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RACP paeds Qld Group > Oncology > Flashcards

Oncology Flashcards

1
Q

somatic mutation

A

mutations acquired after conception
not passed on to offspring

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2
Q

BWS cancer surveillance

A

exam and 3monthly abdo USS until 8 years
Looking for Wilms + hepatoblastoma

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3
Q

t(1;13) or t(2;13)

A

alveolar rhabdomyosarcoma
this translocation is diagnostic
worse prognosis than embryonal RMS
mets to bone marrow

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4
Q

t(8;14)

A

Burkitts lymphoma or B-ALL

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5
Q

t(11;22) in bone

A

Ewings sarcoma

22q12 EWSR1 gene associated with sarcomas

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6
Q

MYC amplification

A

MYC-N= Neuroblastoma

MYC-C (translocation)= Burkitts

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7
Q

RB1 gene

A

RetinoBlastoma
tumor suppression gene
1 gene lost= predisposition
2 genes lost= cancer

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8
Q

Loss of INI1/SMARCB1 gene

A

Rhabdoid brain (ATRT) or kidney

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9
Q

P52 mutation

A

loss of this tumor suppression gene =
Li Fraumeni syndrome
AD
osteosarcoma
soft tissue sarcoma
brain tumors
breast cancer
leukemia

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10
Q

Small round blue cell tumors

A

Neuroblastma
Medulloblastoma
Ewings sarcoma
Whilms
Rhabdomyosarcoma
Retinoblastoma
Hepatoblastoma

blue is nucleus on H+E stain - as rapidly dividing

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11
Q

Causes cytopenia

A

leukemia (blasts) or solid tumor bone marrow infiltration

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12
Q

why do we always use chemo instead of surgery

A

improves survival even in localised cancers
kills micro metastases (too small to see on imaging but we know they are almost always there)

makes surgery easier and safer, as tumor is less vascular (less bleeding) and smaller
Kills residual tumor that has to be left behind

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13
Q

Chemo that has highest risk second cancer

A
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14
Q

what age does renal cell carinoma present

A

> 10 years

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15
Q

Where do Whilms tumor metastasise to

A

lungs
lymph nodes
clots in IVC

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16
Q

WAGR syndrome

A

Wilms tumor (50%)
Aniridia (100%)
Genitourinary abnormalities
Retardation

WT1 deletion

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17
Q

Denys Drash syndrome

A

Nephropathy (proteinurea etc)
Intersex
Wilms tumor

WT1 missense mutation

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18
Q

Ewings sarcoma features

A

Embryonal - small round blue cell
Present in adolescence (but often younger than osteosarcoma)
2nd most common bone malignancy after osteosarcoma
Bone or soft tissue primary
Location: Diaphysis (“D” near “E”) + pelvic bones
Xray: Lytic (less bone, more black), onion skin (compared to osteosarcoma- scleorotic/sunburst)
Mets: lungs, bone, marrow (number of mets is a prognostic feature)
Symptoms: pain, soft tissue mass/swelling, erythema, mass, fever, anemia, unwell
Genetics: t(11;22) in 90%- diagnostic osteosarcoma doesnt have translocation*
EWSR1 rearrangement (chr 22)
Rs: surgery, chemo +/- radiation (radiosensitive unlike Osteosarcoma)
Axial tumors have worse prognosis as difficult to resect

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19
Q

Osteosarcoma features

A

Present in adolescence
Most common bone cancer in children
Location: Metaphysis (“M” near “O”)
- knee and proximal humerus most common
Xray: sunburst, sclerotic (extra bone, more white)
Mets: lung and bones
Symptoms: pain, mass, pathological #
Genetics: retinoblastoma predisposition (RB1), P53 mutation, prior RT
Rx: surgery and chemo, no radiation,

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20
Q

Rhabdomyosarcoma

A

Malignant tumor arising from mesenchymal cells arrested in myogenic differentiation, can arise anywhere in body that has muscle

Embryonal
- “resemble foetal muscle”
- more common
- better prognosis
- younger age
- central
- low-mod invasiveness
- mets to lung
- no translocation

Alveolar
- “resembling pulmunary alveoli”
- worse prognosis
- fusion protein t(1;13) t(2;13)
FOXO1-PAX5 fusion
- mets everywhere
- incurable

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21
Q

Neuroblastoma presentation

A

Localised disease
1. Asymptomatic mass in neck, thorax, abdomen, pelvis
2. Hepatomegaly
3. Symptoms due to mass effect
Spinal cord compression
Motor deficits are most common followed by radicular back pain, bladder an bowl dysfunction, and rarely sensory deficits = MEDICAL EMERGENCY, rapidly progressive
Bowel obstruction
Superior vena cava syndrome
Horner’s syndromeor just ptosis (always check if there is a neck lump with new onset ptosis)
Thorax–>respiratory distress, Horner’s, incidental
Pelvic/sacral–> mass, dysuria, constipation

