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1MRCPCH FOP and TAS > Haematology/Oncology > Flashcards

Haematology/Oncology Flashcards

1
Q

Which type of cancer is related to Down syndrome?

A

Leukaemia

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2
Q

What type of cancer is associated with neurofibromatosis?

A

Glioma

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3
Q

Which cancers are associated with EBV infection?

A

Burkitt lymphoma
Hodgkin lymphoma
Nasophargyngeal carcinoma

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3
Q

What type of cancer is HepB infection associated with?

A

Liver carcinoma

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3
Q

Which immunotherapeutic agent is used for Ph+ve ALL and CML?

A

Imatinib

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3
Q

What type of infection is Kaposi sarcoma associated with?

A

HIV
HHV8

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3
Q

What does imatinib work on to provide cancer growth inhibition?

A

Tyrosine kinase

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3
Q

Which gene mutation causes Philadelphia +ve ALL and CML

A

BCR-ABL fusion gene

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4
Q

What is the significant side effect associated with vincristine?

A

Neuropathy

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4
Q

What are the symptoms of bone marrow infiltration assoc. with ALL?

A

Anaemia - pallor, lethargy
Neutropenia - infections
Thrombocytopenia - bruising, petechiae, nosebleeds
Bone pain

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4
Q

What are the genital symptoms of ALL?

A

Testicular enlargement

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4
Q

Which monoclonal antibody is anti-CD20 ?

A

Rituximab

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5
Q

What is the significant side effect associated with doxorubicin?

A

Cardiotoxicity

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5
Q

What is the peak age of presentation of ALL?

A

2-5years

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6
Q

What is the significant side effect associated with cisplatin?

A

Renal toxicity

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6
Q

What is the significant side effect associated with cyclophosphamide?

A

Haemorrhagic cystitis

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6
Q

What % of childhood leukaemias does ALL represent?

A

80%

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7
Q

What are the signs of CNS involvement in ALL?

A

Headaches
Vomiting
Nerve palsies

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7
Q

What are the general symptoms assoc. with ALL?

A

Malaise
Anorexia

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8
Q

Other than FBC and bone marrow, what other investigations should be ordered in ALL?

A

CXR
LP - identifies CNS disease
Clotting

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8
Q

What is the remission rate in ALL following induction chemotherapy?

A

95%

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8
Q

What are the signs of reticuloendothelial infiltration in ALL?

A

Hepatosplenomegaly
Lymphadenopathy
Superior mediastinal obstruction

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8
Q

What are poor prognostic factors in ALL?

A

Age <1 or >10
WCC >50
MLL rearrangement or t(4;11) hypodiploidy
Persistence of blast in bone marrow after initial chemo regime
MRD +ve after induction therapy

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9
Q

Which immunological phenotype is most common in ALL?

A

B-cell ALL (75%)

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9
Q

What are the typical FBC findings in ALL?

A

Low Hb
Low PLTs
Evidence of circulating blast cells

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9
Q

What is given prior to chemo in ALL to prevent TLS?

A

Additional Iv hydration
Allopurinol

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10
Q

What type of sample is essential for diagnosis and prognosis/treatment planning of ALL?

A

Bone marrow

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11
Q

What treatment options are considered for relapse of ALL?

A

High dose chemo
Whole body irradiation
Bone marrow transplant

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11
Q

What is the most common type of brain tumour in children?

A

Astrocytoma

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11
Q

What is a highly malignant astrocytoma called?

A

Glioblastoma

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12
Q

Which medication is given to ALL patients to prevent PCP?

A

Co-trimoxazole

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12
Q

Where do medulloblastoma tumours arise in the brain?

A

Midline of posterior fossa

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12
Q

What percentage of medulloblastoma have spinal mets at diagnosis?

A

20%

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12
Q

Where do ependymomas typically originate?

A

4th ventricle
May occur anywhere is CSF spaces

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12
Q

Where do craniopharyngeomas typically originate?

A

Squamous remnant of the Rathke pouch

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12
Q

How do brain tumours typically present in older children and adolescents?

A

Persistent or recurrent headaches
Blurred or double vision
Lethargy
Deteriorating school performance
Delayed or arrested puberty

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12
Q

How do brain tumours typically present in infants?

A

Developmental delay/regression
Progressive increase in HC
Lethargy

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13
Q

How would a tumour in the supratentorial cortex typically present?

A

Seizures
Hemiplegia
Focal neurological signs
Aggressive behaviour at school
Headaches

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13
Q

What is the likely diagnosis of a tumour located in the supratentorial cortex?

