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Paediatrics > Childhood cancers > Flashcards

Childhood cancers Flashcards

1
Q

What is hemi hyperplasia?

A

Condition where one side of the body grows quicker than the other, hyperplastic cells rather than hypertrophy. Can predispose child to nephroblastoma so child is screened every 3-6months

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2
Q

Child presents with easy bruising noticed over the last few weeks, also has bleeding from gums and appears pale

  • Examination shows she is pale and bruised over bony prominences
  • Abdo exam reveals hepatosplenomegaly
    • Concerning as if this was just infection the spleen tip might be palpable but nothing more
  • Petechial spots appear when blood pressure cuff is removed

New history of bruising in the family rules out a familial coagulation disorder

A

Investigations

  • FBC shows anemia, raised WCC, low platelets and blood film shows presence of lymphoblasts
    • Diagnostic of acute lymphoblastic leukemia
    • Child will be transferred to oncology unit for further tests such as bone marrow biopsy
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3
Q

Palpable abdominal malignancies?

A
  • Nephroblastoma, Wilm’s tumour, neuroblastoma
    • Nephroblastomas do not cross the midline
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4
Q
A
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5
Q

Most common type of childhood cancer?

A

Leukaemia

  • 25% childhood cancers worldwide
  • In leukemia there is arrest at an early stage of haemopoietic cell development and therefore early lymphoid precursors aka blasts proliferate and replace normal bone marrow cells which reduces the production of normal blood cells
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6
Q

Most common type of leukaemia diagnosed in children?

A

Acute lymphoblastic leukaemia

Caused by abnormal proliferation of immature lymphocytes – usually B cells – called blasts

  • Much more common in children than adults – 55% patients are children and peak incidence is at 2-3 years and declines thereafter
  • Children with certain genetic conditions are at higher risk
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7
Q

Clinical features of acute lymphoblastic leukaemia

A
  • Early symptoms often non-specific
    • Early bruising, easy bleeding e.g. when brushing teeth, lethargy
    • Symptoms occur due to the crowding out of other blood components e.g. thrombocytopenia occurs which accounts for the easy bruising and bleeding gums, anaemia occurs which accounts for the lethargy
  • Bone pain may manifest as limp
  • Hepatosplenomegaly
  • Headaches, cranial nerve abnormalities and neck stiffness may indicate CNS involvement
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8
Q

Diagnosis acute lymphoblastic leukaemia

A
  • Suspected initially when FBC shows thrombocytopenia, anaemia and raised WCC (not WCC can also be normal or low)
  • Lymphoblasts seen on blood film
  • Bone marrow biopsy showing blasts confirms diagnosis and lumbar puncture indicated whether cells have entered CNS
    • Note – intrathecal chemo is given even if lumbar puncture does not indicate CNS involvement as lumbar puncture itself can facilitate entry of leukemic cells into CNS
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9
Q

Management of acute lymphoblastic leukaemia

A

ALL is treated with chemo with several phases

Induction phase: achieves bone marrow remission I.e. normal blood count levels and no lymphoblast visible on blood film – at this stage there may still be residual disease

Consolidation phase: lasts several months, aim is to eliminate all leukaemic cells

Maintenance phase: lasts up to 3 years, aim is to prevent recurrence after remission

Management of CNS spread used to be managed with cranial irradiation but this has been replaced with intra thecal chemo because there is a high risk of neurotoxicity and brain tumours

  • Radiation is used if there is a very high lymphoblast count or any clinical signs of CNS involvement e.g. facial palsy

Relapse occurs in 20%, some will go into remission again but some will need a stem cell transplant

Chemo is given by central line because it is an irritant so a child will have a Hickman line inserted, also allows for blood to be taken from this site so child doesn’t have to go through frequent venepuncture

  • Prognosis
    • Good prognosis, 80-90% 5yr survival
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10
Q

Acute myeloid leukaemia

A
  • Results from abnormal proliferation of myeloblasts
  • Abnormal cells leak into peripheral blood and infiltrate organs
  • Second most common leukemia diagnosed in children – 15% cases
  • Incidence highest in children <1year
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11
Q
  • Similar to those seen in ALL
  • Bone pain and lymphadenopathy are less common than in ALL but organomegaly and haemorrhage are more common
  • Formation of mediastinal masses due to infiltration of body tissues
    • Can lead to resp distress or superior vena cava syndrome: partial occlusion of the SVC
      • Most common presenting symptoms of SVC syndrome are face/neck swelling, distended neck veins, cough, dyspnea, orthopnea, upper extremity swelling, distended chest vein collaterals, and conjunctival suffusion
  • Formation of abdominal masses can also occur, causing pain/ obstruction of GI or uro tract
A
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12
Q

Diagnosis of acute myeloid leukaemia

A
  • FBC will show normocytic anaemia with lower than normal reticulocyte count and thrombocytopenia, neutrophil count remains low
  • Myeloblasts seen in peripheral blood film + auer rods
  • Diagnosis confirmed with bone marrow biopsy
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13
Q

