Neuroblastoma

Question 2

What is the Somatic Nervous System responsible for?

Answer 2

Voluntary control

Question 3

What does the Autonomous Nervous System control?

Answer 3

Involuntary bodily functions

Question 4

What response is associated with the Sympathetic nervous system?

Answer 4

Fight or flight response

Question 5

What is Neuroblastoma?

Answer 5

An embryonal malignancy of the sympathetic nervous system arising from neuroblasts

Question 6

What are the most common sites for Neuroblastoma?

Answer 6

• Adrenal medulla (35%)
• Paraspinal ganglia in abdomen or pelvis (25%)
• Thoracic region (15%)
• Head and Neck primary (5%)

Question 7

What are the three types of tumours related to Neuroblastoma?

Answer 7

• Ganglioneuroma – benign
• Ganglioneuroblastoma – intermediate
• Neuroblastoma – malignant

Question 8

What is the typical appearance of Neuroblastoma cells?

Answer 8

‘Small round blue cell’ composed of dense nests of hyperchromatic cells

Question 9

What is the most common extracranial pediatric solid tumour?

Answer 9

Neuroblastoma

Question 10

What is the male-to-female ratio in Neuroblastoma cases?

Answer 10

1.2 : 1

Question 11

Define an oncogene.

Answer 11

A gene that regulates the development and growth of cancer cells

Question 12

Which oncogene is associated with Neuroblastoma?

Answer 12

MYCN

Question 13

What is the significance of MYCN amplification in Neuroblastoma?

Answer 13

Associated with aggressive growth and poor prognosis

Question 14

What is Anaplastic Lymphoma Kinase (ALK)?

Answer 14

A gene related to neuroblastoma and associated with poorer survival

Question 15

What is Neurofibromatosis?

Answer 15

A genetically inherited condition where nerve tissue grows non-cancerous tumours

Question 16

List some parental factors studied related to Neuroblastoma.

Answer 16

• Smoking
• Alcohol abuse
• Illicit drugs
• Occupational chemicals

Question 17

What are typical symptoms of Neuroblastoma?

Answer 17

• Lethargy
• Abdominal mass
• Pain
• Loss of appetite
• Fever
• Diarrhea

Question 18

What can a very swollen abdomen in a Neuroblastoma patient cause?

Answer 18

Constipation and difficulty passing urine

Question 19

What tests help establish a diagnosis of Neuroblastoma?

Answer 19

• Tumour and lymph nodes pathology
• Blood tests
• Urine tests for VMA and HVA
• Imaging tests (X-ray, ultrasound, CT, MRI, PET-CT)

Question 20

What is the International Neuroblastoma Staging System (INSS)?

Answer 20

A standard based on clinical, radiographical, and surgical findings related to prognosis

Question 21

What defines Stage 1 in the INSS?

Answer 21

Localized or confined to area of origin

Question 22

What characterizes Stage 4S in Neuroblastoma?

Answer 22

Localized primary tumour in infants with limited spread to liver, skin, and/or bone marrow

Question 23

What treatment depends on for Neuroblastoma?

Answer 23

• Age of child
• Size and position of the tumour
• Spread
• N-Myc and ALK status

Question 24

What is the prognosis for low-risk Neuroblastoma?

Answer 24

5 year survival rate of 95%

Question 25

What is the prognosis for high-risk Neuroblastoma?

Answer 25

5 year survival rate of 40-50%

Question 26

What are common treatment modalities for high-risk Neuroblastoma?

Answer 26

• Induction chemotherapy
• Surgical resection
• Radiation therapy

Question 27

What is a common complication of radiation therapy in infants?

Answer 27

Significant late effects

Question 28

What is the recommended dose for tumor bed in neuroblastoma treatment?

Answer 28

2160 cGy in 12 fractions

Question 29

What is the role of VMAT in radiation treatment?

Answer 29

Used to limit doses to surrounding organs

Question 30

True or False: Neuroblastoma tumors often spontaneously regress.

Answer 30

True

Question 31

What is the significance of a bone marrow biopsy in Neuroblastoma?

Answer 31

Shows metastatic tumour deposits