Haem/Onc

Question 1

5 tumours in Pre-school aged kids?

Answer 1

ALL
Non-hodgkin Lymphoma
Neuroblastoma
Wilm tumour
Retinoblastoma

Question 2

5 tumours in school aged kids?

Answer 2

ALL

Brian tumour

Question 3

4 tumours in Adolescent?

Answer 3

ALL
Hodgkin lymphoma
Malignant Bone tumours
Soft tissue carcinoma

Question 4

White pupillary reflex or squint

Answer 4

Retinoblastoma

Question 5

Large abdominal mass in a well kid - occasionally anorexia, abdominal pain, haematuria

Answer 5

Wilm’s tumour

Question 6

Seborrhoeic rash,
Widespread tissue infiltration,
Bone pain/swelling or fracture
Diabetes insipidus

Answer 6

Langerhans Cell histiocytosis

Question 7

Neuroblastoma arises from the neural crest cells and hence most commonly affects which organ?

Answer 7

Adrenal glands

Question 8

Abdominal mass that crosses the midline
Spinal cord compression - Bone pain?
Weight loss, malase
pallor bruising

Answer 8

Neuroblastoma

Question 9

Malaise, anorexia,
Pallor, lethargy
infections
Bruising, petechiae, nose bleeds
Lymphadenopathy, Hepatosplenomegaly

Answer 9

ALL

Question 10

Classicly painless firm lymphadenopathy in the neck which may or not cause airway obstruction.
With B-symptoms - Sweating, pruritus, weight loss and fever

Answer 10

Hodgkin’s lymphoma

Question 11

Most common primary renal neoplasm of childhood - diagnosed between age 2 and 5

Answer 11

Wilm’s tumour

Question 12

WAGR syndrome:
\_\_\_\_\_\_\_
Aniridia (absence of coloured part of the iris)
Genital anomalies
Mental Retardation

Answer 12

Wilm’s tumour

Question 13

Wilm’s tumour is related to another syndrome which consists features of :
Enlargement of body organs - especially TONGUE, hemihypertrophy, renal medullary cyst and adrenal cytomegaly

Answer 13

Beckwith-weidmann syndrome

Question 14

Denys-Drash syndrome consists of gonadal dysgenesis and nephropathy leading to renal failure.
This happens commonly with which tumour?

Answer 14

Wilm’s tumour

Question 15

What is the most common form of soft tissue sarcoma in childhood?

Answer 15

Rhabdomyosarcoma

Question 16

Tumour causing proptosis, nasal obstruction or blood-stained nasal discharge.
Can have Genitourinary symptoms too

Answer 16

Rhabdomyosarcoma

Question 17

______sarcoma is more common than ______ Sarcoma, and ______ sarcoma is seen more often in younger children.

Answer 17

Osteogenic sarcoma is more common than Ewing Sarcoma, but Ewing sarcoma is seen more often in younger children.

They generally tend to happen after puberty

Question 18

___________ in nearly all cases of hepatoblastoma and in some cases of hepatocellular carcinoma

Answer 18

Elevated serum alpha-fetoprotein is detected (aFP)

Question 19

Germ cell tumours arise from primitive germ cells which migrate from the yolk sac endoderm to form gonads in the embryo. They occur most commonly in which region?

Answer 19

Sacrococcygeal region

Question 20

In Germ cell tumours: what are two serum markers vital for diagnosis and monitoring?

Answer 20

aFP and Beta-hCG

Question 21

_________ is a rare disorder characterised by an abnormal proliferation of histiocytes

Answer 21

Langerhans cell histiocytosis

Question 22

Two common associations of Langerhans Cell Histiocytosis?

Answer 22

• Bone lesions - present at any age with pain, swelling or fracture - well defined lytic lesions
• Diabetes insipidus - may be associated with skull disease with proptosis and hypothalamic infiltration

Question 23

60% of all paediatric brain tumours are _______

Answer 23

infratentorial

Question 24

Brain tumour:
Usually in posterior fossa
Well circumscribed
Benign, good prognosis

Answer 24

Pilocytic astrocytoma

Question 25

14 year old with aggressive behaviours at school – headaches and seizure. - Cancer that tends to affect the cortex of the brain and presents with focal neurological signs

Answer 25

Astrocytoma - Glioblastoma Multiforme

Question 26

10yo with headaches, vomiting, poor growth, struggling to see board at school. Can also have Visual loss (bitemporal hemianopia), pituitary failure – growth failure, diabetes insipidus, weight gain

Answer 26

Craniopharyngioma

Question 27

3 year old vomiting in the mornings, unsteady on his feet, new onset convergent squint Presents with truncal ataxia, co-ordination difficulties, abnormal eye movements

Answer 27

Medulloblastoma - affecting

Question 28

Where do medulloblastoma's primarily occur?

Answer 28

Cerebellar and 4th ventricle

Question 29

4 yr old – refuses to walk, unable to climb stairs, squint, facial asymmetry and drooling Brainstem defect: Cranial nerve defects, pyramidal tract signs, cerebellar ataxia, Often no raised intracranial pressure

Answer 29

Brainstem Glioma

Question 30

A primitive neuroectodermal tumour (PNET) Arises in the midline of the posterior fossa. Compresses ventricles and leads to hydrocephalus. Highly malignant and seeds through CNS via CSF,

Answer 30

Medulloblastoma

Question 31

In 4th ventricle causing hydrocephalus Poor prognosis Mostly in the posterior fossa where it behaves like a medulloblastoma

Answer 31

Ependymoma

Question 32

Often cerebellar Associated with von Hippel-Lindau syndrome with retinal angiomas Can produce EPO secondary polycythaemia

Answer 32

Hemangioblastoma

Question 33

Most common supratentorial childhood tumour

Answer 33

Craniopharyngioma

Question 34

Craniopharyngioma is benign and arises from the squamous remnant of ___________

Answer 34

The Rathke pouch

Question 35

Cancer that can originate from any site in sympathetic nervous system, presenting as a mass in neck, chest or abdomen mass (most common site is adrenal gland)

Answer 35

Neuroblastoma

Question 36

Cancer that can cause: Can also present with peri-orbital ecchymosis, abdominal pain, emesis, fever, weight loss, anorexia, “blueberry muffin” skin nodules.

Answer 36

Neuroblastoma

Question 37

Sturge weber syndrome is associated with _________ syndrome in V1 distribution

Answer 37

Sturge Weber syndrome

Question 38

o Adenoma sebaceum – angiokeratomas on face, often in malar distribution o Shrageen patch – isolated raised plaque over lower back, buttocks o Ash leaf – hypopigmentation seen with Wood’s lamp (UV light)

Answer 38

Tuberous Sclerosis