Management of acute myeloid leukaemia

A
  • AML treated with intensive induction chemo followed by consolidation chemo and bone marrow transplant if a match is available

Lower 5yr survival rate than ALL @60%

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14
Q

Chronic myeloid leukaemia

A
  • Caused by unregulated production of myeloid cells in bone marrow leading to excess of granulocytes – usually neutrophils
  • Rare in children
  • Starts with chronic phase where bloods are abnormal but child asymptomatic
  • When symptoms appear they are usually non-specific
  • Followed by an accelerated phase in which there is an increased number of myeloblasts in the blood
  • FBC shows high level of granulocytes at various stages of maturation with normocytic anaemia and a high/ normal platelet count
  • Chemo can control disease but bone marrow transplant is only cure
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15
Q

What is lymphoma?

A
  • Cancer of the lymphatic system
  • Malignant proliferation of cells derived from B or T cells
  • Malignant cells accumulate in lymphatic system and LNs causing solid tumour formation
  • Lymphomas account for 11% childhood cancers
  • M:F 2:1
  • Hodgkins or non Hodgkins
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16
Q

Hodgkins lymphoma

A
  • Reed Stenberg cells
    • Giant, multinucleated cells usually derived from B cells
  • Accounts for 45% cases of lymphoma in childhood
  • Rare before 2yrs
  • Sharp rise in cases during teenage years
  • Linked to EBV
  • Clinical features
    • Most common feature is painless lymphadenopathy which may be present for several months and typically above the clavicle but can be anywhere
    • Unexplained weight loss , fever and night sweats (drenching)
    • Mediastinal masses are common
  • Diagnosis
    • Confirmed by presence of Reed Sternberg cells on LN biopsy
    • After diagnosis CT and PET to look for mets and confirm staging
  • Management
    • Depends on the stage but in all cases involves a combination of chemo and radio
    • Responds well to treatment, 95% @5yrs
    • Mortality is higher from effects of treatment later on e.g. lung fibrosis or congestive heart failure rather than death related to cancer itself – important to tailor intensity of treatment to stage of disease
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17
Q

Non Hodgkins lymphoma

A
  • Most common
    • Mature B cell including Burkitt’s and diffuse large B cell
    • Lymphoblastic lymphoma
    • Anaplastic large cell lymphoma – cells express CD30 antigen which is an activation marker of B and T cells
    • EBV is also implicated in Burkitt’s lymphoma
  • Clinical features
    • Depend on stage and type
    • Children present with enlarging, non-tender lymphadenopathy or due to mass compressing structures
      • Resp symptoms due to effusion or SVC syndrome
      • Abdo pain, constipation
    • Hepatosplenomegaly in 40%
    • Less common for fever, night sweats to be present - these are known as B symptoms and are more likely to occur in higher grade disease
  • Diagnosis
    • Biopsy of mass or LN
    • Bone marrow biopsy to exclude leukemia and assist with staging
  • Management
    • Combination chemo is standard
    • Prognosis good, 85% at 5yrs
18
Q

Second most common cancer type in children?

A

CNS tumours

  • Almost always primary rather than mets
  • 60% arise in cerebellum
  • Main cause of death from childhood cancer
  • Inherited conditions such as neurofibromatosis and tuberous sclerosis are associated with CNS tumours
19
Q

How are CNS tumours classified?

A

Accroding to cellular origin

  • Gliomas arise from glial cells
  • Embryonal tumours arise from neural crest cells
  • Craniopharyngiomas are epithelial tumours that arise from remnants of Rathke’s pouch (embryological structure that forms the anterior pituitary)
20
Q

What are embryonal tumours

A

Embryonal tumours develop from cells that are left over from the early stages of our development

  • Form in the brain and spinal cord
  • Known as primitive neuroectoderm tumours and named according to location
  • Fast growing
  • Most are medulloblastomas which form in the cerebellum
  • Mainly found in children
21
Q

What is craniopharyngiomas?

A
  • 6-10% of all brain tumours in children
  • Most commonly found in pituitary stalk and project into hypothalamus and press on optic chiasm
  • Patients present with visual and endocrine disorders as tumour is near optic chiasm and pituitary
  • Considered histologically benign – 99% @ 5 years
  • Clinical features
    • Symptoms vary depending on size and location, effect on CSF flow etc
    • Clinical exam may show papilloedema indicating increase in ICP
22
Q

Symptoms of brain tumours in children

A
  • < 5 years
    • Vomiting, headache (presenting as irritability because young children struggle to localise pain), abnormal gait, behavioral change, lethargy, abnormal eye movement or squint, increasing head circumference, developmental delay
  • > 5 years
    • Vomiting, headache (persistent, recurrent, worse on waking, abnormal gait, lethargy, seizure, abnormal eye movement, blurred vision, deterioration at school
23
Q

Management of paediatric brain tumours

A
  • Depends on type and location but standard is surgical removal/ chemo/ radio
  • Radiotherapy is avoided in those <3yrs because it can damage developing brain
24
Q

What is neuroblastoma?

