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Paediatrics > Cancer > Flashcards

Cancer Flashcards

1
Q

Do paediatric brain tumours tend to be primary or secondary?

A

> 60% tend to be primary and are located infratentorialy.

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2
Q

What are the different types of brain tumours?

A

Astrocytoma, juvenile and non-juvenile types (40%), medulloblastoma (20%), epyndymoma (8%), brainstem glioma (6%), craniopharyngioma (4%)

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3
Q

What is the difference in the juvenile and non-juvenile types of astrocytomas?

A

Juvenile is slow growing with a good prognosis, non-juvenile is found in the cerebral hemispheres with a poor prognosis.

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4
Q

Where does a medulloblastoma usually arise from?

A

Arises in the midline of the posterior fossa, and up to 20% have spinal mets at DIAGNOSIS.

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5
Q

Where is an epyndymoma usually located?

A

Usually in the posterior fossa.

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6
Q

Where is an astrocytoma usually located?

A

In the cortex, supratentorial. (astro like stars up in the sky or the top of the brain)

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7
Q

Where is a craniapharyngioma usually located?

A

In the midline

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8
Q

Which tumours are usually located infratentorially?

A

Cerebellar- medulloblastoma, astrocytoma and ependymoma and brainstem- brainstem glioma.

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9
Q

Which tumours are usually located in the spinal cord?

A

Astrocytome and ependymoma.

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10
Q

What is the leading cause of cancer death in children in the UK?

A

Brain tumours

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11
Q

What are the clinical features of a brain tumour?

A

Usually related to the amount of raised ICP present, may also exhibit focal signs on site of tumour.

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12
Q

What are signs of raised ICP in infants?

A

Vomiting, separation of sutures, tense fontanelle, inc. head circumference, head tilting/posturing, developmental delay/regression.

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13
Q

What are signs of a raised ICP in children?

A

Headache- WORSE IN MORNING, vomiting in the morning when waking up, behaviour change, visual disturbance and papilloedema.

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14
Q

What are the symptoms if the tumour is supratentorial (in the cotex)?

A

Seizures, hemiplegia, focal signs. (astocytoma/glioblastoma multiforme)

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15
Q

What are the symptoms if the tumour is midline?

A

Visual field loss, bitemporal hemianopia, pituitary failure, diabetes insipidus, weight gain. (craniopharyngioma)

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16
Q

What are the symptoms if the tumour is in the cerebellum/4th ventricle?

A

Truncal ataxia, coordination difficulties, abnormal eye movements. (medulloblastoma)

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17
Q

What are the symptoms if the tumour is in the brainstem?

A

Cranial nerve defects, pyramidal tract signs, cerebellar signs (ataxia), often no ICP. (brainstem glioma)

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18
Q

NB

A

Persistance back pain should always warrant an MRI, and head aches and behavioural changes may be a sign go raised ICP.

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19
Q

Why should an LP never be performed if a brain tumour is suspected?

A

If there is raised ICP, removing the CSF from the spinal canal can cause a reduction is pressure, acts like a suction and pulls the brainstem through the foramen magnum (herniation).

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20
Q

What is leukaemia?

A

Group of cancers, originating in the bone marrow and results in a high number of abnormal WBCs. Lymphoblastes differentiate into T or B cells (plasma cells) therefore ALL leads to a raised WCC due to acute proliferation.
Bone marrow is also responsible for the production of other cells- RBCs, WBCs, platelets… in ALL the overproduction of lymphoblasts causes inhibited production of all other normal cells.

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21
Q

What is the most common childhood malignancy?

A

Leukaemia- accounting for 25%, whilst ALL is the most common childhood leukaemia accounting for 80% of paediatric cases. (the remainder is usually acute myeloid/acute non-lymphocytic anaemia) (chronics are rare)

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22
Q

What is the common age of prevention of leukaemia?

A

2-6

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23
Q

What are the clinical features of leukaemia?

A

Short history (days or weeks), symptoms result from disseminated disease, reduced normal cell production and systemic ill heath. SO malaise, anorexia, Reticulo-endothelial infiltration causing hepatosplenomegaly, lymphadenopathy. BM infiltration causing anaemia, neutorpenia(infection), thrombocytopenia (bruising) and bone pain. CNS-head aches, vomiting, nerve palsies, tests- testicular enlargement.

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24
Q

What investigations are carried out in leukaemia?

A

FBC- showing thrombocytopenia, low Hb and increasing circulating leukaemia blast cells.
BM- confirms diagnosis and identifies immunological and cytogenetic characteristics useful in determining prognosis.
CXR- identifies a mediastinal mass, characteristic of t cell disease.

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25
Q

What are the 4 stages of treatment of leukaemia?

A

Induction of remission, intensification, CNS consolidation, continuing therapy and relapse.

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26
Q

What is induction of remission?

A

It is 4 weeks long and this implies eradication of leukaemia blasts and restoration of normal bone marrow function, combination chemo is given and current treatment schedules give remission rates of 95%.

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27
Q

What is intensification?

A

Block of intense chemo given to consolidate remission- improves cure rates but increases toxicity.

28
Q

What is CNS consolidation?

