Paeds Oncology Flashcards
Paediatrics
Epimeiology Leukaemia
Which one is the most common in Children
Acute Lymphoblastic Leukaemia (ALL) accounts for 80% Ages 2-5
Acute Myeloid Leukaemia under 2
Slightly more common in Boys
RFs for Leukaemia
Down’s Syndrome, Klinefelter Syndrome, Noonan Syndrome & Fanconi’s Anaemia
Pathophysiology Leukaemia
Malignant condition of stem cells in the bone marrow
- Genetic Mutation in precursor cells in bone marrow
- Excess production of WBC
- This causes supression of other cell lines leading to underproduction
Leukaemia
Signs & Symptoms ALL
onset over several weeks
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
Leukaemia
Types of ALL
- Common ALL (75%), CD10 present, pre-B phenotype
- T-cell ALL (20%)
- B-cell ALL (5%)
The poor prognostic factors of ALL
Overall cure rate is 80%
Chemo side fx: Stunted growth, cardiotoxicity, infertility etc.
- Age < 2 years or > 10 years
- WBC > 20 * 109/l at diagnosis
- T or B cell surface markers
- non-Caucasian
- male sex
Investigations Leukaemia
- FBC: Low Hb, thrombocytopenia, neutropenia, evidence of Leukemic blast cells.
- CT Scan for staging
- Bone marrow examination for diagnosis
Management Leukaemia
Chemotherapy
Others: Radiotherapy, Bone Marrow transplant surgery
Malignant disease of the CNS
Brain Tumour Signs & Symptoms
Leading cause of cancer death in children
Almost always Primary, MRI to diagnose
- 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
Peak age 3Y
What is Wilm’s Tumour?
very rare after age 10Y
- Most common Renal tumour of childhood - most common U5
- Malignant Disease with tumour originating from embroynal renal tissue.
Signs and Symptoms in Wilm’s Tumour
Most often child is well
Most common site of metastasis: Lungs
- 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
Associations of Wilm’s Tumour
Genetic Markers etc.
- Beckwith-Wideman Syndrome
- WAGR syndrome
- Hemihypertrophy
- WT1 gene - Chromosome 11
Management of Wilm’s Tumour
Chemotherapy followed by delayed nephrectomy
7% of Childhood malignancies
What is Neuroblastoma?
- Malignant embyonal tumour derived from neural crest tissue in Adrenal medulla and Sympathetic NS
- Common in 2Y old
Seen in: Adrenal Glands, Sympathetic Chain
Signs and Symptoms of Neuroblastoma?
- Pallor
- Weight loss
- Abdominal Mass
- Hepatomegaly
Paraplegia, Lymphadenopathy, Proptosis, Compression of nerves
Proptosis: Bulging of eye
Paraplegia: impairment in functions of lower limbs
Investigations of Neuroblastoma?
Raised Urinary Catecholamine Metabolite levels
* VMA - vanillylmandelic acid
* HVA - homovanillic acid
Detection using Biopsy Bone Marrow & MIBG scan
Management Neuroblastoma
High risk of relapse
- Surgery in non-metastasised cancer
- Chemo otherwise
Poor prognosis: over 18m old, Raised Ferritin & LDH
What is Retinoblastoma?
Average age 18m Old
- Malignant tumour of Retinal Cells (20% unilateral)
- Autosomal Dominant
Retinoblastoma tumour suppressor gene on chromosome 13
Signs of Retinoblastoma
- Red pupilliary reflex replaced by white pupillary reflex
- Strabismus
- Visual Deterioration
Strabismus: Squint
Management Retinoblastoma
- Enucleation is a option
- Other options include external beam radiation therapy, chemotherapy and photocoagulation
Enucleation: Removal of eye ball
Two Types of Bone Tumour
- Osteosarcoma (15-30Y)
- Ewing Sarcoma (seen in younger children)
Both have male predominance
Ewing: Location by femoral diaphysis is commonest site
What Features of a mass raise suspicion of sarcoma?
- Tissue mass >5cm
- Deep location or Intramuscular
- Rapid growth
- Painful lump
Bone Cancer
Differentiating between Osteosarcoma and Ewing Sarcoma
Histologically & Treatment
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
Signs and Symptoms of Bone Cancer
Ewing and Osteosarcoma
- Persistent localised bone pain
- Preceding the detection of a mass
Investigations of Bone Cancer
- X ray
- Followed by MRI and Bone scan
Ewing: substantial soft tissue mass
Hepatoblastoma
common age 2-3Y
Presents with Abdo distention w/ mass often painless and no jaundice
- Primart malignant liver tumour.
- Raised serum alpha-fetoprotein
- Chemo used aswell as surgery, liver tranplant required if resection not possible.
