What is the most common malignancy in children
Acute Lymphoblastic Leukaemia (ALL)
What is ALL
Malignancy of the Lymphoid Cells (peak age 4-7):
- Affects B and T lymphocyte cell lines - arresting maturation and prompting uncontrolled proliferation of immature blast cells of the lymphoid line (multiplying out of control)
- This leads to marrow failure and tissue infiltration
What is AML
Malignancy of the Granulocytes (neutro, baso, esinophils)
- Arrests maturation and prompting uncontrolled proliferation of immature blast cells of the myeloid line (multiplying out of control)
- This leads to marrow failure and tissue infiltration
What do the acute luekaemias lead to and what does this result in
Bone Marrow Failure and Tissue Infiltration:
- Thrombocytopenia (bleeding)
- Anaemia
- Neutropenia (infection)
What are the signs and symptoms of Luekaemia
Sudden onset of symptoms and rapid progression (days to weeks)
- Fever
- Fatigue
- Frequent infections
- Lymphadenopathy
- Hepatomegaly and/or splenomegaly
- Anaemia
- Bruising and petechiae
- Bone or joint pain
- Weight Loss and Dyspnoea
CNS involvement rare: headache, N&V, irritability, papillodoema, cranial nerve involvement (3rd,4th,6th,7th)
Intracranial/spinal mass may present as nerve compression
Testicular involvement rare - but often presents as painless testicular cancer
What are the investigations of Leukaemia
- CXR: mediastinal mass
- Testicular Examination - US if enlarged
- FBC: Anaemia, Neutropenia, Thrombocytopenia, blast cells
- Blood Film - presence of blast cells!!!
- Bone Marrow aspiration and biopsy diagnostic:
ALL: >20% lymphoblasts
AML: >20% myoblasts and Auer Rods - Lumber Puncture: to assess CNS involvement
What chromosomal abnormality is seen in ALL and what does it predict
Philadelphia Chromosome - poor prognosis
What is the treatment for ALL
Support
Immediate IV Abx if infection due to neutropenia
Chemotherapy - 4 phases
- Induction
- Consolidation +/- CNS treatment
- Intensification
- Maintenance
Stem Cell Transplant - poor prognostic factors, high risk patients, relapsed patients
What is the treatment for AML
Support
Immediate IV Abx if infection
Intensive Chemotherapy
Stem Cell Transplant - poor prognostic factors, high risk patients, relapsed patients
What is the prognosis for ALL
80% cure
Good prognostic factors: Age 2-10, female, WCC<50 and no CNS involvement
How do CNS tumours present
- Headache: often worse early morning/lying down
- Vomiting: especially early morning
- Papilloedema
- Squint
- Nystagmus
- Ataxia
- Personality or behaviour change
What are 3 types of CNS tumours found in children
Pilocytic Astrocytoma
Pontine Glioma
Medulloblastoma
What is the treatment for CNS tumours
Surgery
- Resection
- VP shunt
Chemotherapy (most are ineffective as don’t cross blood/brain barrier)
- Single agent
- Combination treatment
Radiotherapy
- For malignant tumours in older children
- Whole brain not used in very young can damage developing nervous system
What is the most common cause of Lymphodenopathy
Very common in childhood (up to 50%)
- Mostly due to self limiting benign cause due to bacterial/viral infection
Other causes
- HIV infection
- Auto immune conditions
- Storage disorders
- Malignancy
When should you biopsy lymphodenopathy
- Enlarging node without clear infective cause
- Persistently enlarged node
- Unusual site e.g. supraclavicular
- If have associated symptoms and signs
- Fever, weight loss, enlarged liver/spleen
- If CXR abnormal
How do you treat paediatric Lymphoma
Chemotherapy
- Combination Chemo to treat NHL and HL
- Determined by histology and stage
Radiotherapy
- Hodgkin’s – to residual bulk disease
- rarely used in NHL
Surgery – mainly limited to biopsy
High dose chemotherapy with stem cell transplant mainly for relapse
How may a child with a malignant abdominal mass present
Abdominal mass Pain Haematuria Constipation Hypertension Weight Loss
What are the differentials for an abdominal mass in a child
Hepatoblastoma Wilms tumour Neuroblastoma Lymphoma/leukaemia Sarcoma Constipation Enlarged kidneys – polycystic disease
What are useful lx for a abdominal mass
US
CT
Biopsy
What may be present on Ex and Ix of a Neuroblastoma
- Child with proptosis, as result of orbital involvement from metastatic neuroblastoma
- CT scans of head show orbital mass
- CT scan of abdomen shows large mass crossing midline and encasing the aorta and IVC
What may present on Ex and Ix of a Wilms Tumour
- Child with distended abdomen, otherwise well. Known to have hemi-hypertrophy (risk for developing Wilms)
- Painless, palpable abdo mass
- CT scan of abdomen shows large tumour within kidney
- CT scan of chest shows pulmonary metastases
What is the treatment for Neuroblastoma
Surgery
- Primary - if resectable (mild disease no further treatment)
- Chemotherapy may be given before or after to control disease if intermediate risk
Chemotherapy
Type determined by stage and biology
High dose with HPSC (haemopoietic progenitor stem cell transplant) - high risk groups
May be given if resection not possible
Radiotherapy
Mainly for high risk group or at relapse
What is the most common type of renal tumour in children
Wilms Nephroblastoma
What Increases the risk of Wilms Tumour
Both two do with abnormalites on chromosome 11:
- Beckwith - Weidmann Syndome
- WAGR - Wilms tumor, anirida, genitourinary anomalies and retardation
