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MBBS IV - Junior Clerkship WCS > JC124 (Paediatrics) - Childhood cancer > Flashcards

JC124 (Paediatrics) - Childhood cancer Flashcards

1
Q

Compare cancer in adult and children

  • Pathological types
  • Sites
  • Classification
  • Stage at diagnosis
A
  • Pathological types
    Adult: mostly carcinoma
    Children: Lymphoid malignancies, CNS tumors, Embryonal tumors
    (rarely carcinoma)
  • Sites:
    Adult: superficial or deep seated in visceral organ
    Children: usually deep seated in organs
  • Classification:
    Adult: by organ
    Children: by tissue of origin
  • Stage at diagnosis:
    Adult: Early or late
    Children: Usually Late
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2
Q

Compare cancer in adults and children

  • Efficacy of screening
  • Treatment outcome/ prognosis
A

Screening:

  • Adult: screening programs can be effective
  • Children: Not effective screening due to aggressive nature of most paediatric cancers

Treatment:

  • Adult: variable response
  • Children: Usually good response with good prognosis
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3
Q

Paediatric cancers

  • Typical age of presentation
  • Demographics
A
  • Typical age of presentation: birth to 4 years old
  • Demographics: Sligh male predominance
  • Ethnicity: More germ cell tumor in east Asians, less Hodgkin’s disease, Wilm’s tumor, Ewing’s sarcoma than Caucasians
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4
Q

List the top 10 most common paediatric cancers

A 
Acute leukaemia
Brain tumours
Non-Hodgkin lymphoma
Extracranial germ cell tumor
Neuroblastoma
Rhabdomyosarcoma
Osteosarcoma
Ewing sarcoma / peripheral PNET
Hepatoblastoma
Nephroblastoma
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5
Q

Etiologies of paediatric cancers

A
  1. Unknown in majority:
    - Possible acquired spontaneous mutation during developmental stage
    - Difficult to prove suspicion
  2. Genetic predisposition
    - Germline mutations in cancer-predisposing genes
    - Mutation in epigenetic control mechanisms: Oncogenes, Tumor suppressor genes (e.g. P53) , DNA repair genes (e.g. BRCA1)
  3. Immunodeficiency
    - More common in male (X-linked PIDs)
    - Increase susceptibility to cancer
  4. Environmental (short exposure)
    - Physical, radiation, chemical, biological agents

Family history is mostly unreliable for prediction of paediatric cancer

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6
Q

List familial paediatric cancers with autosomal inheritance

A
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7
Q

Difference in cancer treatment approach between paediatrics and adults

A

General difference from adults:
 More responsive to therapy
 Tolerate therapy better
 Different volume distribution for infants
 Long-term complications: effect on development, late complications

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8
Q

Long-term complications of paediatric cancer treatment

A

 Learning disability (cranial irradiation)
 Growth retardation (tumor in pituitary area)
 Subfertility (high-dose alkylating agent, e.g. cyclophosphamide)
 Organ dysfunction (e.g. ifosfamide - renal tubular damage)
 Second malignancy (e.g. radiotherapy - 9-10% develop secondary neoplasm
on irradiation site after 20-25 years)

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9
Q

Supportive care options for paediatric cancer treatment

A

 Blood product support
 Treat infection (antibiotics, antifungals, antivirals)
 Treatment of metabolic complications – tumour lysis syndrome (urate oxidase)
 Symptoms control (i.e. pain, vomiting)
 Nutritional support
 Psychological support
 Family support: empathetic approach to breaking bad news

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10
Q

Approach to breaking bad news about cancer in children

A

Be empathetic, prepare to listen
Provide affirmative diagnostic information, avoid using ambivalent terms
Do not provide too much information within a short time; just mention the framework of the
treatment plan & side effects
Provide “hope” but not “false hope” in prognosis
Help to pacify guilty feeling
 Non-inherited nature of most childhood cancers
 Non-contagious to other family members

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11
Q

Modalities of cancer treatment

General indication for each type

A

Chemotherapy - Mainstay therapy

Surgery - Mainstay treatment for solitary tumors

Radiation therapy - Mainly for brain tumors, solid tumors, palliative therapy

Targeted therapy - Novel technique for selected cancers

Cellular therapy: Stem cell (e.g. HSCT), Gene therapy, Immune modulation

Immune checkpoint inhibitors - selected cancers

Hormonal therapy (e.g. ER/PR+ breast cancer)

