Oncology Flashcards
What is a retinoblastoma?
Retinoblastoma is an eye cancer that begins in the retina — the sensitive lining on the inside of your eye
What mutation is a retinoblastoma?
Autosomal dominant
Caused by a loss of function of the retinoblastoma tumour suppressor gene on chromosome 13
Around 10% of cases are hereditary
What are the symptoms of retinoblastomas?
Absence of red-reflex, replaced by a white pupil (leukocoria) - the most common presenting symptom
Strabismus
Visual problems
How do you diagnose a retinoblastoma?
- Eye exam
- Abscence of red reflex
- MRI
What is the management of a retinoblastoma?
- Enucleation is not the only option
- External beam radiation therapy
- Chemotherapy
- Photocoagulation
What is Wilm’s tumour?
Wilms’ nephroblastoma is one of the most common childhood malignancies
What is the most likely age to get Wilm’s tumour?
children under 5 years of age, with a median age of 3 years old.
What are the symptoms of Wilm’s 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)
How do you diagnose Wilm’s tumour?
- Ultrasound - confirm solid or cystic nature of mass
- IV urethrogram my show calyceal distortion by intrarenal mass
- Staging - CT
What is the management for Wilm’s tumour?
- Children with an unexplained enlarged abdominal mass in children - possible Wilm’s tumour - arrange paediatric review with 48 hours
- Nephrectomy
- Chemotherapy
- Radiotherapy if advanced disease
- Prognosis: good, 80% cure rate
What is acute lymphoblastic leukaemia (ALL)?
ALL THE CHILDREN
Acute lymphoblastic leukaemia (ALL) is the most common malignancy affecting children and accounts for 80% of childhood leukaemias.
The peak incidence is at around 2-5 years of age and boys are affected slightly more commonly than girls
What are the features of acute lymphoblastic leukaemia?
- Anaemia: lethargy and pallor
- Neutropaenia: frequent or severe infections
- Thrombocytopenia: easy bruising, petechiae
- Bone pain (secondary to bone marrow infiltration)
- Splenomegaly
- Hepatomegaly
- Fever is present in up to 50% of new cases (representing infection or constitutional symptom)
- Testicular swelling
What are the different types of Acute lymphoblastic leukaemia?
common ALL (75%), CD10 present, pre-B phenotype
T-cell ALL (20%)
B-cell ALL (5%)
What are the poor prognostic features of acute lymphoblastic leukaemia?
- age < 2 years or > 10 years
- WBC > 20 * 109/l at diagnosis
- T or B cell surface markers
- non-Caucasian
- male sex
What chromosome is present in chronic myeloid leukaemia?
Which chromsomes does it involve?
Philadelphia
9 and 22
What are the features of chronic myeloid leukaemia?
- Anaemia: lethargy
- Weight loss and sweating are common
- Splenomegaly may be marked → abdo discomfort
- An increase in granulocytes at different stages of maturation +/- thrombocytosis
- Decreased leukocyte alkaline phosphatase
- May undergo blast transformation (AML in 80%, ALL in 20%)
What is the management of chronic myeloid leukaemia?
- Imatinib is now considered first-line treatment
- Hydroxyurea
- Interferon-alpha
- Allogenic bone marrow transplant
What are the cells present in Hodgkin’s lymphoma?
Reed-Sternberg cell
What are the symptoms of hodgkin’s lymphoma?
- Lymphadenopathy (75%) - painless, non-tender, asymmetrical
- Systemic (25%): weight loss, pruritus, night sweats, fever (Pel-Ebstein)
- Alcohol pain in HL
- Normocytic anaemia, eosinophilia
- LDH raised
What are the B symptoms of hodgkin’s lymphoma?
- Weight loss > 10% in last 6 months
- Fever > 38ºC
- Night sweats
What is the staging system used in Hodgkin’s lymphoma?
Ann-Arbor
What is the treatment of Hogdkin’s lymphoma?
Combined chemotherapy
How do you monitor the progression of Hodgkin’s lymphoma treatment?
