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Peads > Malignancy > Flashcards

Malignancy Flashcards

1
Q

how do brain tumours in children differ to adults?

How common are brain tumours in children?

A
  • They are more often primary (unlike adults)
  • And they are usually infratentorial meaning they are in the cerebellum or below
  • Brain tumours are the most common solid tumour in children (+the leading cause of cancer death in children)
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2
Q

What are some common types of brain tumours

A

Astrocytoma (40%) (completely benign or highly malignant
Medullablastoma (20%)-check for spinal mets
Ependymyoma (8%)
Craniopharyngoma (remnant of Rathke’s pouch-not malignant but local invasion)

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3
Q

What are some symptoms of brain tumours in children? (lots)

How might children under 4 present

A
  • Either general signs of raised ICP (headache, nausea, vomiting, walking, coordination and vision changes-papilodema)
  • face changes (cranial nerves)
  • Weight loss/precocious puberty/short stature
  • spinal tumours-incontinence, weakness in limbs, pain
  • children under 4 might have macrocephaly, lethargy and hyperrexlexia
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4
Q

How should brain tumours be investigated?

A

Brain tumours visualised on MRI scan

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5
Q

How should brain tumours be managed?

A
  • Surgery if possible with the main incentive be to alleviate any hydrocephalus
  • Sometimes anatomical position means excision is not safe -For this reason even tumours that are histologically benign can cause death
  • Prognosis is not good (even survivors might have neuro problems)
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6
Q

What is a Wilm’s tumour/nephroblastoma?

A
  • Tumour arising from the embryonic renal tissue - this means it will be present from birth but might not be found until later as children won’t develop symptoms straight away
  • tumour suppressor gene on chromosome 11
  • COMMONEST RENAL TUMOUR OF CHILDHOOD
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7
Q

What are the symptoms of Wilms tumour?

How old are children when they present?

A
  • Most usually with large abdominal masses that are found incidentally - ASYMPTOMATIC
  • Symptoms may include:
    • HEAMATURIA AND HYPERTENSION
    • abdominal pain
    • anorexia
    • anemia (bleeding)
  • Most children present before 5 years
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8
Q

How should a Wilm’s tumour be investigated/diagnosed?

A

MRI, USS and CT are all useful
LOOK IN LUNG FOR METS
-Biopsy of tumour to confirm (will contain bone, muscle and cartilidge)

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9
Q

How are Wilm’s tumours managed?

A
  • There is usually initial chemotherapy and then later nephrectomy (useful to stage)
  • Radiotherpay is sometimes offered to patients with very advanced disease
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10
Q

What is the prognosis for Wilm’s tumour?

What is a syndrome associated with Wilms tumour?

A
  • GOOD - approx 80%. 60% for mets

- WAGR syndrome associated in 10% (Wilms tumour/aniridia (black eyes with no colour) /GU problems/ retardation (mental)

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11
Q

What are the most common forms of malignancies in children?

What is the most common childhood cancer?

A

HAEMATOLOGICAL MALIGNANCY

Specifically LEUKAEMIA IS THE MOST COMMON CANCER IN CHILDHOOD

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12
Q

What is the most common form of leukaemia in kids?

A

Acute lymphoblastic leukaemia ALL

a fairly large chunk also AMLs

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13
Q

What is the peak of incidence of leukaemia?

Why do symptoms occur and how quickly do they occur?

A

Early childhood peak onset: 2-5 years

  • Symptoms occur due to disseminated disease and the infiltration of malignant cells into tissues (leukaemic blast cells)
  • Usually they come on over a course of a few weeks but can be much more rapid
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14
Q

What are some symptoms of ALL in children? (5 categories)

A

GENERAL:

  • Malaise
  • Anorexia

BONE MARROW (x3):

  • Neutropenia (infection due to rubbish neutrophils)
  • Anaemia (pallor and lethargy)
  • Thrombocytopenia (nose bleeds-always think ALL, bruising)
  • Bone pain

RETICULO-ENDOTHELIAL:
-Hepatosplenomegaly and lymphadenopathy (can obstruct-breathing problems?)

CNS (headaches, vomiting and nerve palsies)
TESTES (testicular enlargement)

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15
Q

What investigations should be done for ALL?

A

FBC - (anaemia, thrombocytopenia, neutropenia)
Blood film-look for leukaemia blast cells
Us and Es
Liver function and coagulation
BONE MARROW biopsy to diagnose
CXR to look for mediastinal mass (T cell disease)

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16
Q

When would changes be seen on CXR?

A
  • Mediastinal involvement in CXR would be suggestive of T cell disease
  • T cell disease occurs in 15%
  • B cell disease occurs in 75% (rest are mixed)
17
Q

How is ALL managed initially?

A

Make them fit for chemotherapy:

  • Correct anaemia (possibly with transfusion)
  • Platelet transfusion for bleeding
  • Leukopharesis given for leukostasis (lack of perfusion due to white cells-emergency)
  • Blood cultures and antibiotics if granulocytopenia

Prevent tumour lysis syndrome:

  • Hydration
  • Allopurinol for RENAL PROTECTION (increase in Ph, K, uric acid)

-Then after 4 weeks of this start chemotherapy

18
Q

What is the chemotherapy treatment schema for ALL?