A

Astrocytoma - glioblastoma multiforme

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13
Q

What is the typical management of a tumour in the supratentorial cortex e.g glioblastoma multiforme?

A

Surgery, XRT +/- chemo

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13
Q

What is the prognosis of a supratentorial cortex tumour?

A

Poor
Typically <30%

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14
Q

Where are astrocytomas typically found?

A

Cerebral hemispheres
Thalamus
Hypothalamus

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14
Q

How do midline tumours typically present?

A

Visual field loss (bitemporal hemianopia)
Pituitary failure (growth failure)
DI
Increased weight
Headaches
Vomiting

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14
Q

What would be the typical diagnosis of a midline tumour?

A

Craniopharyngeoma

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15
Q

How is a cranipharyngeoma managed?

A

Surgical excision
+/- XRT

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15
Q

What is the long term prognosis of craniopharyngeoma?

A

Good survival
Risk of long term visual impairment and pituitary insufficiency

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15
Q

How do tumours of the cerebellum and 4th ventricle present?

A

Truncal ataxia
Coordination difficulties
Abnormal eye movements
Vomiting in the mornings
New-onset converging squint

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15
Q

What is the usual diagnosis of a cerebellum and 4th ventricle tumour?

A

Medulloblastoma

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16
Q

What is the treatment of medulloblastoma?

A

Surgery
Chemo
XRT

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16
Q

What is the prognosis of medulloblastoma?

A

Survival rate is around 50%

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16
Q

How do brainstem tumours typically present?

A

Cranial nerve defects
Pyramidal tract signs
Cerebellar signs
Refusing to walk
Facial asymmetry and droolong

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17
Q

What is the usual diagnosis of a brainstem tumour?

A

Brainstem glioma

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17
Q

What is the usual treatment in brainstem glioma?

A

Palliative XRT

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17
Q

What is the prognosis of brainstem glioma?

A

<10%

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18
Q

How can spinal tumours present?

A

Back pain
Peripheral weakness in arms and legs
Bladder/Bowel dysfunction

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19
Q

Is non-Hodgkin lymphoma more common in childhood or adolescence?

A

Childhood

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20
Q

Is Hodgkin lymphoma more common in childhood or adolescence?

A

Adolescence

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21
Q

What are the clinical features in Hodgkin lymphoma?

A

Painless lymphadenopathy
Nodes may compress airway or SVC
History often many months long
Systemic symptoms: sweating, weight loss, fever and pruritus - uncommon even in advanced disease

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21
Q

What is the management of Hodgkins lymphoma?

A

Combination chemo +/- XRT
PET scans used to monitor response

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22
Q

What investigations should be done in hodgkins lymphoma?

A

Lymph node biopsy
Radiological assessment of nodal sites
Bone marrow biopsy

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22
Q

What are the cure rates for hodgkins lymphoma?

A

80%
60% for disseminated disease

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23
Q

How does T-cell Hodgkins lymphoma present?

A

Mediastinal mass +/- SVC obstruction
Varying degree of bone marrow infiltration

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24
Q

What are the signs of SVC obstruction?

A

Dyspnoea
Facial swelling and flushing
Venous distension of neck, upper chest and arms

25
Q

How is NHL managed?

A

Multiagent chemo

25
Q

How does B-cell NHL present?

A

Lymphadenopathy in neck, head or abdomen with a short history of illness
Abdo disease may cause obstruction, mass or intussusception

25
Q

What is the prognosis of NHL?

A

Survival >80%

25
Q

What investigations should be done in NHL?

A

Biopsy
Radiological assessment of all nodal sites
Examine bone marrow and CSF

26
Q

Where is Burkitt lymphoma most commonly seen in the world?

A

Malaria endemic areas in Africa

26
Q

Which viral infection is associated with Burkitt lymphoma?

A

EBV

26
Q

Where does neuroblastoma arise from?

A

Neural crest tissue in the adrenal medulla and sympathetic nervous system

26
Q

In which group of people can neuroblastoma sometimes spontaneously resolve?

A

Infants <1y/o

26
Q

What are the common features of neuroblastoma?

A

Pallor
Weight loss
Abdominal mass
Hepatomegaly
Bone pain
Limp

26
Q

What are the less common features of neuroblastoma?

A

Paraplegia
Cervical lymphadenopathy
Proptosis
Periorbital bruising
Skin nodules
Opsoclonus myoclonus

26
Q

What investigations should be done in neuroblastoma?

