Malignant Disease Flashcards
What are the important causative factors in malignant disease
Genetics, infections, environmental
What effect on genes causes cancer? Eg of some diseases?
Activation of oncogenes & loss of tumour suppressor
retinoblastoma, Down’s syndrome, Ataxia-telangiectasia, Li-Fraumeni
Which two infections can lead to increased risk of malignancy? What malignancy?
Epstein Barr virus - transforms B cells -> Burkitt’s lymphoma
HIV - targets T helper (CD4) cells -> susceptibility to lymphoid malignancies
Important environmental risk factor for malignancy
Previous treatment for malignancy in a child
Localised mass in Abdo? Thorax? Soft tissue ? ….makes you think of ?
A- wilm’s tumour, neuroblastoma
T- non-hodgkins lymphoma
S- rhabdomyosarcomas
Which malignancy if constitutional symptoms ? What are constitutional Sx?
Weight loss, night sweats, fever
Lymphoma
Which malignancy if bone marrow failure? Bone pain?
Acute leukaemia
Bon tumours , leukaemia
Give some egs of consequences of mass effect?
Air way obstruction due to enlarged lymph nodes
Increased ICP due to CNS tumours
Malignancy investigations
Histology - biopsy / bone marrow aspiration
Imaging - USS, X-ray, MRI
Tumour markers (in some)
Which tumours do you not take a biopsy from?
CNS
Egs of some tumour markers
A-fetoprotein (in liver Ca)
Increased urinary catecholamines (in neuroblastoma)
What is the most common childhood malignancy ? What characterises it?
Leukaemias
proliferation of immature WBCs
What is the commonest childhood leukaemia? Which only affects adults?
Acute - 97%
CML - rare, CLL only adults
Which cells are included in the ALL lineage of leukaemia ? % of leukaemia ?
Lymphoid
T cells, B cells, natural killer cells
80%
Which cells are in the AML cells of leukaemia
Granulocytic / macrocytic
Eosinophils, basophils, monocytes, platelets, RBCs
Usual onset? Features of leukaemia and causes?
Insidious with pallor & malaise (anaemia); haemorrhagic diathesis (purpura, easy brusing, epistaxis - thrombocytopenia) ; hepatosplenomegaly / lymphadenopathy (macrocyte infiltration) ; bone pain (marrow cavity expansion); infections (neutropenia)
Investigations for leukaemia
FBC - anaemia, thrombocytopenia, WBCs variable Blood film - *blast cells* *bone marrow aspirate* (Chest X-ray - ?mediastinal mass Serum chemistry LP)
Management of ALL?
Chemotherapy (5 phases)
Haemopoietic stem cell transplantation
What are the 5 phases of chemo for ALL
Induction (3-5 drugs to decrease tumour load)
Interim management
Consolidation (continued systemic therapy after remission)
Intensification
Maintenance (for 2 years)
When does haemopoietic stem cell transplantation get used?
Only in high risk cases in 1st remission / relapse
What drug is used to protect kidneys from what in ALL management?
Allopurinol protects kidneys from
Effects of rapid cell lysis
Which has worst prognosis ALL/ AML?
AML - more intensive treatment
What is associated strongly with AML
80% have chromosomal abnormalities eg. Downs
What are lymphomas? 2 types and their usual ages?
Malignancies of the cells of the immune system
Non-hodgkins (young children)
Hodgkins (adolescents & young adults)
Predominant site of disease in NHL? What do the clinical features reflect?
Lymph nodes
Pattern of normal lymphoid cell migration
Features of NHL depending on what is affected?
T- cell malignancies - present as ALL or NHL usually with a mediastinal mass (+/- pleural effusion)
B-cell malignancies - NHL with localised lymph node disease (head/neck/abdo)
Abdominal disease - pain (+/- palpable mass) - usually advanced B cell
Treatment of NHL
Combination of chemo / radio
+/- surgical debunking for abdominal tumours
What is SVC syndrome?
Complication of (usually) a tumour compressing SVC -> oedema of face and arms
Complication of therapy for lymphomas / Leukaemia ? What is it?
Tumour lysis syndrome
Due to release of cellular contents into blood stream when cells die
What changes in blood are characteristic for tumour lysis syndrome
Hyperkalaemia
Hypocalcaemia
Hyperphosphataemia
Hyperuricaemia
Where are most CNS tumours
60% infratentorial
Presentation of CNS tumours
Most have general features of raised ICP, but may get focal signs Vomiting (esp. Early morning) Headache ( worse lying down / early morning) Papilloedema Strabismus (VI nerve palsy) Nystagmus Ataxia Behavioural / personality change
What causes strabismus
Paralysed lateral rectus -> eye drawn in nasally
Investigations for CNS tumours
MRI
LP - Not advised if raised ICP
Biopsy (not always possible eg. Brainstem tumour)
2 types of CNS tumours and egs.