A
  • Tumour originating in neural crest part of the sympathetic NS
  • Third most common childhood cancer and most common solid tumour in those <1 year
  • Incidence highest in first year of life
  • Very rare in children >5 years
  • 50% occur in adrenal glands but can grow anywhere in sympathetic NS e.g. abdomen, chest
  • By time of diagnosis 50% have metastasised
25
Q

Clinical feature of neuroblastoma

A
  • Depend on affected site
    • Abdomen: distension, constipation, pain
    • Thorax: SOB, Horner’s syndrome, SVC syndrome
    • Spinal cord: leg weakness, loss of ability to walk or crawl
    • Orbital bone: bruising around eyes
    • Endocrine: HTN, diarrhoea
    • Skin: blueberry muffin
    • Eyes: rapid eye movements
26
Q

Diagnosis of neuroblastoma

A
  • Requires histo confirmation from biopsy or bone marrow aspirate
  • CT and bone marrow aspirate to detect mets
27
Q

Management of neuroblastoma

A
  • Depends on risk category assigned to patient
    • Low risk: surgical excision of tumour
    • Intermediate: surgery + chemo
    • High risk: surgery, chemo, radio + high dose chemo + peripheral blood stem cell rescue
28
Q

What is retinoblastoma

A

Embryological tumour of retinal cells

3% childhood cancers

40% cases: mutation in RB1

  • If mutation present tumour more likely to be bilateral
  • No mutation present, tumour likely to be unilateral
29
Q

Clinical features of retinoblastoma

A
  • Leukocoria (white pupil) is 1st sign usually
  • Noted in flash photography of child
  • Can also present with squint
30
Q

Diagnosis of retinoblastoma

A
  • Diagnosed on clinical appearance and examination is carried out under anesthetic
  • Biopsy is not needed
  • USS and MRI may be needed to stage
31
Q

Management of retinoblastoma

A
  • Aim is to treat tumour while preserving vision
  • Small tumours treated with cryotherapy or laser
  • Local radio can be applied to eye
  • Larger tumours may require removal of eye
  • Excellent prognosis – 99% at 5yrs
32
Q

What is nephroblastoma?

A
  • Embryonal tumours of kidney
  • 90% of renal tumours
  • Rare, accounts for 6% childhood cancers
  • Peaks 1-3yrs
  • 5% bilateral
  • 10% associated with other conditions e.g. Beckwith Wiedemann syndrome and hemi hyperplasia
33
Q

Clinical features of neprhoblastoma

A
  • abdominal mass (most common presenting feature)
  • painless haematuria
  • flank pain
  • other features: anorexia, fever
  • unilateral in 95% of cases
  • metastases are found in 20% of patients (most commonly lung)
34
Q

Diagnosis of nephroblastoma

A
  • Initial investigation is USS which identifies a tumour
  • CT or MRI to determine stage
  • Histological examination after nephrectomy
    • Transcutaneous biopsy not recommended as may cause mets
35
Q

Management of nephroblastoma

A
  • Nephrectomy = 1st line followed by chemo
  • If bilateral, masses are removed in attempt to preserve some kidney function
  • Radiotherapy if disease is advanced
  • 85% @5yrs
36
Q

Child with an unexplained large abdominal mass - what to do?

A

Refer for paeds review within 48hrs - possible Wilm’s tumour

37
Q

What is rhabdomyosarcoma?

A
  • Type of connective tissue tumour arising from primitive muscle cells
  • Incidence peaks at 3 years
  • Presents with hard, swelling typically in head/ neck, chest or abdo wall
  • Biopsy to confirm diagnosis
  • Management via surgical excision + chemo + radio
  • Worst prognosis of childhood cancers
  • 5 year survival = 70%
38
Q

What is osteosarcoma?

A
  • Most common bone tumour in children
  • Arises from primitive mesenchymal bone-forming cells
  • Incidence increases with age of child ad peaks during teenage growth spurt
  • Rare in those <5
  • Common tumour location = metaphyses of long bones – usually distal femur
  • Child may be asymptomatic, some present with pathological fracture
  • Management: surgical resection + chemo
    • Amputation may be needed
    • Metastasises easily
    • Poor prognosis – 70% at 5yrs in localised disease <30% if mets
39
Q

What is Ewing’s sarcoma?

A
  • Primitive neuroectodermal tumour of connective tissue
  • Rare
  • Most common in bones of leg and pelvis but can be anywhere
  • Peak incidence late adolescence
  • Can cause onion skin appearance on x-ray
  • Management: chemo 1st line
  • 2/3 @ 5 years
40
Q
A

Paediatrics (19 decks)

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