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12
Q

Examples of anti-toxicity regimens for cancer treatment

A

Anti-toxicity regimen:
 Methotrexate + folinic acid (leucovorin)
 Cyclophosphamide + Mesna
 Anthracycline + ICRF-187

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13
Q

Examples of immune modulation treatment for cancer

A

Monoclonal Ab for activation of NK cells and complement activation

Leukapheresis for synthetic proliferation of Cytokine-induced killer cells (CIK), lymphokine-
induced killer cells (LIK) and reinjection

Ex-vivo dendritic cells mix with cancer lysate and reinjection

Genetically modify Cytotoxic T-cells with cancer antigen sequence, co-stimulatory domains and signalling domain for hyperactive response against cancer

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14
Q

Myeloproliferative disorders (MPD)

  • Haematological abnormality
  • Disease entities
A

Abnormal proliferation of haematopoietic cells: High cell count but normal morphology (abnormal morphology in late stage/ severe MPD)

 PRV (polycythaemia vera)
 ET (essential thrombocythaemia)
 MF (myelofibrosis)
 CML (chronic myeloid leukemia)

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15
Q

Myelodysplastic syndrome

  • Haematological abnormality
  • Disease entities
A

Abnormal differentiation of haematopoietic cells

 RA (refractory anemia)
 RARS (refractory anemia with ring sideroblasts)
 RAEB-I (refractory anemia with excess blast)
 RAEB-II
 CMML (chronic myelomonocytic leukemia)
/JMML (juvenile myelomonocytic leukemia)

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16
Q

Types of leukaemia that occur in children

A

ALL - most common
AML - second most common
CML and MDS (rare)
CLL (ultra rare)

Origin: marrow, lymph node, thymus

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17
Q

Clinical features of paediatric leukaemia

A

Marrow failure
 Pallor
 Multiple petechiae and bruises (over limbs, H&N)

Constitutional symptoms
 Fever, LOA, LOW

Organ infiltration
 Scattered lymphadenopathy
 Moderate hepatosplenomegaly
 Bilateral shin tenderness (bone pain more common in ALL than AML because infiltrate marrow space and necrosis)
 Non-tender testicular swelling (more common in ALL than AML)

Leucostasis (involvement of CNS)
 Headache (uncommon)
 Nuchal rigidity

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18
Q

Paediatric leukaemia

  • Investigations for diagnosis
A

Peripheral blood: CBC with differential, Blast count, Electrolytes and urate and LDH for TLS

Peripheral blood smear

  • Morphology
  • Cytochemical staining (e.g. PAS, acid phosphatase for ALL)

Immunophenotyping: find lineage based on CD expression by flow cytometry

Bone marrow

  • Cytogenetics: FISH and Karyotyping
  • Molecular genetics: RT-PCR
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19
Q

Genetic abnormalities in paediatric ALL and AML

A

ALL:
Translocation t(12;21) (TEL-AML1 fusion transcript):
 Found in 23% children, uncommon in adult (2.8%)
Translocation t(9;22) Philadelphia chromosome (worse prognosis)
 Uncommon in children (3%), but found in 23.6% adult

AML: de novo AML – common fusion transcript:
 t(15;17)
 t(8;21)
 Inversion 16

20
Q

Treatment scheme for childhood ALL

A

First 4-6 months:

  1. Induction&raquo_space; consolidation&raquo_space; CNS prophylaxis
  2. Bone marrow transplant for high- risk group, not upfront
  3. 2nd consolidation

Following 1.5-2 years:
- maintenance (3rd consolidation)

21
Q

Treatment scheme for childhood AML

A

Short and intensive (toxic) as compared to ALL:
First 4-6 months:
1. Induction and consolidation
2. CNS prophylaxis (intrathecal chemotherapy)
3. Bone marrow transplant performed upfront +/- maintenance therapy

22
Q

Prognosis and outcome of paediatric ALL and AML

A

ALL: Berlin-Frankfurt-Munster (BFM) protocol
 Event-free survival of 75-80%
 Overall survival of 80-90%
 High-risk: survival still >50%

AML: Survival mostly 40%
 Overall survival of 60% for low-risk, 50% for high-risk

23
Q

Causes of ALL relapse in children

Survival of relapsed ALL

A

 7% unrelated second leukemia (new)
 8% same as diagnosis (retain abnormal original clone)
 34% clonal evolution from diagnosis
 51% clonal evolution from a preleukemic clone