Metastatic disease
1. Non-specific symptoms of marrow failure: fever, bruising, petechiae, pallor
2. “Racoon eyes” - Ptosis and periorbital ecchymoses suggests orbital metastases
3. Bone pain, limp, refusal to walk

Systemic symptoms
1. Produce catecholamines–> sweating, hypertension (NB. hypertension may also be due to renal artery compression)
2. Tumour lysis syndrome
3. DIC
4. Weight loss
5. Irritability
6. Intractable diarrhoea (VIP)
Subcutaneous nodules

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22
Q

3 types of neuroblastoma risk

A

High risk malignant
- MYCN amplification
- rx kitchen sink including stem cell transplant and immune therapy (Dinutuximab)

Intermediate risk
- chemo and surgery
- no stem cell transplant, no immune therapy, radiation rare

Low risk
- localised
- small ones in babes can resolve spontaneously (highest rate of spontenous resolution of any cancer)
- larger ones cured with resection alone

Blueberry nodules on a baby + rapidly expanding liver + resp distress –> quick death

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23
Q

Familial adenomatous polyposis

A

APC mutation
hundreds to thousands of precancerous colorectal polyps (adenomatous polyps). If left untreated, affected individuals inevitably develop cancer of the colon and/or rectum at a relatively young age.
Risk hepatoblastoma in affected kids

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24
Q

Risk factors hepatoblastoma

A

BWS
FAP
Li Fraumeni
T18
NF-1
Ataxia telangiectasia
TS
Fanconi anemia

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25
Q

AFP is elevated in

A

Hepatoblastoma
HCC
Germ cell tumors
Ataxia telangiectasia

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26
Q

Hepatoblastoma chemo

A

cisplatin
carboplatin

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27
Q

Sodium thiosulfate

A

antidote for cyanide poisoning
protects against cisplatin induced hearing loss

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28
Q

Germ cel tumors

A

begins in cells that give risk to sperm or eggs
can occur anywhere in body
benign or malignant
GCT: 1/3 gonadal, 2/3 extragonadal from aberrant migration

Most are curable but some are highly malignant

Risk increased with gonadal dysgenesis - eg Kleinfelters- mediastinal GCC
Turners - increased risk gonadoblastoma

Immature germ cell tumors (malignant) are positive for AFP or bHCG
Mature teratomas (benign) are negative for AFP and bHCG
**as AFP normal values change with age quickly in first year of life, may need to do serial measurements

Rx: platinum chemotherapy (cisplatin or carboplatin) or surgery alone (eg ovarian teratoma doesnt need chemo)

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29
Q

WT1 mutation associated with

A

Wilms tumor/Denys Drash/WAGR syndrome
AML

somatic mutations mean the cancer is there in that tissue
germline mutations are inherited and give predisposition to cancer eg WAGR, Denys Drash, Frasier syndrome

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30
Q

what do you do if a kid presents with lower back pain and subjective alteration in perianal sensation

A

urgent MRI spine
Alterations in perianal sensation/saddle parasthesia often preceed complete loss of sensation, bladder/bowel incontience and weakness
Cauda equine and cord compression may progress or complete in under 24 hours, and defects at that stage are typically irreversible
Therefore dont wait for full blown clauda equina, look for subtle signs as the longer the nerve is damaged, the lower the chance of recovery
would need high dose steroids and surgery
to decompress

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31
Q

is HSCT used in solid tumors?

A

yes
Autologous transplant to allow more/higher dose chemo to be given

**allogenic STC used in leukemias “graft versus disease”

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32
Q

tumor suppressor vs oncogenes

A

Tumour suppressor genes (inactivation)
Regulators of cellular growth and apoptosis
Inactivation of BOTH alleles required for a tumour suppressor gene
Inheritance of one germline mutation can be AD
A second mutation at somatic level still required
In inherited mutations, one inactivated allele may be inherited and the other undergoes spontaneous inactivation
Examples: P53 (usually initiates apoptosis) , APC, Rb, BRCA

Proto-oncogenes (activation)
Activating mutation in ONE gene results in an oncogene , via:
1. Amplification
2. Point mutations
3. Translocation
These gees interfere with apoptosis, continue to proliferate
Examples
Chromosome translocation
T(1;19) – pre- B ALL
T(14:18) – C Myc in Burkitt’s
T(9:22) – Philadelphia chromosome in ALL, CML

