Malignancy

Question 1

Where are brain tumours usually in children and what’s their origin?

Answer 1

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

Question 2

What are some common types of brain tumours

Answer 2

Astrocytoma (40%) - benign or malignant
Medullablastoma (20%)
Ependymyoma (8%)
Craniopharyngoma

Question 3

What are some symptoms of brain tumours in children?

Answer 3

Either general signs of raised ICP or specific focal neurological signs

Question 4

How should brain tumours be investigated?

Answer 4

Bets visualised on MRI scan

Question 5

How should brain tumours be managed?

Answer 5

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

Question 6

What is a Wilm’s tumour?

Answer 6

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

Question 7

How does Wilm’s tumour present?

Answer 7

Most usually with large abdominal masses that are found incidentally - ASYMPTOMATIC
Symptoms may include abdominal pain, anorexia, anaemia, haemturia and hypertension

Question 8

How should a Wilm’s tumour be investigated?

Answer 8

MRI, USS and CT are all useful

LOOK IN LUNG FOR METS

Question 9

How are Wilm’s tumours managed?

Answer 9

There is usually initial chemotherapy and then later nephrectomy
Radiotherpay is sometimes offered to patients with very advanced disease

Question 10

What is the prognosis for Wilm’s tumour?

Answer 10

GOOD - approx 80%

Question 11

What are the most common forms of malignancies in children? And what is the most common specific malignancy?

Answer 11

HAEMATOLOGICAL MALIGNANCY

Specifically LEUKAEMIA IS THE MOST COMMON

Question 12

What is the most common form of leukaemia in kids?

Answer 12

Acute lymphoblastic leukaemia

a fairly large chunk also AMLs

Question 13

What is the peak of incidence of leukaemia?

Why do symptoms occur and how quickly do they occur?

Answer 13

Peak inc: 2-5 years
Symptoms occur due to disseminated disease and the infiltration of malignant cells into tissues
Usually they come on over a course of a few weeks but can be much more rapid

Question 14

What are some symptoms of ALL in children?

Answer 14

GENERAL: malaise and anorexia
BONE MARROW: Neutropenia (infection), Anaemia (pallor and lethargy) and thrombocytopenia (nose bleeds, bruising)
RETICULO-ENDOTHELIAL: Hepatosplenomegaly and lymphadenopathy
OTHER: CNS (headaches, vomiting and nerve palsies), TESTES (testicular enlargement)

Question 15

What investigations should be done for ALL?

Answer 15

FBC - might show anaemia and thrombocytopenia and circulating leukaemia cells
BONE MARROW
CXR

Question 16

When would changes be seen on CXR?

Answer 16

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)

Question 17

How is ALL managed initially?

Answer 17

Correct anaemia (possibly with transfusion)
Give hydration therapy
Give allopurinol as a renal protectant against rapid cell lysis
Then after 4 weeks of this start chemotherapy

Question 18

What is the chemotherapy treatment schema for ALL?

Answer 18

INDUCTION with vincristine and steroid (dex)
CNS PROTECTANT AND CONSOLIDATION (IT methotrexate, vincristine, steroid, thiopurine)
INTERIM - weekly vincristine, 5 daily steroid and daily 6-mercaptopurine
DELAYED INTENSIFICATION - Vincristine, dex, doxirubicin, L-Asparginase, IT methotrexate

Question 19

What are some poor prognostic factors for ALL?

Answer 19

If child aged under 1 or above 10
If WCC is >5x10^( (high tumour load)
If there are cytogenic abnormalities t(4;11)
If there is slow initial response to therapy
Minimal residual disease assessment

Question 20

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

Answer 20

NHL - no Reed-Sternberg Cells

Question 21

How does lymphoma usually present?

Answer 21

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

Question 22

How can you tell between benign lymphadenopathy and lymphoma?

Answer 22

Lymphadenopathy in lymphoma is larger and a lot firmer

Question 23

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

Answer 23

Lymph node biopsy and radiological assessment of all lymph node sites