A

Cytotoxic drugs used in chemo poorly penetrate the CNS meaning leukaemia cells may survive here. Additional treatment is given intrathecally to prevent CNS relapse

29
Q

What is continuing therapy?

A

Chemo of modest intensity continued over period of time, up to 3 years.

30
Q

What is relapse?

A

High intensity chemo used.

31
Q

What is lymphoma?

A

Group of blood cancers found in lymphocytes, can be grouped into hodgkins and non-hodgkins.

32
Q

Which type of lymphoma is more common in childhood?

A

NHL, (HL is more common in adolescence)

33
Q

In the general population, 90% of lymphomas are…?

A

NHL

34
Q

What age does hodgkins lymphoma usually strike?

A

Very rare before the age of 5 and rises with age.

35
Q

What type of infection commonly precedes hodgkins lymphoma?

A

EBV

36
Q

What does histology look like in hodgkins lymphoma?

A

Reed-steinberg cells present

37
Q

How does hodgkins lymphoma commonly present?

A

Commonly presents with painless lymphadenopathy, most frequently in the neck or the mediastinum. (lymph nodes may become so large that they cause airway obstruction). Clinical history is often long- over months.

38
Q

Although B symptoms are not often present, what are they?

A

Sweating, weight loss, pruritus, fever

39
Q

What investigations are required in NHL?

A

LN biopsy, radiological assessment of all nodal sites and bone marrow biopsy

40
Q

What is seen in serology of NHL?

A

EBV serology?

41
Q

Describe stage 1 NHL?

A

Single site

42
Q

What system stages NHL?

A

Ann Arbor system

43
Q

Describe stage 2 NHL?

A

More than one site and on one side

44
Q

Describe stage 3 NHL?

A

On both sides of the diaphragm

45
Q

Describe stage 4 NHL?

A

Disseminated disease

46
Q

What is the prognosis for NHL?

A

Good prognosis, 90% can be cured, even with disseminated disease, cure rate is 60%.

47
Q

What are the 2 types of hodgkins lymphoma?

A

Lymphoblastic (T-cell/pre-B) and mature B cell (Burkitt/Burkitt like) large lymphoma

48
Q

So lymphoblastic lymphoma involves what cells?

A

T-cells and pre-B cells

49
Q

What are the clinical features of NHL?

A

May also present with a mediastinal mass and varying degrees of BM infiltration, may also cause SVC obstruction.
B cell malignancies present more commonly with localised LN disease, usually in the head, neck or abdomen.
Abdominal disease will present as pain (intestinal obstruction), palpable mass or intussception in some cases.

50
Q

What is the staging system for NHL?

A

St Jude System

51
Q

Describe stage 1 NHL?

A

Single site or nodal area (NOT abdomen or mediastinum)

52
Q

Describe stage 2 NHL?

A

Regional nodes, abdominal disease

53
Q

Describe stage 3 NHL?

A

Disease on both sides of the diaphragm

54
Q

Describe stage 4 NHL?

A

Bone marrow or CNS disease (INCLUDE examination of CSF in investigations of NHL, as well as biopsy of nodes etc & bone marrow)

55
Q

What is the management of NHL?

A

Lymphoblastic is treated like ALL; Mature-B treated with series of intense chemotherapy
Survival rates are ~80%

56
Q

What is a neuroblastoma?

A

It is a tumour arising from the neural crest tissue in the adrenal medulla and sympathetic nervous system.

57
Q

When are neuroblastomas most commonly diagnosed?

A

Before age of 5, average is 2 years.

58
Q

What is the clinical presentation of a child with neuroblastoma?

A

Most children have an abdominal mass but primary tumour can arise from anywhere along the sympathetic chain from the neck to the pelvis.
CF = weight loss, pallor, abdo mass, hepatomegaly, bone pain, limp.
Less common= paraplegia, cervical lymphadenopathy, proptosis, periorbital bleeding, skin nodules.

59
Q

How do you investigate for a neuroblastoma?

A

Raised urine catecholamine. Also ultrasound, MRI

60
Q

How do you manage a neuroblastoma?

A

localised can be treated with surgery alone
Metastatic disease treated with chemotherapy, surgery & radiotherapy - risk of relapse is higher
& prospect of cure is ~30%
• Majority of children present with late stage disease & have a poor prognosis

61
Q

What is an MIBG scan?

A

An MIBG scan is used to look for uncontrolled or abnormal cell growth in the body. It maps metastatic tumour marrow. The dark areas are evidence of high isotope uptake and the pattern is consistent with widespread metastatic disease.

62
Q

What is a Wilm’s tumour?

A

It is a tumour which originates from embryonal renal tissue (most common renal tumour of childhood).

63
Q

What age do children present with Wilms’ tumours?

A

> 80% present before age 5, rarely seen after 10

64
Q

What is the most common presentation with a Wilms’ tumour?

A

Most common present with a large abdominal mass found incidentally in an otherwise normal child.

65
Q

What is the management of a wilms tumour?

A

Initial chemo followed by delayed nephrectomy. (80% cure rate- good prognosis).

Paediatrics (23 decks)

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