Oncology Flashcards
Which cells are affected by ALL?
B or T cell, but B cell is more common
How does ALL present?
2-5 years Insidious Malaise, anorexia Pallor Lethargy Bruising, petechiae, nose bleeds Bone pain Hepatosplenomegaly Lymphadenopathy Headache, vomiting, nerve palsies
What investigations should be done for suspected aLL?
FBC - low Hb, thrombocytopenia, blast cells
Bone marrow investigation
Chest X-ray
What are the poor prognostic signs in ALL?
Presenting WCC >50 <2 and >9 Boys Chromosomal abnormalities/translocations, particularly Philadelphia chromosome Hypidiploidy Afro-Caribbean ethnicity CNS disease
How is ALL managed?
Remission induction
Intensification
Intrathecal chemo in some
Continuing therapy
How might an ALL Relapse be managed?
High dose chemo with total body irradiation and BMT
What is AML?
Heterogeneous group of disorders involving precursors of myeloid, monocyte, erythroid and megakaryocyte lines
How is AML subdivided?
7 types according to morphology and immunophenotyping
How common are chromosomal abnormalities in AML?
At least 80% have chromosomal abnormalities, with translocations the most common
How does management of AML compare to ALL?
Treatment is more intensive, but shorter than ALL (usually 6 months)
BMT plays a much more prominent role
What is the prognosis in ALL?
80% 5 year survival
What is the prognosis in AML?
50% 5 year survival
What are the immediate dangers at presentation of a leukaemia?
Infection Hyperleucocytosis/hyperviscosity Tumour lysis syndrome Bleeding Obstruction from mediastinal mass
What are the broad types of brain tumour?
Asytrocytoma (40%) Medulloblastoma (20%) Ependymoma (8%) Brain stem glioma (6%) Craniopharyngioma (4%)
Where do medulloblastomas come from?
Midline of the posterior fossa
How common are spinal mets at diagnosis in medulloblastoma?
Up 2o 20%
Where do ependymomas arise?
Mostly in posterior fossa
Where do craniopharyngiomas arise from?
Squamous remnant of Rathke pouch
What are the clinical features of a supratentorial tumour?
○ Seizures
Hemiplegia
Focal neurological signs
What are the clinical features of a midline brain tumour?
Visual field loss - bitemporal hemianopia
Pituitary failure - growth failure, diabetes insipidus, weight gain
What are the clinical features of a cerebellar or fourth ventricle tumour?
Truncal ataxia
Coordination difficulties
Abnormal eye movements
What are the clinical features of a brainstem tumour?
Cranial nerve defects
Pyramidal tract signs
Cerebellar signs e.g. ataxia
Often no raised ICP
Which tumours are seen in the posterior fossa?
Cerebellar astrocytoma
Medulloblastoma
Ependymoma
What is the most common tumour in children?
Cerebellar astrocytoma
What might a cerebellar astrocytoma involve?
Vermis, cerebellar hemispheres or both
What are the features of a cerebellar astrocytoma?
Cystic, slow growing
What are the features of a medulloblastoma?
Highly malignant, rapidly growing Arises from cerebellar vermis Often causes hydrocephalus Can metastasize along CSF pathways Often solid
What is the prognosis for medulloblastoma?
75% 5 year survival
Who has a poorer prognosis in medulloblasoma?
Younger children
Where do ependymomas come from?
4th ventricle
What can an ependymoma cause?
Hydrocephalus
What is the prognosis in an ependymoma?
Poor, due to localisation of tumour
What are the features of brainstem tumours?
Varies in degree of malignancy Peak incidence 5-9 years Presents with multiple cranial nerve palsies and long tract signs, possible vomiting Treatment is with radiotherapy Poor survival
What are the kinds of tumours seen in the supratentorial region?
Cerebral astrocytoma
Ependymoma
Optic glioma
What are the features of a cerebral astrocytoma?
○ Presentation depends on location, but often leads to seizures
Low grade tumours are benign and more common
High grade tumours are rarer
What are the features of a supratentorial ependymoma?
