Paeds Oncology

Question 1

Paediatrics

Epimeiology Leukaemia

Which one is the most common in Children

Answer 1

Acute Lymphoblastic Leukaemia (ALL) accounts for 80% Ages 2-5

Acute Myeloid Leukaemia under 2

Slightly more common in Boys

Question 2

RFs for Leukaemia

Answer 2

Down’s Syndrome, Klinefelter Syndrome, Noonan Syndrome & Fanconi’s Anaemia

Question 3

Pathophysiology Leukaemia

Malignant condition of stem cells in the bone marrow

Answer 3

• Genetic Mutation in precursor cells in bone marrow
• Excess production of WBC
• This causes supression of other cell lines leading to underproduction

Question 4

Leukaemia

Signs & Symptoms ALL

onset over several weeks

Answer 4

Gen Symptoms: Night sweats, malaise, anorexia
Bone marrow infiltration: Anaemia, Infection, bruising, bone pain.

FEVER PRESENT 50% Cases

Other organ infiltration: headaches, vomitting, nerve palsies

Question 5

Leukaemia

Types of ALL

Answer 5

• Common ALL (75%), CD10 present, pre-B phenotype
• T-cell ALL (20%)
• B-cell ALL (5%)

Question 6

The poor prognostic factors of ALL

Overall cure rate is 80%

Chemo side fx: Stunted growth, cardiotoxicity, infertility etc.

Answer 6

• Age < 2 years or > 10 years
• WBC > 20 * 109/l at diagnosis
• T or B cell surface markers
• non-Caucasian
• male sex

Question 7

Investigations Leukaemia

Answer 7

• FBC: Low Hb, thrombocytopenia, neutropenia, evidence of Leukemic blast cells.
• CT Scan for staging
• Bone marrow examination for diagnosis

Question 8

Management Leukaemia

Answer 8

Chemotherapy
Others: Radiotherapy, Bone Marrow transplant surgery

Question 9

Malignant disease of the CNS

Brain Tumour Signs & Symptoms

Leading cause of cancer death in children

Almost always Primary, MRI to diagnose

Answer 9

• Evidence of raised ICP
• Focal Neurological signs
• Papilloedema (late sign)
• Spinal tumours: Back pain, weakness in legs, bladder or bowels.

Treatment: Surgery to treat hydrocephalous

Most common types: Astrocytoma, Meduloblastoma

Question 10

Peak age 3Y

What is Wilm’s Tumour?

very rare after age 10Y

Answer 10

• Most common Renal tumour of childhood - most common U5
• Malignant Disease with tumour originating from embroynal renal tissue.

Question 11

Signs and Symptoms in Wilm’s Tumour

Most often child is well

Most common site of metastasis: Lungs

Answer 11

• Large Abdo mass (often unilateral)
• Haematuria is also common
• Anorexia, Fever, Lethargy
• Flank pain

Can also develop High BP and Anaemia

Complications: 90% cure rate, prognosis is poor in relapse, metastatic disease also poor cure rate in comparison

Question 12

Associations of Wilm’s Tumour

Genetic Markers etc.

Answer 12

• Beckwith-Wideman Syndrome
• WAGR syndrome
• Hemihypertrophy
• WT1 gene - Chromosome 11

Question 13

Management of Wilm’s Tumour

Answer 13

Chemotherapy followed by delayed nephrectomy

Question 14

7% of Childhood malignancies

What is Neuroblastoma?

Answer 14

• Malignant embyonal tumour derived from neural crest tissue in Adrenal medulla and Sympathetic NS
• Common in 2Y old

Seen in: Adrenal Glands, Sympathetic Chain

Question 15

Signs and Symptoms of Neuroblastoma?

Answer 15

• Pallor
• Weight loss
• Abdominal Mass
• Hepatomegaly

Paraplegia, Lymphadenopathy, Proptosis, Compression of nerves

Proptosis: Bulging of eye
Paraplegia: impairment in functions of lower limbs

Question 16

Investigations of Neuroblastoma?

Answer 16

Raised Urinary Catecholamine Metabolite levels
* VMA - vanillylmandelic acid
* HVA - homovanillic acid

Detection using Biopsy Bone Marrow & MIBG scan

Question 17

Management Neuroblastoma

High risk of relapse

Answer 17

• Surgery in non-metastasised cancer
• Chemo otherwise

Poor prognosis: over 18m old, Raised Ferritin & LDH

Question 18

What is Retinoblastoma?

Average age 18m Old

Answer 18

• Malignant tumour of Retinal Cells (20% unilateral)
• Autosomal Dominant

Retinoblastoma tumour suppressor gene on chromosome 13

Question 19

Signs of Retinoblastoma

Answer 19

• Red pupilliary reflex replaced by white pupillary reflex
• Strabismus
• Visual Deterioration

Strabismus: Squint

Question 20

Management Retinoblastoma

Answer 20

• Enucleation is a option
• Other options include external beam radiation therapy, chemotherapy and photocoagulation

Enucleation: Removal of eye ball

Question 21

Two Types of Bone Tumour

Answer 21

1. Osteosarcoma (15-30Y)
2. Ewing Sarcoma (seen in younger children)

Both have male predominance

Ewing: Location by femoral diaphysis is commonest site

Question 22

What Features of a mass raise suspicion of sarcoma?

Answer 22

• Tissue mass >5cm
• Deep location or Intramuscular
• Rapid growth
• Painful lump

Question 23

Bone Cancer

Differentiating between Osteosarcoma and Ewing Sarcoma

Histologically & Treatment

Answer 23

Osteosarcoma: Mesenchymal cells with osteoblastic differentiation treated with limb preserving surgery and chemo.
Ewing: small round tumour. Chemo with Surgery and sometimes Radiotherapy

Limb amputation is a big possibility

Question 24

Signs and Symptoms of Bone Cancer

Ewing and Osteosarcoma

Answer 24

• Persistent localised bone pain
• Preceding the detection of a mass

Question 25

Investigations of Bone Cancer

Answer 25

• X ray
• Followed by MRI and Bone scan

Ewing: substantial soft tissue mass

Question 26

Hepatoblastoma

common age 2-3Y

Presents with Abdo distention w/ mass often painless and no jaundice

Answer 26

• Primart malignant liver tumour.
• Raised serum alpha-fetoprotein
• Chemo used aswell as surgery, liver tranplant required if resection not possible.