A

Urinary catacholamine levels - VMA/HVA raised
Biopsy
Bone marrow sampling
MIBG scan

26
Q

What age range does neuroblastoma typically present in?

A

<5y/o

26
Q

Amplification of which oncogene confers a poor prognosis in neuroblastoma?

A

MYCN

27
Q

What percentage of Wilms tumour presents <5y/o

A

80%

27
Q

What is the prognosis in metastatic neuroblastoma?

A

Cure rate is 40%

27
Q

How is localised neuroblastoma treated?

A

Surgery alone

27
Q

How is metastatic neuroblastoma treated?

A

Chemo
XRT
Surgery

27
Q

Where does Wilms tumour originate from?

A

Embryonal renal tissue

28
Q

Which syndrome is associated with Wilms tumour?

A

Beckwith-Wedemann syndrome

28
Q

What are the common presenting features of Wilms tumour?

A

Abdo mass (often only Sx in an otherwise well child)
Haematuria

28
Q

What investigations are done in Wilms tumour?

A

USS
CT/MRI for staging

28
Q

What are the less common presenting features in Wilms tumour?

A

Abdo pain
Anorexia
Anaemia
HTN

29
Q

What is the management for Wilms tumour?

A

Initial chemo
Delayed nephrectomy
XRT in advanced disease

29
Q

What percentage of Wilms tumour is bilateral at diagnosis?

A

5%

30
Q

What percentage of Wilms tumour has metastatic disease at presentation?

A

15%

31
Q

What is the cure rate for Wilms tumour?

A

80%

32
Q

What is the most common type of soft tissue sarcoma in chdilren?

A

Rhabdomyosarcoma

32
Q

What is the cure rate for metastatic disease in Wilms tumour?

A

60%

32
Q

How do bone tumours typically present?

A

Persistent localised bone pain
Most otherwise well

33
Q

Where does Rhabdomyosarcoma originate from?

A

Primitive mesenchymal tissue

33
Q

On which chromosome is the retinoblastoma susceptability gene located?

A

13

34
Q

How would a Rhabdomyosarcoma present in the head/neck region?

A

Proptosis
Nasal obstruction
Blood-stained nasal discharge

34
Q

What is the cure rates of Rhabdomyosarcoma?

A

Around 65%

34
Q

How would a Rhabdomyosarcoma present in the GU tract?

A

Dysuria
Urinary obstruction
Scrotal mass
Blood stained PV discharge

35
Q

What percentage of Rhabdomyosarcoma is metastatic at diagnosis?

A

15%

35
Q

How are bone tumours managed?

A

Combination chemo prior to surgery
En bloc resection can avoid amputation
XRT in Ewing sarcoma

36
Q

What investigations should be done in Rhabdomyosarcoma?

A

Biopsy
Radiological assessment

36
Q

How is Rhabdomyosarcoma managed?

A

Mixture of chemo/XRT/surgery depending on location/stage of tumour and age of patient

36
Q

Are Ewing sarcoma and Osteosarcoma more common in males or females?

A

Males

37
Q

How does Kaposi present?

A

Generalised lymphadenopathy
Purple/brown skin rash (rarer in children)

37
Q

Is osteosarcoma or Ewing sarcoma more common in younger children?

A

Ewing

37
Q

What investigations should be done in bone tumours?

A

Plain x-ray
MRI and bone scan
CT scan for lung or other mets
Bone marrow sampling

37
Q

How is Kaposi sarcoma diagnosed?

A

Biopsy

37
Q

How do liver tumours present in children?

A

Abdo distension or abdo mass
Rarely pain or jaundice

37
Q

At what age does retinoblastoma typically present?

A

Within first 3 years of life

37
Q

How many retinoblastoma cases are hereditary?

A

All bilateral cases
20% unilateral cases

38
Q

How do bone tumours appear on an x-ray?

A

Destruction and variable periosteal new bone formation
In Ewing - substantial soft tissue mass

38
Q

Where is Kaposi sarcoma common?

A

Sub-Saharan Africa

39
Q

How is retinoblastoma inherited?

A

AD with incomplete penetrance

39
Q

What are the commonest primary liver tumours in children?

A

Hepatoblastoma (65%)
Hepatocellular carcinoma (25%)

39
Q

How is retinoblastoma managed?

A

Chemo
Local laser therapy to retinas
XRT in more advanced disease

39
Q

What marker is almost always raised in hepatoblastoma and some hepatocellular carcinoma?

A

Alpha-fetoprotein

39
Q

How does retinoblastoma typically present?

A

White pupillary reflex
Squint

39
Q

What investigations should be done in retinoblastoma?