Survival of relapsed ALL patients:
 50-60% still salvaged
 The later the relapse, the better the outcome; late relapses may only be treated by chemotherapy instead of transplant

24
Q

Common childhood brain tumors

A

Gliomas:

  • Astrocytoma (e.g. cerebellar astrocytoma)
  • Ependymoma
  • Others (e.g. brainstem glioma, optic glioma)

PNET (primitive neuroectodermal tumor)

  • Medulloblastoma
  • Others (e.g. pineoblastoma)
CNS germ cell tumor (60% at pineal gland)
- Germinoma (73%)
- Non-germinoma (27%):
o Yolk sac tumor
o Choriocarcinoma
o Teratoma (16%)
o Mixed

Others:

  • Craniopharyngioma
  • Atypical teratoid rhabdoid tumors (ATRT)
25
Q

Childhood medulloblastoma

  • Presentation
A

May obliterate 4th ventricle: progressive obstructive hydrocephalus
On-and-off headache and vomiting especially upon waking up
Unsteady gait
Motor incoordination
Cerebellar signs

26
Q

Tumor markers for childhood brain tumors

A

Non-germinoma:

  • Yolk sac tumour ↑αFP in CSF, serum
  • Choriocarcinoma ↑β-HCG
27
Q

Classification of astrocytoma and prognosis

A

Childhood astrocytoma:
 Grade I, II: good survival (90-100%); functional impairment depends on location of tumor
 Grade III, IV, brainstem: poor outcome (usually fatal)

28
Q

Modalities of treatment for childhood brain tumor

A
  1. Surgery
  2. Radiotherapy:
    - IMRT (intensity-moderated
    radiotherapy)
    - TOMO
    - Proton Beam
  3. Chemotherapy
  4. Targeted therapy: Avastin, bevacizumab – VEGF inhibitor, Nimutuzumab, m-TOR inhibitor…

5, +/- ventriculo-peritoneal shunt

29
Q

Prognosis of childhood medulloblastoma

A
30
Q

Prognosis of childhood germ cell brain tumor

A

Germinoma:
 Good survival (~90%)
 Late mortality may not be due to relapse but secondary complication related to previous treatment e.g. cranial radiotherapy
 Secondary complications: secondary brain tumor, progressive vascular anomaly in brain/ laminar necrosis

Non-germinomatous GCT:
overall survival not as great (~70%)

31
Q

Radiological feature of cortical laminar necrosis (secondary complication of cranial radiotherapy)

A

MRA (angiogram): serpingineous hyperintensity along the cortex - lesions due to neuronal loss

a/w high lactate and low NAA/tCr (N-acetylaspartate/total creatine) due to anaerobic glycolysis

32
Q

Long-term neurological impairments in childhood brain tumor survivors
Risk factors of higher impairment

A

> 1 neurological impairment in 70% (due to treatment, or original tumor causing brain dysfunction):

a) Motor impairment (56%)
b) Subnormal IQ (30%)
c) Visual impairment (24%)
d) Brain atrophy (21%)
e) Epilepsy* (21%)

Risk factors:
 <3 years
 High initial IQ
 Form of treatments: posterior fossa radiotherapy/ craniospinal irradiation +/- chemo

33
Q

3 clinical presentations of childhood non-hodgkin lymphoma

A

Lymphoblastic NHL
 Diffuse bulky LN
 Mediastinal enlargement (airway/ SVC obstruction)
 Hepatosplenomegaly

Burkitt’s NHL (sporadic form)
 Enlarged cervical/ supraclavicular LN
 Intussusception (abdomen)

Anaplastic Ki-1 NHL
 Thin-looking
 Skin nodules
 Pleural effusion
 Hemophagocytic lymphohistiocytosis
34
Q

Non-Hodgkin lymphoma (NHL)

Diagnostic investigations

A

Diagnosis – tissue biopsy:
excisional/incisional biopsy (resect the whole LN)

** avoid needle biopsy c.f. adults - single cell isolates can be lymphoma/ neuroblastoma/ rhabdomyosarcoma and has no bearing on treatment planning **

35
Q

Classification of Childhood non-Hodgkin Lymphoma

A
36
Q

Staging methods and treatment prognosis of childhood non-hodgkin lymphoma

A

Staging:
 Imaging e.g. MRI
 Radioisotopes scan

Treatment outcome: good survival (70-80%)