PET scan
WHAT IS A PILOCYTIC ASTROCYTOMA?
The most common primary brain tumour in children
Where does a pilocytic astrocytoma occur?
- Hypothalamus
- Optic nerve
- Cerebellum
What are the clinical features of a pilocytic astrocytoma?
In children, juvenile astrocytomas may arise in the hypothalamus and present with failure to thrive, emaciation, and features of panhypopituitarism.
Visual field scotomas progress to complete field loss. Orbital extension produces proptosis
What are the investigations for a pilocytic astrocytoma?
- Histology: Rosenthal fibres (corkscrew eosinophilic bundle)
- MRI
What are the treatment options of pilocytic astrocytomas?
Surgical excision
WHAT IS A NEUROBLASTOMA?
A neuroblastoma is a tumour derived from neural crest tissue and so, is properly regarded as an APUD tumour.
Most common place is adrenal medulla
What are the clinical features of neuroblastoma?
- Abdominal mass
- Pallor, weight loss
- Bone pain, limp
- Hepatomegaly
- Paraplegia
- Proptosis
What is the staging for a neuroblastoma?
- Stage I - single organ disease
- Stage II - regional node involvement but not across the midline
- Stage III - tumour across the midline
- Stage IV - skeletal, soft tissue or nodal metastases
- Stage IV is extended to stage IVS to group together patients with what would otherwise be group I or II, with skin, liver or bone marrow metastases, but not bone metastases
What are the investigations of a neuroblastoma?
- Raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
- Calcification may be seen on abdominal x-ray
- Biopsy
What is the treatment for a neuroblastoma?
- Stage I - surgery alone
- Stage II - surgery and local radiotherapy to tumour bed
- Stage III - surgery, chemotherapy and radiotherapy
- Stage IV - chemotherapy
Neuroblastomas are radiosensitive.
WHAT IS AN OSTEOSARCOMA?
Most common primary malignant bone tumour
Where is the most common place for an osteosarcoma?
Metaphyseal region of long bones prior to epiphyseal closure, with 40% occuring in the femur, 20% in the tibia, and 10% in the humerus
What are the clinical features of osteosarcoma?
Pain and localised tenderness are common initial presentations. T
he pain tends to be constant, worst at night, and becomes increasingly more severe.
What are the investigations for osteoasarcomas?
X-ray
Sunray spicules - new bone breaches the cortex and radiates outwards, like a sunburst, into adjacent soft tissue
Codman’s triangle - periosteum lifted into a triangle as the tumour emerges through the cortex
Biopsy
Essential for diagnosis
What is the treatment for osteosarcomas?
Best results require complete eradication of the primary tumour by radical surgery combined with chemotherapy.
WHAT IS EWING’S SARCOMA?
Ewing’s sarcoma is a malignant small cell tumour of bone, more common in males and having its highest incidence in the age group 5 to 20 years.
It usually occurs in the diaphysis of long bones.
Where does Ewing’s sarcoma affect?
Occurs most frequently in the pelvis and long bones.
Tends to cause severe pain
What are the investigations for Ewing’s sarcoma?
Bone destruction with overlying onion-skin layers of periosteal bone formation
What is the treatment for Ewing’s sarcoma?
Surgical intervention for Ewing’s sarcoma is usually restricted to biopsies of the tumour.
Treatment is usually a combination of radiotherapy and cytotoxic chemotherapy; rarely it is treated surgically.
WHAT IS A HEPATOBLASTOMA?
Hepatoblastoma is a very rare primary liver cell cancer that is most predominant in infants.
What are the clinical features of hepatoblasoma?
The usual presentation is one of a large, painless abdominal mass.
A raised serum alpha-feto protein is suggestive of the diagnosis.
What are the investigations for hepatoblastoma?
A raised serum alpha-feto protein is suggestive of the diagnosis.
What is the treatment of hepatoblastoma?
If there is limited disease then a partial hepatectomy may be curative.
Conversion of an inoperable lesion to an operable one may be achieved by intensive chemotherapy.
