Malignancy Flashcards by Seldon House

how do brain tumours in children differ to adults?

How common are brain tumours in children?

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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What are some common types of brain tumours

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

How might children under 4 present

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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How should brain tumours be investigated?

Brain tumours visualised on MRI scan

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How should brain tumours be managed?

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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What is a Wilm’s tumour/nephroblastoma?

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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What are the symptoms of Wilms tumour?

How old are children when they present?

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

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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How are Wilm’s tumours managed?

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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What is the prognosis for Wilm’s tumour?

What is a syndrome associated with Wilms tumour?

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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What are the most common forms of malignancies in children?

What is the most common childhood cancer?

HAEMATOLOGICAL MALIGNANCY

Specifically LEUKAEMIA IS THE MOST COMMON CANCER IN CHILDHOOD

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What is the most common form of leukaemia in kids?

Acute lymphoblastic leukaemia ALL

a fairly large chunk also AMLs

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What is the peak of incidence of leukaemia?

Why do symptoms occur and how quickly do they occur?

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

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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What investigations should be done for ALL?

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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When would changes be seen on CXR?

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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)

How is ALL managed initially?

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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

What is the chemotherapy treatment schema for ALL?

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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

What are some poor prognostic factors for ALL?

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-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)

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

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NHL - no Reed-Sternberg Cells

How does lymphoma usually present?

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

How can you tell between benign lymphadenopathy and lymphoma?

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Lymphadenopathy in lymphoma is larger and a lot firmer

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

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

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

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

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

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drugs dont easily penetrate CNS and TESTES

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

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

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

Which lymphoma do you get Reed-Sternberg Cells

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

What does a mediastinal mass indicate?

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

What has better survival Hodjkins of Non Hodjkins?

Hodjkins has better prognosis

What are the classic B cell symptoms?

B Cell symptoms (more common in hodjkins because non hodjkins also includes T cell) Night sweats Weight loss Fever Itching

The difference between Hodjkins and Non Hodjkins? what cells presentation age

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

What is the treatment for lymphoma

- 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

Whats a neuroblastoma?

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.

What are the signs and symptoms of neuroblastoma?

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

Investigations of neuroblastoma?

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

Treatment of neuroblastoma?

Treatment of neuroblastoma - Surgery if early - Chemotherapy/stem cell transplantation if late

Malignancy Flashcards

(36 cards)