30-40% of ependymomas are supratentorial
More malignant than the infratentorial ones
Tendency to metastasize
Poor prognosis
What are the features of an optic glioma?
1/2 pre chiasmatic, 2/3 post chiasmatic Generally pilocytic astrocytomas 1/4 occur with NF type 1 Pre chiasmatic: proptosis, visual loss (late) Post chiasmatic: visual loss
What are the clinical features of craniopharyngiomas?
Endocrine:
Delayed growth
Hypothyroidism
Diabetes insipidus
Raised ICP:
Headache
Ataxia
Local features Bitemporal hemianopia Depressed consciousness Vomiting Nystagmus
Which tumours are associated with posterior fossa syndrome and when?
Medulloblastoma - 12-48 hours postoperatively
What are the cardinal features of posterior fossa syndrome?
Hemiparesis
Mutism
Cerebellar dysfunction
Supranuclear cranial nerve palsy
In how many patients does posterior fossa syndrome persist?
50%
Which groups get non-Hodgkin lymphoma vs Hodgkin?
Non-Hodgkin is more common in childhood, and Hodgkin is more frequently seen in adolescence.
How does Hodgkin lymphoma present?
Painless lymphadenopathy, usually in the neck
Lymph nodes are larger and firmer than benign ones
Often a long clinical history
‘B’ symptoms are uncommon but include sweating, pruritus, weight loss, fever
How should a Hodgkin lymphoma be investigated?
Lymph node and bone marrow biopsy
What is the prognosis for Hodgkin lymphoma?
> 80% can be cured, 60% with disseminated disease
How does a non-Hodgkin lymphoma present?
Abdominal mass - usually B cell disease
Mediastinal mass in T cell
Can cause SVC obstruction
Head and neck masses - no specific cell
What is the prognosis for non-Hodgkin lymphoma?
> 80% in T and B cell disease
What is the pathophysiology of tumour lysis syndrome?
Occurs with bulky disease e.g. high count ALL, B cell NHL; undergoes lysis with treatment, resulting in the intracellular contents of potassium, phosphate and nuclear debris being released into the circulation
Uric acid crystals and phosphate precipite out with calcium into crystals.
What are the biochemical and clinical features of tumour lysis syndrome?
Fluid overload
High phosphate, potassium, urea and creatinine
Hypocalcaemia
How is tumour lysis syndrome treated?
Hyperhydration
Uric acid lowering agents e.g. urate oxidase or allopurinol
Treatment of hyperkalaemia
Consideration of fluid filtration or dialysis
Where does a neuroblastoma arise from?
Arises from neural crest tissue in the adrenal medulla and sympathetic nervous system
Who gets neuroblastoma?
Mostly under 5 years
How does neuroblastoma present?
Abdominal mass most commonly, but primary tumour can be anywhere along the sympathetic chain from neck to pelvis
Can cross the midline, enveloping major blood vessels and lymph nodes
May have paravertebral tumours causing spinal cord compression
Over 2 years, symptoms are mostly from metastatic disease: bone pain, bone marrow suppression causing weight loss, malaise
What investigations should be done in neuroblastoma?
Raised urinary catecholamines
Biopsy
Bone marrow sampling
MIBG scan
How is a neuroblastoma managed?
Surgery
Chemotherapy - may need high dose therapy with autologous stem cell rescue
Radiotherapy
What is the prognosis for neuroblastoma and what affects this?
Most children over 1 present with advanced disease and have a poor prognosis
Overexpression of N-myc, evidence of deletion on chromosome 1 and gain of material on chromosome 17q in tumour cells are associated with poorer prognosis
Risk of relapse is high
Cure rate is about 30%
Where does a Wilms tumour originate from?
Embryonal renal tissue
How does a Wilms tumour present?
Over 80% are under 5; very rare over 10 years
Large abdominal mass, often found incidentally in an otherwise well child
Haematuria
Hypertension
How is a Wilms tumour managed?