 Burkitt lymphoma (BL), DLBCL: up to 90%
 Lymphoblastic lymphoma (LBL): 80%
 Anaplastic large cell lymphoma (ALCL): 75%
 Primary mediastinal large B-cell lymphoma (PMLBCL): 50% **

37
Q

Neuroblastoma

  • Cell of origin
  • Organs affected
  • Clinical presentation
  • Confirmatory Ix
A
  • Cell of origin: neural crest cells (progenitor cells of sympathetic nerve)
  • Organs affected: neuroblastoma can be found along the path of neural crest cell migration:
     Sympathetic chain (trunk, ganglia)
     Adrenal gland (medulla)
  • Clinical presentation:
     Abdominal mass
     Cachexia
  • Confirmatory Ix:
     MIBG scan detects neural crest cells in neuroblastoma, pheochromocytoma
     Elevated catecholamines, urine VMA (vanilylmandelic acid) and HVA (homovanillic acid)
38
Q

Treatment for early-stage neuroblastoma

A

Early-stage neuroblastoma:

  1. Surgical resection
  2. 5 courses of chemotherapy (cyclophosphamide + doxorubicin)
  3. Second look surgery to find residual disease&raquo_space; salvage chemotherapy with Etoposide + carboplatinum
39
Q

Treatment for late-stage neuroblastoma

A

Do everything:
1. Chemotherapy

  1. Surgery
  2. Stem cell transplant
  3. Local irradiation
  4. Targeted therapy (monoclonal antibodies):
     Anti-GD2 attacks GD2 (tumor-specific antigen) on surface of neuroblastoma cells
     GM-CSF priming to increase opsonization of neutrophils, monocytes, macrophages
  5. Differentiating agent (isotretinoin (13-cis-retinoic acid))
40
Q

Types of extracranial germ cell tumor and organs affected

A

Germinoma: Dysgerminoma

Non-germinomatous GCT:
 Yolk sac tumour
 Choriocarcinoma
 Malignant teratoma (e.g. sacrococcygeal teratoma)

Distribution: 
 Thyroid and Pineal gland 
 Mediastinum
 Retroperitoneal
 Gonads
 Sacrococcygeal
41
Q

Treatment and prognosis of extracranial germ cell tumor

A

 Mainly treated with JEB (carboplatin, etoposide & bleomycin) for 4-6 courses

 Good overall survival with surgery (90-100%)

42
Q

Rhabdomyosarcoma

Types and organs affected

A

Embryonal type - chromosomal gain/ loss
 1/3 in head and neck (e.g. parameningeal area, jaw)
 1/3 in pelvis

Alveolar type (usually older children, adolescents) - specific fusion transcript
 Trunk
 4 limbs

43
Q

Treatment outcome of childhood rhabdomyosarcoma

A

Survival not good (50-60%)

 H&N, pelvis: hard to resect, poor resection margin - not removed completely by surgeon

 Residual disease treated by radiation and chemotherapy
(not perfect cure)

44
Q

Compare osteosarcoma and Ewing’s sarcoma

  • Location
  • X-ray feature
A

Osteosarcoma:

  • Metaphysis (near proximal/ distal end of long bone, e.g. femur)
  • X-ray: Sunburst/ sunray appearance at Codman’s triangle

Ewing’s sarcoma:

  • Diaphysis (middle part of long bone)
  • X-ray: Onion skin (grows layer by layer)
45
Q

Compare osteosarcoma and Ewing’s sarcoma

  • Treatment options
  • Outcome
A

Osteosarcoma;
1. Excision
2. Limb salvage: prosthesis (total joint replacement)/ allograft
3. Amputation
4. Rotationplasty
Prognosis: Localized - good survival (60-80%); metastasized - poor survival (20-30%)

Ewing’s sarcoma:
1. Excision
Resectable - good survival (85%)
Unresectable - poor survival (40-50%)

46
Q

Name 2 childhood liver cancers

A

A. Hepatoblastoma (chemo + surgery, good survival 80%)

B. Hepatocellular carcinoma (HCC) (uncommon due to universal vaccination program)

47
Q

List types of childhood kidney tumor

A

Wilms’ tumour (nephroblastoma): 2/3 (huge proportion) of childhood kidney tumor

Others: 
 Clear cell sarcoma
 PNET
 Mesoblastic nephroma
 inflammatory myofibroblastic tumor, mesoblastic fibroadenoma, RCC ...etc

MBBS IV - Junior Clerkship WCS (131 decks)

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