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Paediatrics > Malignancy > Flashcards

Malignancy Flashcards

1
Q

Age at presentation

A

Leukaemia affects children at all ages
-Early childhood peak
Neuroblastoma and wilms tumour in first 6y
Hodgkin lymphoma and bone tumours in adolescence

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2
Q

Short term side effescts of chemotherapy

A 
Bone marrow suppression
Immunosupression
Gut mucosal damage
Nausea and vomiting
Anorexia
Alopecia
Undernutrition
Anaemia
Thrombocytopenia and bleeding
Neutropenia
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3
Q

Drug specific side effects

A

Doxorubicin: cardiotoxicity
Cisplatin: renal failure and deafness
Cyclophosphamide: haemorrhagic cystitis
Vincristing: neuropathy

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4
Q

Functions of chemotherapy

A

Primaru curative .e.g. ALL
Control primary or metastatic disease .e.g. sarcoma, neuroblastoma
Adjuvant treatment for residual disease .e.g. Wilms

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5
Q

High dose therapy with stem cell rescue

A

Transplantation of bone marrow stem cells to intensify treatment to potentially lethal doses of chemo
Stem cells may be allogeneic or autologous
Principally use in the management of high risk or relapse leukaemia (allogenic) and solid tumours (autologous)

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6
Q

Examples targeted therapies

A

Tryosine kinase inhibtors .e.g. Imatinib
-Taregtting BCR-ABL fusion gene (philadealphia chromosome ALL and CML)
Monoclonal antibodies .e.g. rituximab
-Anti CD20 for lymphoma
-Anti GD2 for high risk neuroblastoma

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7
Q

Presentation Acute Leukaemia

A

Peak age 2-5y
Develops over the course of several weeks
Malaise
Anorexia
Anaemia: pallor, lethargy
Neutropenia: infection
Thrombocytopenia: bruising, petechaie, nose bleeds
Bone marrow infiltation: bone pain
reticulo-endothelial: hepatosplenopmegalthy, lymphaneopathy
-superior mediastinal obstruction (uncommon)
CNS: headaches, vominting, nerve palsies
Testis: testicular enlargement

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8
Q

Ix Leukaemia

A 
Abnormal FBC
-low Hb
-thrombocytopenia
Circulating leukaemic blast cells
Bone marrow examination for diagnosis
Clotting screen (risk of DIC)
LP for CSF involvement
Chest Xray for mediastinal mass (T cell disease)
Blood film
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9
Q

Mx ALL

A 
Blood transfusion for anaemia
Allopurinol to protect renal function
Stages:
-Remission induction: chemo + steroids
-Intensification: consolidates remission
-CNS: intrathecal chemo
-Continuing therapy: ~3y w cotrimaxole prophylaxis
-Relapse treatment: high dose chemo +/- irridation
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10
Q

Types of brain tumours

A

Astrocyoma ~ 40%
Medulloblastoma ~20% midline of posterior fossa
Ependymoma ~8% Posterior fossa
Brainstem glioma ~6% Poor prognosis
Craniophyngioma ~4% Squamous remnant of Ranthke pouch
Atypical teratoid/rhabdoid- rare, aggressive tumour

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11
Q

Poor prognostic factors in ALL

A

Age: <1y or >10y
Tumour load: >50 x 10^9
Genetics: MLL rearrangement, t4:11 haploidy
Chemo response: persistance of blasts in marrow
MRD: detectable MRD after induction

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12
Q

Presentation brain tumours

A 
Persistent/recurrent vomiting
Balance, co-ordination/walking problems
Behavioural change
Abnormal eye movements
Seizures without fevers
Abnormal head positioning
Persistent or recurrent headache
Blurred/double vision
Lethargy
Deteriorating school performance
Delayed or arrested puberty
Slow growth
Developmental delay/regression
Progressive increased in head circumference: separation of suture, bulging fontanelle
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13
Q

Brain tumour site and clinical features

A

Supratentorial: seziures, hemiplegia, focal neurology
Midline (craniopharyngioma): visual field loss, pituitary dailure (growth, DI, weight gain)
Cerebellar (medulloblastoma, astrocytoma, ependymoma): truncal ataxia, co-ordination difficulty, abnormal eye movements
Brainstem (glioma): cranial nerve defect, pyramidal tract signs, ataxia, no raised ICP

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14
Q

Ix Brain tumour

A

MRI scan

LP for suspected CSF metastasis (not in raised ICP)

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15
Q

Mx Brain tumour

A 
Surgery first line
-treat hydrocephalus
-provide tissue diagnosis
-maximum resection
-Brain and optic pathway no op
Radio/chemo variable
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16
Q

Long term effects brain tumours

A

Neurological disability
Growth and endocrine problems
Neuropsych and educational issues

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17
Q

Types of lymphoma

A

Hodgkin
Non-hodgkin
Burkitts

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18
Q

Features hodgkin lymphoma

A 
Painless lymphadenopathy
-usually in the neck
Larger and firm lymph nodes
Nodes may cause airway obstruction
Long history
Systemic features uncommon
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19
Q

Ix Hodgkin lymphoma

A

Lymph node biopsy
Radiological assessment of nodal sites
Bone marrow biopsy (staging)

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20
Q

Mx Hidgkin lymphoma

A

Combination chemotherapy
+/- rediotherapy
PET scanning to monitor treatement response
80% cure rate

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21
Q

Features non-hodgkin lymphoma

A 
Mediastinal mass
Bone marrow infltration
SVC obstruction: dysponea, facial swelling, flushing, venous distention in neck, chest and arms
Abdominal pain from obstruction
-palpable mass
-intussusception
22
Q

Ix Non-hodgkin lymphoma

A

Biopsy
Radiological assessment of all nodal sites
Examination of bone marrow and CSF