○ Chemotherapy
Delayed nephrectomy
Radiotherapy for more advanced disease
What is the prognosis in Wilms tumour?
> 80% cure rate
What is Wilms tumour also known as?
Nephroblastoma
What is the commonest soft tissue sarcoma in childhood?
Rhabdomyosarcoma
Where does a rhabdomyosarcoma originate from?
Primitive mesenchymal tissue
How does a rhabdomyosarcoma present?
Head and neck are the site in 40%:
Proptosis
Nasal obstruction
Bloodstained nasal discharge
Genitourinary: Dysuria Urinary obstruction Scrotal mass Bloodstained vaginal discharge
Metastatic disease
Present in approx 15% at diagnosis
What is the cure rate for rhabdomyosarcoma?
About 65%
What is langerhans cell histiocytosis?
Clonal accumulation and proliferation of abnormal bone marrow-derived Langerhans cells
These cells, functioning as potent antigen-presenting cells, along with white cells cause inflammatory tissue damage
What is the prognosis for langerhans cell histiocytosis?
Very variable - spontaneous regression to death
How is langerhans cell histiocytosis managed?
Corticosteroids
Vinblastine
Methotrexate
6-mercaptopurine
What is haemophagocytic lymphohistiocytosis?
Rare disease caused by abnormal proliferation of histiocytes in tissues and organs, causing an uncontrolled and ineffective immune response with high fatality rate
How is familial haemophagocytic lymphohistiocytosis inherited?
AR
What is the defect in familial haemophagocytic lymphohistiocytosis
5 genetic mutations which cause a defect in NK and T cell cytotoxic function, leading ot strong immunological activation of phagocytes and inflammatory mediators
How is haemophagocytic lymphohistiocytosis treated?
○ Active treatment with corticosteroids and chemotherapy first
Definitive treatment is bone marrow transplant
What is secondary haemophagocytic lymphohistiocytosis also known as?
Macrophage activating syndrome
What can precipitate haemophagocytic lymphohistiocytosis?
Infection, rheumatological, immune deficiencies, malignant and metabolic disorders
What are the typical findings in haemophagocytic lymphohistiocytosis?
Fever Hepatosplenomegaly Cytopenia Hypertriglyceridaemia Coagulopathy Hypofibrogenaemia Elevated soluble CD25 Liver dysfunction Elevated ferritin Neurological symptoms
Who doesn’t get bone tumours?
Pre pubertal children
What is the most common kind of bone tumour?
Osteogenic sarcoma
Which group is Ewing sarcoma more common in ?
Younger children
Where does osteogenic sarcoma often occur?
Predominantly in metaphyses of long bones
0% occurring at the knee joint
Commonly leads to lung metastasis
Where does Ewing sarcoma commonly occur?
More often in flat bones
Can be extra osseous rarely
What are the clinical features of bone tumours?
Limbs are the most common site
Persistent localised bone pain
At diagnosis, most are otherwise well
What investigations should be done if there is a suspected bone tumour?
Plain XR followed by MRI And bone scan
In Ewing sarcoma, there is often a substantial soft tissue mass
Chest CT to look for lung mets
Bone marrow sampling to exclude involvement
How are bone tumours managed?
○ Combination chemotherapy before surgery
Radiotherapy in Ewing sacoma
What is a retinoblastoma?
Malignant tumour of retinal cells
How is retinoblastoma inherited?
All bilateral tumours are hereditary, and 20% of unilateral cases
Gene is on chromosome 13q14
Dominant inheritance with incomplete penetrance
How does retinoblastoma present?
Usually within the first 3 years; children with hereditary form should be screened regularly from birth
White pupillary reflex instead of red
Squint
How is retinoblastoma managed?
Chemotherapy to shrink the tumours
Local laser treatment to the retina
Enucleation may be needed in more advanced disease, or radiotherapy
What is the prognosis in retinoblastoma?
Most patients are cured, but there is a significant risk of secondary malignancy among survivors of hereditary retinoblastoma
