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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
what are the complications of Hodgkin's lymphoma?
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
26
what are the different types of ALL?
common - 75% T cell only - 20% B cell only - 5%
27
what are the complications of ALL?
neutropenic sepsis - give tazocin hyperuricaemia - give allopurinol secondary cancer stunted growth
28
what are the different types of brain tumours?
Astrocytoma - the most common - varies from benign to highly malignant Medulloblastoma - arises in the midline of the posterior fossa ependymoma brainstem glioma craniopharyngioma
29
what are the classifications of brain tumours?
supratentorial - cortex - astrocytoma Infratentorial - cerebellar - medulloblastoma, astrocytoma, ependymoma. - brainstem, - brainstem glioma spinal cord - astrocytoma, ependymoma Midline - craniopharyngioma
30
what are the clinical features of a supratentorial tumour?
seizures hemiplegia focal neurological signs
31
what are the symptoms of a midline tumour?
visual field loss - bitemporal hemianopia | pituitary failure - growth failure, diabetes insipidus, weight gain
32
what are the symptoms of cerebellar and IVth ventricle tumour?
truncal ataxia coordination difficulties abnormal eye movements
33
what are the symptoms of a brainstem tumour?
cranial nerve defects pyramidal tract signs cerebellar signs - ataxia *prognosis is poor diffuse brainstem gliomas are inoperable
34
what are the most common sites for a low grade glioma?
cerebellum and optic pathway
35
what is the most common malignant brain tumour in children?
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
how do children with an ependymoma present?
they usually present with obstructive hydrocephalus
37
how do brainstem gliomas present?
cranial nerve palsies, ataxia and pyramidal tract signs
38
how do primitive neuroectodermal tumours present?
headache, vomiting and ataxia
39
what does a craniopharyngioma present with?
visual field loss due to compression of the optic chiasm | pituitary dysfunction - growth failure and diabetes insipidus
40
what is a craniopharyngioma?
slow growing midline epithelial tumours arising from the remnant Rathke's pouch
41
what are the symptoms of a retinoblastoma?
absent or abnormal light reflex, squint or visual deterioration
42
what is a Wilm's tumour?
it is a nephroblastoma - the most common form of renal malignancy in childhood. it arises from embryonic renal tissue
43
what age group does a Wilm's tumour present in?
2-5 years
44
what are the clinical features of a Wilms' tumour?
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
what are Wilms' tumours associated with ?
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
what investigations would you perform for a wilms' tumour ?
FBC Renal function - decreased clearance creatinine/raised serum creatinine LFT urinalysis abdominal US CT scan of abdomen - claw signed in involved kidney biopsy
47
how do you treat a wilm's tumour?
chemotherapy nephrectomy radiotherapy if advanced disease
48
what is a neuroblastoma?
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
what are the common sites for neuroblastoma involvement?
they can occur anywhere in the sympathetic nervous system commonly occur in the adrenals the sympathetic chain - abdomen, thorax, pelvis, neck
50
what are the clinical features of a neuroblastoma?
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
what investigations would you perform for a neuroblastoma?
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
how do you treat a neuroblastoma?
surgical resection chemo radio
53
what are poor prognostic factors in neuroblastoma?
``` age >18 months stage 3 and 4 disease raised serum ferritin raised LDH raised neurone- specific enolase (NSE) unfavourable histology MYCN oncogene amplification ```
54
what is a retinoblastoma?
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
how does a retinoblastoma present?
leukocoria - most common (white pupillary reflex) | strabismus
56
how do you diagnose and treat a retinoblastoma?
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
what are the two most common forms of bone tumours in childhood?
osteosarcoma - older children, most common | Ewing's sarcoma: younger children, less common
58
what is an osteosarcoma?
malignant tumour of the bone-producing mesenchymal cells
59
how does an osteosarcoma present?
localised pain and welling pathological fracture rarely erythema
60
what is the most common site for an osteosarcoma?
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
how would you diagnose an osteosarcoma?
``` plain x-ray of bony lesion biosy LDH and alkaline phosphatase - will usually be raised MRI CT chest isotope bone scan ```
62
how do you treat osteosarcomas?
chemotherapy followed by surgery
63
how does an Ewing's sarcoma present?
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
how do you diagnose and Ewing's tumour?
``` plain x-ray of bony lesions biopsy LDH and alkaline phospatatse MRI CT chest isotope bone scane bone marrow aspirates and trephines ```
65
what is a rhabdomyosarcoma?
it is the most common soft tissue sarcoma in childhood - commonly in aged <10 years most are either embryonic or alveolar subtypes
66
what are the clinical features of rhabdomyosarcoma?
mass, pain and obstruction of: - bladder - pelvis - nasopharynx - parameningeal - orbit - intrathoracic lymph involvement in common, distant metastases are rare
67
what investigations would you perform for rhabdomyosarcoma?
``` CT or MRI of primary site biopsy CT if chest bone marrow aspirates isotope bone scan LP ```
68
what is the most common primary paediatric hepatic tumour? and what is seen in this type of tumour?
hepatoblastoma usually seen in the first year of life serum AFP levels are raised by >80%

Phase 3a (41 decks)

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