Astrocytic (eg high grade (supratentorial), low grade (Cerebellar), brainstem glioma)
Neuroepithelial eg. Primitive neuroectodermal tumours (PNET) (including Cerebellar medulloblastoma)
Commonest brain tumour ? Grade / growth / age usually?
Astrocytomas 40%
Cerebellar astrocytomas, low grade, slow growing cystic gliomas in 6-9 year olds
Presentation of astrocytomas
Headache, vommiting (+- pappilodema) - obstructive hydrocephalus
Cerebellar signs - ataxia, nystagmus, uncoordination
Diplopia, strabismus ( CN VI palsy)
What are primitive neuroectodermal tumours also called? % of brain tumours? Which age commonest?
Medulloblastoma, 20%
Most common malignant brain tumour of childhood - peak in boys 2-6
Usual location of medulloblastoma
Midline & invade 4th ventricle & Cerebellar hemispheres
What is often found at diagnosis of medulloblastoma
Spinal mets in 20%
Presentation of medulloblastoma
Vomiting, headache, Ataxia
Treatment of medulloblastoma
Surgical excision + adjuvant radiotherapy of whole of CNS
Chemo in +++ risk
What % of CNS tumours are craniopharyngiomas? What do they arise from?
4%
squamous remnant of Rathke’s pouch
Presentation of craniopharyngiomas ? Why /how are they easily seen?
Visual field loss (optic chiasm compression), pituitary dysfunction (DI / growth failure)
calcified and so visible on skull radiographs
Treatment of craniopharyngiomas ? Complication?
Surgical excision - good
Risk of pan hypopituitarism / visual impairment
What is a neuroblastoma ? Usual age?
Malignancy of neural crest tissue in the adrenal medulla & sympathetic nervous system
Features of neuroblastoma depend on location, but give some anyway?
Abdo mass - most common (firm non tender)
Systemic signs - pallor, weight loss, bone pain
Unilateral proptosis - pre orbital swelling & eccymosis due to eye mets
Mediastinal mass on chest X-ray
Diagnosis of neuroblastoma
Increased urinary catecholamines (vanillymandelic acid, hormovanillic acid)
Biopsy (usually possible) & MIBG scan
What happens in MIBG scan
Radio labeled tumour specific agent
Treatment of neuroblastoma
Surgical resection, chemotherapy, irradiation
What gives a poorer prognosis in neuroblasoma
Over expression of N-myc oncogene (arise being older & metastatic disease)
What’s the proper name for Wilms’ tumour ? What tissue is it from? Usual age? And where?
Nephroblastoma
Embryonal renal tissues of metanephros
Rare >5 years \unilateral in 95%
Features of nephroblastoma
Most common is asymptomatic abdominal mass that doesn’t cross the midline
Can get abdo pain, heamaturia, hypertension (25% cases)
anirida (absent iris)
Diagnosis of Wilms tumour ? Where do mets commonly go?
Made from characteristic appearance on CT (intrinsic renal mass with mixed solid & cystic densities)
Lungs and liver
Treatment of nephroblastoma
Surgical resection of primary tumour
Chemo according to stage of disease
Most important soft tissue sarcoma ? Where do they arise from?
Rhabdomyosarcomas
Primitive mesenchyme
Soft tissue Sarcoma location and effects
Head & neck - proptosis, nasal obstruction
GU - obstruction , dysuria
When are bone tumours most common? What % of childhood cancer ?
Uncommon before puberty
4%
2 types of bone tumours
Osteogenic sarcoma
Ewing’s sarcoma
What is an osteogenic sarcoma? What age? Features?
Tumour of bone producing mesenchyme
Older children
Local pain & swelling
Where is the most common place for osteogenic sarcoma ? Diagnosis? Treatment?
50% around knee joint in the metaphysis of the distal femur / proximal tibia
X-ray shows sunburst appearance - tumour breaks through cortex & spiralates as new bone forms
Surgery
Age of Ewing’s sarcoma? Tissue origin? Where does it affect?
Younger children , less common then osteogenic, Unknown tissue origin
Usually affects long bones (mid-proximal femur) but does affect flat ones eg pelvis
Features of Ewing’s ? Diagnosis? Treatment?
Pain and localised swelling are usual presenting complaints
X-ray shoes destructive lesion with periosteal elevation or a soft tissue mass (onion skin appearance )
Radio + chemo
What is the cause of a absent red reflex in neonate ? Cause? How often is it bilateral and what does this mean?
Retinoblastoma
Deletion of RB gene on chromosome 13
40% - all bilateral are hereditary
