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Malignancy Flashcards

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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

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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

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A

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

23
Q

Mx Non-hodgkin lymphoma

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A

Multiagent chemotherapy

Survival 80%

24
Q

Features Burkitt’s Lymphoma

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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
Features neuroblastoma
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
Ix Neuroblastoma
Raised urinary catecholamines metabolite (VMA/HVA) Confirmatory biopsy Bone marrow sampling and MIBG scan
27
Prognosis neuroblastoma
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
Mx neuroblastoma
Localised primary- surgery alone May resolve spontaneously in infants Mets- chemo (high dose with autologous stem cell rescue), surgery, radio
29
Presentation neuroblastoma
``` Pallor Weight loss Abdo mass Hepatomegaly bone pain Limp Paraplegia Cervical lymphadenopathy Proptosis Periorbital bruising Skin nodules ```
30
Features Wilms tumour
Embryonal renal tissue Most common renal tumour of childhood Present <5y Rare >10y
31
Presentation Wilms tumour
``` Abdominal mass Haematuria Abdominal pain Anorexia Anaemia Hypertension ```
32
Ix Wilms tumour
US/CT/MRI: intrinsic renal mass distorting normal structure Staging to assess lung mets Contralateral kidney function
33
Mx Wilms tumour
``` initial chemotherapy Delayed nephrectomy -histological staging radiotherapy in advanced disease Good prognosis except in relapse ```
34
Features soft tissue sarcoma
Cancers of connective tissue .e.g. muscle or bone Most common: rhabdomyosarcoma -orginates from primitive mesenchymal tissue
35
Clinical features sarcoma
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
Ix sarcoma
Biopsy Full radiological disease Mets search
37
Mx Sarcoma
Multimodality: chemo, surgery, radio Ill-define tumour margins makes excision unsuccessful Cure rate ~65%
38
Features bone tumours
Uncommon before puberty Limbs most common site Localised persistent bone pain
39
Ix Bone tumour
``` 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
Mx Bone tumour
Combination chemotherapy En bloc resection of tumour with endop[rosthetic resection Radiotherapy in Ewings, pelvic or axial
41
Features retinoblastoma
White pupillary reflex Squint All bilateral tumours are hereditory (chromosome 13) Present within 3y
42
Ix Retinoblastoma
MRI | Examination under anaestetic
43
Mx Retinoblastoma
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
Features Kaposi sarcoma
``` 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
Long term sequelae of childhood cancer
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
Manifestations of Langerhans cell histiocytosis
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
Pathology Langerhans cell histiocytosis
Rare disorder | Abnormal proliferation of histiocytes
48
Primary malignant liver tumours
Hepatoblastoma | Hepatocellular carcinoma
49
Presentation liver tumour
Abdominal distention Mass Pain and jaundice- rare
50
Ix liver tumours
Elevated serum alpha feroprotein | Good prognosis in hepatoblastoma
51
Germ cell tumrous pathology
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
Mx germ cell tumours
Very responsive to chemotherapy

Paediatrics (22 decks)

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