Oncology Flashcards
What are the 2 Types of ALL
b-cell
T-cell
Whats the pathophysiology of ALL?
stem cell DNA mutations cause hyper proliferation of WBC - excessive production of immature blast cells
Environmental risk factors of developing cancer in children
Radiation
Infection
Parental smoking during pregnancy
Known genetic risk factors
Down’s syndrome- increase risk of leukaemia
Familial retinoblastoma
Which types of childhood cancers are caused by infections
Berkitts lymphoma
Hodgkin’s lymphoma
Nasopharyngeal carcinoma - EBV
Hepatocellular carcinoma - hep B
Kaposi sarcaom HHV8
What is a paraneoplastic phenomena
Symptoms that are not directly attributable to the tumour but arise as part of body’s response to disease
w.g. pain, lethargy, pallor
What is opsoclonus-myoclonus -ataxia syndrome?
Dancing eyes
Example of paraneoplastic phenomenon
Caused by neuroblastoma
Abnormal eye jerking
What is the treatment for opsoclonus-myoclonus-ataxia syndrome?
immunosuppression
Presentation of pancytopenia in children with malignancy
Pallor/ lethargy - low Hb
Recurrent fever/ infection - low WBC
Bruising or petechiae - low PLTS
Lymphadenopathy/ unexplained mass presentation
Lymph node with diameter greater than 2cm
Non-tener, ruberò, hard or fixed
Supraclavicular or axillary location
Systemic symptoms - fever/ weight loss/ night sweats
Respiratory symptoms
Orthopneoa
Reduced a/e - pleural effusion
Presentation if bone and joint pain
Persistent back pain
Night pain
Reluctance to weight-bear or new limp
Presentation if abdominal mass
General malaise - neuroblastoma
HTN - Wilms or neuroblastoma
Presentation if raised intracranial pressure
Heachache
Papilloedema
III and VI cn palsies
Neurological signs
cerebellar signs
Visual disturbances
What should you investigate if suspecting liver tumour or malignant germ cell tumour?
Alpha-fetoprotein AFP
b-human chorionic gonadotrophin B-HCG
What is AFP associated with
Epithelial liver tumours
Hepatiblastoma and hepatocellular carcinoma
Yolk sac tumours
Embryonaly carcinoma
What is G1 in the cell cycle
‘Gap phase’ - synthesis of the components required for DNA synthesis
What is S phase in cell cycle
DNA synthesis phase before second gap phase G2
What happens in G2
Cell prepares for mitosis
What happens in M phase of cell cycle
Final phase - actively replication cells is mitotic phase
What is G0 in cell cycle
Rest phase
How does vincristine work?
block the function of the spindle apparatus required for mitosis
How does etoposide work?
Causes DNA stand breaks or directly damage DNA by forming crosslinks
Where does antimetabolites work?
S phase - blocks synthesis of normal nucleic acids required for DNA synthesis
Name antimetabolites
Methotrexate
Cytarabine
Mercaptopurine
What cancers are at increase risk of Tumour lysis syndrome
High-count ALL and NHL (B cell)
Cause of tumour lysis syndrome
Rapid lysis of malignant cells on initiating chemotherapy
Features of tumour lysis syndrome
Hyperkalaemia
Hyperuricaemia
Hyperphosphataemia
Hypocalcmaeia
What cancers are at risk of hyper viscosity syndrome?
High-count leukaemia
Cause of hyper viscosity
Sludging of venous blood in small vessels
Large number of circulating blast cells -> poor perfusion -> stroke and death
Blood transfusion dangerous
Raised intracranial pressures features
headaches
vomiting
ataxia
papilloedema
Febrile neutropenia features
temp >38
neutrophil count <1
Stage 1 Wilm’s Tumour
Tumour confined to kidney and is completely removed at time of surgery
Stage 2 Wilms Tumour
Tumour has broken through the kidney lining but still removed by surgery
Stage 3 will’s tumour
Some of the tumour if left behind at time of surgery- tumour breaks or complete surgical removal sis not possible
Stage 4 Wilm’s tumour
Spread to other parts of body - lungs or lymph nodes
Stage 5 Wilm’s tumour
Tumour in both kidneys
Which Investigation differentiates B cell from T cell NHL
flow cytometry - identify presence of absence of protein markers on cell surface
What genetic syndromes are Wilm;s Tumours ass. with?
Beckwith-Wiedemann syndrome
WAGR syndrome
microdeletions of short arm in chromosome 11 (11p)
What would results show of biopsy of ?hogkins lymphoma
lymphocytic and histolytic cells
Which lineage of cells does Hodgkin’s lymphoma come from?
clonal transformation of B- cells
What are the four subtypes of Hodgkin’s lymphoma?
Nodular sclerosing - most common
Mixedixed cellularity
Lymphocyte rich
Lymphocyte depleted - rare in children
Presentation of Hodgkin’s lymphoma
Painless enlargement of supraclavicular lymph nodes
B- symptoms
Splenomegly
Lymph nodes biopsy in Hodgkin’s lymphoma
Reedsternberg cells
Staging of Hodgkin’s lymphoma
Stage 1 - single lymph node or extranodal site
Stage 2 - 2 or more lymph nodes on same side of diaphragm
Stage 3 - Lymph-node involvement on both sides of diaphragm
Stage 4 - Disseminated or multiple involvement of extra nodal sites
What percentage of lymphoblasts in bone marrow is needed for a definitive diagnosis of ALL
20% or more
Prognosis is worse for…..
<1 year olds
What Xray findings are in long bones with ALL
osteolytic medullary lesions
What is a neuroblastoma?
Embryonal cancer of peripheral sympathetic nervous system
What is a good prognostic indictator in neuroblastomas?
high levels of TRk gene give a favourable outcome
Poor prognosis of ALL
Male
Age <2
WBC >50
T-cell phenotype
CNS disease
Pathophysiology of hereditary spherocystosis
Abnormality of cytoskeleton of RBCS
what does osmotic fragility test show
increase hamolysis
not specific to hereditary sphereocystosis
What would you see on blood film in patients with hereditary spherocysotis
spheroidal cells
What would you see in neuroblastoma
VMA or hva (URINE)
What gene is related to retinoblastoma
RB1 on 13 q chromosome
What is histological signs of ALL
blast cells
high nucleus to cytoplasm ratio
What is burrkitt lymphoma
type of non-hodgkin lymphoma
What gene is ass with bruit lymphoma
MYC gene