23
Q

Mx Non-hodgkin lymphoma

A

Multiagent chemotherapy

Survival 80%

24
Q

Features Burkitt’s Lymphoma

A

Endemic variant occurs in regions with EBV
Characteristically involves the jaw and other facial bones
Usually associated with HIV and immunosupression post-transplantation

25
Q

Features neuroblastoma

A

Arise from neural creast tissue in adrenal medulla and sympathetic nervous sytem
Most common before 5y
Abdominal mass
Large complex mass, crossing midline, enveloping blood vessels and lymph nodes
Paravertebral tumours: spinal cord compression
Children >2y typicaly mets: bone pain, bone marrow suppression

26
Q

Ix Neuroblastoma

A

Raised urinary catecholamines metabolite (VMA/HVA)
Confirmatory biopsy
Bone marrow sampling and MIBG scan

27
Q

Prognosis neuroblastoma

A

Age and stage at presentation main influencer
Most children >1y advanced disease and poor prognosis
Amplification of MYCN oncogene predicts aggressive behaviour
Risk of relapse high

28
Q

Mx neuroblastoma

A

Localised primary- surgery alone
May resolve spontaneously in infants
Mets- chemo (high dose with autologous stem cell rescue), surgery, radio

29
Q

Presentation neuroblastoma

A 
Pallor
Weight loss
Abdo mass
Hepatomegaly
bone pain
Limp
Paraplegia
Cervical lymphadenopathy
Proptosis
Periorbital bruising
Skin nodules
30
Q

Features Wilms tumour

A

Embryonal renal tissue
Most common renal tumour of childhood
Present <5y
Rare >10y

31
Q

Presentation Wilms tumour

A 
Abdominal mass
Haematuria
Abdominal pain
Anorexia
Anaemia
Hypertension
32
Q

Ix Wilms tumour

A

US/CT/MRI: intrinsic renal mass distorting normal structure
Staging to assess lung mets
Contralateral kidney function

33
Q

Mx Wilms tumour

A 
initial chemotherapy
Delayed nephrectomy
-histological staging 
radiotherapy in advanced disease
Good prognosis except in relapse
34
Q

Features soft tissue sarcoma

A

Cancers of connective tissue .e.g. muscle or bone
Most common: rhabdomyosarcoma
-orginates from primitive mesenchymal tissue

35
Q

Clinical features sarcoma

A

Head and neck:
-Most common
-proptosis, nasal obstruction, bloodied nasal discharge
Genitourinary:
-dysuria, obstruction, scrotal mass, bloodied vaginal discharge
Mets:
-lung, liver, bone, bone marrow
-15% of patients at diagnosis (poor prognosis)

36
Q

Ix sarcoma

A

Biopsy
Full radiological disease
Mets search

37
Q

Mx Sarcoma

A

Multimodality: chemo, surgery, radio
Ill-define tumour margins makes excision unsuccessful
Cure rate ~65%

38
Q

Features bone tumours

A

Uncommon before puberty
Limbs most common site
Localised persistent bone pain

39
Q

Ix Bone tumour

A 
Plain Xray
-destruction
-perosteal new bone formation
-soft tissue mass in ewings sarcoma
MRI and bone scan
Chest CT: lung mets
Bone marrow sampling
40
Q

Mx Bone tumour

A

Combination chemotherapy
En bloc resection of tumour with endop[rosthetic resection
Radiotherapy in Ewings, pelvic or axial

41
Q

Features retinoblastoma

A

White pupillary reflex
Squint
All bilateral tumours are hereditory (chromosome 13)
Present within 3y

42
Q

Ix Retinoblastoma

A

MRI

Examination under anaestetic

43
Q

Mx Retinoblastoma

A

Enucleation of the eye in advanced disease
Chemotherapy in bilateral disease to shrink tumours
local laser to retina
Radiotherapy mostly used in recurrence
Patients usually left visually impaired
Risk of 2’ malignancy esp. sarcoma

44
Q

Features Kaposi sarcoma

A 
Blood or lymph vessel triggered by HHV8
Rare in UK
Relevant in immunodeficiency .e.g. HIV
Generalised lymphadenopathy
(Adults present with purple/brown skin rash)
Chemo and antiretrovirals
45
Q

Long term sequelae of childhood cancer

A

Nephrectomy
Toxicity from chemo affecting renal function, cardiac function
Growth hormone deficiency
Bone growth retardation
Gonadal irradiation/chemo agents causing infertility
Neuropsych problems from cranial irradiation and brain surgery
Secondary malignancy
Educational disadvantage

46
Q

Manifestations of Langerhans cell histiocytosis

A

Bone lesions: pain, swelling, fracture
-Xray shows lytic lesion with well defined border, often skull
Diabetes insipidus
-proptosis and hypothalamic infiltration
Systemic LCH: most aggressive, seborrhoeic rash in infancy
-involvement of gums, ears, lungs, liver, spleen, lymph nodes, bone marrow

47
Q

Pathology Langerhans cell histiocytosis

A

Rare disorder

Abnormal proliferation of histiocytes

48
Q

Primary malignant liver tumours

A

Hepatoblastoma

Hepatocellular carcinoma

49
Q

Presentation liver tumour

A

Abdominal distention
Mass
Pain and jaundice- rare

50
Q

Ix liver tumours

A

Elevated serum alpha feroprotein

Good prognosis in hepatoblastoma

51
Q

Germ cell tumrous pathology

A

Arise from primitive germ cells migrating from yolk sac endoderm to form gonads in sacrococcygeal region
Most found in the gonads
alphaFP and betaHCG serum markers

52
Q

Mx germ cell tumours

A

Very responsive to chemotherapy

Paediatrics (22 decks)

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