Paediatric Malignant Disease

Question 1

What is a chondrosarcoma?

Answer 1

Malignant neoplasm of cartilage

Question 2

What are 3 embryonal tumours?

Answer 2

1) Rhabdomyosarcoma
2) neuroblastoma
3) Wilm’s tumour

Question 3

What are examples of children who are at increased risk of cancer?

Answer 3

1) Down’s: Leukaemia
2) Immunocomprised: Lymphoma

Question 4

What is an abnormal redflex a sign of?

Answer 4

Retinoblastoma

Question 5

What are 5 signs of ALL?

Answer 5

1) Fever
2) Fatigue
3) Frequent infections
4) Bruising
5) Bone pain: Don’t want to weight bear
6) Anaemia
7) Lymphadenopathy/Hepatosplenomegaly

Question 6

What investigations could be done to a child with ALL?

Answer 6

1) Blood film
2) BM aspirate
3) CXR
4) Serum chemistry
5) LP

Question 7

What is the main treatment for ALL?

Answer 7

5 phase Chemotherapy –> Stem cell transplant may be needed for high risk/relapsed (2 years girls, 3 years boys)
1) induction 2) consolidation 3) interim maintenance 4) intensification 5) maintenance

Question 8

What are the 5 signs of CNS malignancy in children?

Answer 8

1) Early morning headache
2) headache worse on lying down
3) Vomiting
4) papilloedema
5) squint
6) nystagmus
7) ataxia
8) personality/behavioural change

Question 9

What is the general treatment for CNS malignancy?

Answer 9

1) Surgical resection & VP shunt (reduce risk of coning)
2) Chemotherapy (Less as chance as less drugs that can penetrate BBB)
3) Radiotherapy

Question 10

What are the main signs that lymphadenopathy could be due to malignancy rather than benign cause?

Answer 10

1) Enlarged node –> Infective cause
2) persistently enlarged
3) Unusual site (Supra-clavicular)
4) B symptoms
5) Abnormal CXR

Question 11

What is the treatment for lymphoma?

Answer 11

Combination chemotherapy: High dose therapy and stem cell transplantation offered to those who have relapsed

Question 12

What are the main differentials for abdominal mass in children?

Answer 12

1) Hepatoblastoma
2) Wilm’s tumour
3) neuroblastoma
4) lymphoma/leukaemia
5) Constipation/bowel obstruction
6) enlarged polycystic kidneys

Question 13

What investigations would you do on an abdominal mass?

Answer 13

1) USS
2) Biopsy
3) CT

Question 14

Define a neuroblastoma

Answer 14

Tumours arising from neural crest tissue in adrenal medulla and sympathetic NS

Question 15

What are 2 signs of a neuroblastoma tumour?

Answer 15

1) Abdominal mass (Crosses midline and envelopes major vessels and lymph nodes)
2) Symptoms of metastases e.g. bone pain, weight loss, pallor, limp, hepatomegaly

Question 16

What is the treatment for neuroblastoma malignancy?

Answer 16

1) Surgery: Localised primaries often cured by surgery
2) Radiotherapy: High risk groups
3) Chemo: Given before/after surgery to control disease

Question 17

Define Wilm’s tumour

Answer 17

Nephroblastoma: Malignancy from embryonal renal tissue
Signs: Abdominal mass, Haematuria, Abdominal pain, Anorexia, Anaemia
Treatment: Chemo, Nephrectomy and RT for higher stage

Question 18

Retinoblastoma: Aetiology, Signs and treatment

Answer 18

Aetiology: Sporadic, Familial (AD), Mutations in RB1 (TSG) on Cr13
Signs: Loss of red reflex, pain around eye and poor vision
Treatment: Laser therapy 1st line! (Chemo/RT, Photo Therapy, Surgical repair/removal)

Question 19

What are the main late effects of cancer treatment in children?

Answer 19

1) Endocrine related e.g. growth and development problems
2) Intellectual
3) Fertility problems
4) Psychological issues
5) Cardiac and Renal toxicity