A
  1. REMISSION INDUCTION
    • Reduce blast cells to undetectable account
    • Restore normal bone marrow function
      ○ Vincristine and steroid (dex)
      ○ An anthracycline drug such as daunorubicin
      ○ Asparaginase
    1. CNS/TESTES PROTECTION and CONSOLIDATION
      - Because relapse happens in 100% of cases
      ○ High dose methotrexate
      ○ Repeat induction
    2. MAINTANANCE (around 2 years)
      ○ 6 MP (mercaptopurine)
      ○ Methotrexate low dose
19
Q

What are some poor prognostic factors for ALL?

A

-If child aged under 1 or above 10
-If WCC is >20x10^9 (high tumour load)
-Slow speed of response to initial chemotherapy
-Minimal residual disease assessment
-Males do worse
-Non-caucasians do worse
-T or B cell surface markers
- Cytogenic/molecular genetic abnormalities in tumour cells
○ e.g. MLL rearrangement t(4;11)
○ Philidelphia chromosome (9,22)

20
Q

Is Hodgkin’s or Non-Hodgkin’s lymphoma more common in children?

A

NHL - no Reed-Sternberg Cells

21
Q

How does lymphoma usually present?

A

-Large, non-tender cervical lymphadenopathy
-Sometimes there are B symptoms
(night sweating, weight loss, pruritus, fever)

22
Q

How can you tell between benign lymphadenopathy and lymphoma?

A
  • Lymphadenopathy in lymphoma is larger and a lot firmer

- Can cause obstruction(Airway/superior vena cava/spinal cord/gut)

23
Q

If a child presents with extensive lymphadenopathy how should they be diagnosed?

A
  • Lymph node biopsy
  • Radiological assessment of all lymph node sites
  • Bone marrow biopsy
  • Blood film

(can also do uric acid and IgG/IgM level)

24
Q

In ALL what areas should you further protect in treatment and why?

A

drugs dont easily penetrate CNS and TESTES

  • CNS give intrathecal methatrexate and radiation
  • TESTES give radiation PROTECTION (can freeze sperm)
25
Q

In ALL what should you give for philidelphia chromosome?(t9.22)

A

For ALL patients with Philadelphia (t9.22) chromosome translocation give a tyronise kinase inhibitor (e.g. imatinib)

26
Q

.

A

.

27
Q

Which lymphoma do you get Reed-Sternberg Cells

A

Reed-Sternberg Cells are found in Hodjkins lymphoma affecting the B cells (hodjkins is bae)

28
Q

What does a mediastinal mass indicate?

A

T cell disease (either Non hodjkins or acute lymphoblastic anaemia)

29
Q

What has better survival Hodjkins of Non Hodjkins?

A

Hodjkins has better prognosis

30
Q

What are the classic B cell symptoms?

A

B Cell symptoms (more common in hodjkins because non hodjkins also includes T cell)

Night sweats
Weight loss
Fever
Itching

31
Q

The difference between Hodjkins and Non Hodjkins?

what cells
presentation
age

A

Cells

  • Hodjkins is B cell (can be affected by EBV)
  • Non Hodjkins is T or B (majority B)

Presentation

  • Hodjkins normally neck lump
  • Hodjkins presents earlier stage (better prognosis)

Age
-Hodjkins is younger adults

32
Q

What is the treatment for lymphoma

A
  • Combination chemotherapy with or without radiotherapy
  • PET used to monitor treatment response
    Prognosis is good with around 80% of patients making a full recovery and about 60% of patients even with disseminated disease making a recover
33
Q

Whats a neuroblastoma?

A

What is a neuroblastoma?

  • Most common tumour in INFANTS (younger presentation is better 1yr+ poor prognosis)
  • This is a brain tumour that arises from the NEURAL CREST TISSUE in the ADRENAL MEDULLA and SYMPATHETIC NERVOUS SYSTEM.
34
Q

What are the signs and symptoms of neuroblastoma?

A

SIGNS
-Can have abdominal mass if originating in adrenal gland
-Compression of complicated tumour
○ Lungs- breathing difficulties
○ Facial nerves (Horners syndrome miosis/ptosis/anhidrosis)
○ Spinal cord -bowel/bladder problems and muscle weakness

  • In terms of SYMPTOMS they are usually of metastatic disease:
    ○ Bone pain/bone fractures (classically base of skull fracture-Racoon eyes
    ○ Bone marrow problems (tiredness/bruising/bleeding/infection)
    ○ Weight loss
35
Q

Investigations of neuroblastoma?

A

-MEASURE CATECHOLAMINES
Confirmatory biopsy
CT scan (how big)
Bone scan and FBC to assess bone marrow spread

36
Q

Treatment of neuroblastoma?

A

Treatment of neuroblastoma

  • Surgery if early
  • Chemotherapy/stem cell transplantation if late

Peads (29 decks)

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