastoma?

A

abdominal mass - a firm, non tender abdominal mass is the most common mode of presentation
abdominal distension
abdominal pain
bone pain is common in patients with metastatic disease
systemic signs: pallor, weight loss, bone pain from disseminated disease
hepatomegaly or lymph nodes enlargement
compression of nerves - Horner’s syndrome
decreased appetite
panda eyes - periorbital ecchymosis - in patients with orbital metastases

51
Q

what investigations would you perform for a neuroblastoma?

A

urine catecholamine - catecholamine degradation products homovanillic acid (HVA) and vanilylmandelic acid (VMA) are secreted by the majority of tumours

FBC, serum electrolytes, creatinine/urea, LFTs

USS abdomen - heterogenous mass with internal vascularity; may show calcifications or areas of necrosis

CT/MRI

MIBG scan

52
Q

how do you treat a neuroblastoma?

A

surgical resection
chemo
radio

53
Q

what are poor prognostic factors in neuroblastoma?

A 
age >18 months 
stage 3 and 4 disease 
raised serum ferritin 
raised LDH 
raised neurone- specific enolase (NSE)
unfavourable histology 
MYCN oncogene amplification
54
Q

what is a retinoblastoma?

A

Most common intraocular malignancy in children.
In 30% to 40% of cases, the disease is associated with a germline mutation in the RB1 gene, which carries an associated increased risk of secondary non-ocular tumours.

55
Q

how does a retinoblastoma present?

A

leukocoria - most common (white pupillary reflex)

strabismus

56
Q

how do you diagnose and treat a retinoblastoma?

A

fundoscopy and examination under anaesthesia
wide-field fundus photography and spectral domain optical coherence tomography ophthalmic A and B scan USS

treat with surgery

57
Q

what are the two most common forms of bone tumours in childhood?

A

osteosarcoma - older children, most common

Ewing’s sarcoma: younger children, less common

58
Q

what is an osteosarcoma?

A

malignant tumour of the bone-producing mesenchymal cells

59
Q

how does an osteosarcoma present?

A

localised pain and welling
pathological fracture
rarely erythema

60
Q

what is the most common site for an osteosarcoma?

A

most affect the long bones around the knee (distal femur and proximal tibia) and humerus

delay in diagnosis is common

metastasis are seen in 15-25% of cases and are mainly found in the lungs

61
Q

how would you diagnose an osteosarcoma?

A 
plain x-ray of bony lesion 
biosy
LDH and alkaline phosphatase - will usually be raised 
MRI
CT chest 
isotope bone scan
62
Q

how do you treat osteosarcomas?

A

chemotherapy followed by surgery

63
Q

how does an Ewing’s sarcoma present?

A

usually occurs in bone but can also occur in soft tissue
localised pain and swelling, sometimes a pathological fracture.
Commonly affects the diaphysis of long bones
the most common site is the femoral diaphysis
metastasis to lungs and bone common

64
Q

how do you diagnose and Ewing’s tumour?

A 
plain x-ray of bony lesions 
biopsy 
LDH and alkaline phospatatse 
MRI 
CT chest 
isotope bone scane 
bone marrow aspirates and trephines
65
Q

what is a rhabdomyosarcoma?

A

it is the most common soft tissue sarcoma in childhood - commonly in aged <10 years
most are either embryonic or alveolar subtypes

66
Q

what are the clinical features of rhabdomyosarcoma?

A

mass, pain and obstruction of:

  • bladder
  • pelvis
  • nasopharynx
  • parameningeal
  • orbit
  • intrathoracic

lymph involvement in common, distant metastases are rare

67
Q

what investigations would you perform for rhabdomyosarcoma?

A 
CT or MRI of primary site 
biopsy 
CT if chest 
bone marrow aspirates 
isotope bone scan 
LP
68
Q

what is the most common primary paediatric hepatic tumour? and what is seen in this type of tumour?

A

hepatoblastoma
usually seen in the first year of life
serum AFP levels are raised by >80%

Phase 3a (41 decks)

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