A

MRI
EUA

40
Q

What causes Kaposi sarcoma?

A

HHV8

40
Q

How is Kaposi sarcoma treated?

A

Chemo
Antiretroviral therapy

41
Q

How are liver tumours typically managed?

A

Chemo
Surgery
In inoperable cases - transplantation

42
Q

What is the prognosis for primary liver malignancy?

A

Hepatoblastoma - most cured
Hepatocellular carcinoma - less favourable

43
Q

Where do germ cell tumours arise from?

A

Primitive germ cells which migrate from yolk sac endoderm

44
Q

Where do most benign germ cell tumours form?

A

Sacrococcygeal region

45
Q

Where do most malignant germ cell tumours form?

A

Gonads

45
Q

What markers are important to check in germ cell tumours?

A

Alpha-fetoprotein
B-HCG

45
Q

How are germ cell tumours managed?

A

Chemotherapy

46
Q

What is the general prognosis in germ cell tumours?

A

Good outcomes

46
Q

How does Langerhans cell histiocytosis present?

A

Bone lesions:
- pain, swelling, fracture
DI
- skull disease with hypothalamic infiltration
Systemic
- seborrheic rash
- soft tissue involvement

46
Q

What is Langerhans cell histiocytosis?

A

Abnormal proliferation of histiocytes which is responsive to chemotherapy

46
Q

What does x-ray typically show in Langerhans cell histiocytosis?

A

Characteristic lytic lesions with well defined borders

47
Q

Which cancer is the C-myc oncogene associated with?

A

Burkitt’s lymphoma

47
Q

Which cancer is the BCR-ABL oncogene associated with?

A

Philadelphia chromosome in CML

48
Q

Which cancer is the Cyclin D oncogene associated with?

A

Breast
Oesophageal
Bladder
Lung

49
Q

Which cancer is the HER2 proto-oncogene associated with?

A

Breast cancer

49
Q

Which cancer is the RAS oncogene associated with?

A

Solid tumours

50
Q

Mutations in what chromosome are associated with Beckwith-Wiedemann syndrome?

A

11p15

50
Q

What are the typical features of Beckwith-Wiedemann syndrome?

A

Macrosomnia
Asymmetric limb growth
Macroglossia
Neonatal hypoglycaemia
Umbilical hernias
Increased rates of cleft palate and various tumours (most common is Wilms)

51
Q

In what malignancy does N-myc confer a poor prognosis?

A

Neuroblastoma

51
Q

What would be seen in a blood film for hereditary spherocytosis?

A

Small, dense, hyperchromic RBCs without central pallor

51
Q

What causes hereditary spherocytosis?

A

Genetic mutation in the structural proteins in the RBC membrane (ankyrin and spectrin)

52
Q

Deficiency of what factor is noted in haemophilia A?

A

Factor VIII

52
Q

How is G6PD deficiency inherited?

A

X-linked recessive

53
Q

What would typically be seen on the blood film in a post-splenectomy patient?

A

Howell-Jolly bodies - spherical granular inclusions in erythrocytes

53
Q

Which monoclonal antibody is used for resistant ITP?

A

Rituximab

54
Q

What causes Glanzmann’s thrombasthenia?

A

Deficiency of glycoprotein IIb/IIIa on the surface of the platelet

54
Q

What are the symptoms of Glanzmann’s thrombasthenia?

A

Easy bruising
Excessive bleeding after trauma or epistaxis

55
Q

How is Glanzmann’s thrombasthenia inherited?

A

AR (occasionally AD)

56
Q

What would bee seen in investigations in a diagnosis of Glanzmann’s thrombasthenia?

A

Normal platelet count
Prolonged bleeding time

57
Q

What are the symptoms of vWD?

A

Menorrhagia
Recurrent nosebleeds
Easy bruising
FHx clotting disorders

58
Q

How is vWD inherited?

A

AD

58
Q

What role does vWF play in haemostasis?

A

Platelet activation
Adherence to the damaged epithelium

58
Q

What deficiency presents with inability to prolong APTT which a heparin infusion?

A

Antithrombin III deficiency

59
Q

What does antithrombin II do?

A

Inhibits thrombin - deficiency leads to VTE

60
Q

What is the main function of astrocytes?

A

Control of the blood brain barrier

61
Q

How is haemophilia B inherited?

A

X-linked recessive

62
Q

Where are the most common sites of extramedullary ALL relapse in children?

A

Testicles
Brain

63
Q
A

1MRCPCH FOP and TAS (23 decks)

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