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Flashcards in Solid tumours Deck (14)
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1
Q

What is the most common solid tumours after brain tumours?

A

Neuroblastoma

  • 8% of childhood tumours
  • most frequently diagnosed cancer in infants
  • median age 2 years
2
Q

Where does neuroblastoma arise from?

A

Neural crest primitive sympathetic nervous system

  • adrenal medulla (40% of children, 25% of infants)
  • retroperitoneal sympathetic chain (30%)

Infants tend to present with localised disease in cervical / thoracic region

Child with abdominal neuroblastoma tend to present with disseminated disease

3
Q

What are conditions a/w neuroblastomas?

A

Beckwith-Wiedeman (but usually with wilms)
Hirschsprung’s
NF1

4
Q

What are the genetic markers of prognostic significance in neuroblastoma?

A
N-myc (proto-oncogene) amplicfication on chr 2 
- 25% of neuroblastoma 
- present in 30% of advanced disease 
shorter time to relapse 
poor prognosis 

Triploidy / hyperploidy = good outcome

Chr 1 p deletion
- most common - poor out common

5
Q

What are some specific clinical features of neuroblastoma presentation?

A

Abdominal mass
- crosses midline

Cervical
- horner syndrome

HTN (catecholamines) 
secretory diarrhoea (VIP / somatostatin secretion) 

Paraneoplastic phenomenon

  • opsoclonus-myoclonus (chaotic eye movement, truncal ataxia, myoclonus)
  • dancing feet
6
Q

What investigations are required for neuroblastoma work up?

A

primary
- CT / MRI +/- biopsy

metastases

  • BM aspirate (bilateral)
  • bone scan
  • abdo CT / MRI / USS
  • Chest x-ray / CT

markers

  • increased urinary VMA (end product of dopamine)
  • increased urinary HVA (end product of adrenaline / NA)
7
Q

What treatment is used for high risk neuroblastoma?

A

high dose chemo with stem cell rescue

Maintenance retinoic acid improves survival rate from 20-50%

8
Q

What is Wilm’s tumour?

A

Nephroblastoma
- think well toddler with a big belly

One of the best prognostic tumours in children with 90% expecting cure

Makes up 5% of childhood cancers

9
Q

What are conditions a/w Wilm’s tumour?

A

Non-overgrowth (aWT1 gene)

  • aniridia
  • trisomy 18
  • Urogenital malformations: hypospadias, cryptorchidism, renal fusion
  • Denys-drash (ambiguous genitalia, renal impairment in 1st year due to mesangial nephrotpathy, wilms by 2nd year)
  • WAGR syndrome (stands for Wilm’s, aniridia, genitourinary abN, mental retardation)

Overgrowth syndromes (aWT2 gene)

  • Beckwith Weidemann
  • Sotos’
  • Isolated hemihypertrophy
10
Q

What is the significance of WT1 (chr 11p13) in Wilms?

A

encodes a zinc finger transcription factor critical for normal kidney development

  • a/w aniridia and genitourinary malformations
  • only present in 5-10% of wilms
11
Q

What is the significance of WT2 (chr11p15) in Wilms?

A

a/w overgrowth syndromes

neighbouring gene in same locus as BWS

12
Q

What is the significance of p53 in wilms?

A

poor prognostic factor

- present in 85% of anaplastic wilms

13
Q

What are prognostic factors of importance in Wilms?

A

Tumour size (>500g is worse)
Stage
Histology

14
Q

What clinical presentation would you expect from Wilms’?

A

Abdominal mass in otherwise healthy child
HTN (25%)
Macroscopic haematuria (20%)
Pain / fever / vomiting / anorexia (rare)
Signs of venous obstruction (tumour thrombosis / IVC occlusion)
Risk of rupture (limit palpation as spillage –> upstages to stage III requiring more aggressive therapy

10-15% have mets at diagnosis
- lymph nodes, veins, lung (80%), liver

Check coagulation!
- acquired von Willebrand syndrome in 5-10% can occur due to hydralonic acid