Gene amplification = N myc in neuroblastoma (poor prognosis)
Point mutation
1p in AML – NRAS signal transducer, point mutation

translocations are usually proto onco genes*

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33
Q

T (14:18)

A

C Myc
Burkitts lymphoma

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34
Q

T(9:22)

A

Philadelphia chromosome
ALL, CML
BAD PROGNOSTIC FACTOR

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35
Q

T (1:19)

A

pre B-ALL

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36
Q

Inactivation in DNA repair genes results in

A

Fanconi anaemia (AR leukaemia)
Bloom’s syndrome (AR leukemia and lymphomas)
Ataxia-telangiectasia (AR lymphoreticular cancers)
Dysplastic nevus syndrome (AD melanoma)

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37
Q

Dyskeratosis congenita predisposes to cancer by which mechanism

A

Mutations in telemere maintenance pathways
Short telemeres

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38
Q

Viruses and cancer predisposition

A

EBV: Burkitt lymphoma
Diffuse large cell B cell lymphoma
Hodgkin lymphoma
Post-transplant lymphoproliferative disorder
Nasopharyngeal carcinoma
Leiomyosarcoma
Gastric adenocarcinoma

Hepatitis B:	Hepatocellular carcinoma
Hepatitis C:	Hepatocellular carcinoma
	
HPV	:Cervical carcinomas
	Anus, penis, vulva/vagina, oropharyngeal carcinomas
HHV8:	Kaposi’s sarcoma
	Primary effusion B-cell lymphoma
	Plasma cell variant of Castleman disease
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39
Q

Li Fraumeni syndrome

A

mutation in p53- inactivation of tumor suppression gene

Sarcomas (soft tissue and osteosarcoma), leukeminas,astrocytoma, medulloblastoma, breast cancer, bone, lung, brain

If adrenocorticocarcinoma or choroid plexus carcinoma, LFS unless proven otherwise
If medulloblastoma + Li-Fraumeni, almost certainly Sonic Hedgehog subtype

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40
Q

NF1 cancers

A

optic glioma
neurofibroma
phaeochromocytma
meningioma
astrocytoma

soft tissue sarcoma
breast cancer >age 50

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41
Q

NF2 cancers

A

bilateral acoustic neuromas, meningiomas, epyndymomas

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42
Q

Von Hippel-Lindau disease

A

Autosomal dominant, mutation of tumour suppressor gene VHL
Cysts, benign and malignant tumours

hemangioblastoma
renal cell carcinoma
pheochromocytoma

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43
Q

Bloom syndrome

A

Short stature, photosensitive telangiectatic erythema(red rash in sun exposed areas, esp face/cheeks)
Immune deficiency
Excessive number of broken chromosomes due to DNA repair defects
Increased risk leukaemia, lymphoma, solid tumours(AML most common

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44
Q

Ataxia telangiectasia

A

AR
Mutation in ATM tumour suppressor gene (11q22-23)
Neurodegenerative; ataxia, telangiectasia, immunodeficiency with sinopulmonary infections, impaired organ maturation, X-ray hypersensitivity
Lymphoma, leukaemia

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45
Q

Defects in immune surveillace lead to ..

A

Leukaemia+ lymphoma
1. Wiskott-Aldrich
2. SCID, CVID
3. X linked lymphoproliferative syndrome
4. Kostmann syndrome
1. Congenital neutropaenia
Risk of myelodysplastic syndrome/ leukaemia

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46
Q

Downs syndrome cancer risk

A

500 fold increase in AML. 25% of kids with TAM develop AML
20 fold increase in ALL

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47
Q

Gorlins syndrome

A

medulloblastoma + BCC

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48
Q

MEN 2A

A

Medullary carcinomas of thyroid
PTH adenoma
Phaeochromocytoma

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49
Q

Alkylating agents examples and MoA

A

Add alkyl groups –> cell cycle arrest –> death
Work in all phases of cell cycle

Cyclophophamide
Ifosfamide
- both alkylate guanine and thus inhibit DNA synthesis

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50
Q

Cyclophosphamide side effects

A

N/V
Myelosuppresion
Alopecia
HAEMORRHAGIC CYSTITIS
PULMUNARY FIBROSIS
SIADH
INFERTILITY
Secondary neonplasm

**use hyperhydration + mesna to prevent haemorrhagic cystitis

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51
Q

Ifosfamide side effects

A

N/V
Myelosuppression
Haemorrhagic cystits (less common than cyclophosphamide)
SIADH
Iphosphamide encephalopathy (rx thiamine, methylene blue)
Renal tubular acidosis (proximal RTA/Fanconi)
Infertility

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52
Q

Antimetabolites MoA

A

Disrupt DNA synthesis
Cause cell death during S phase of cell cycle

Eg
Methotrexate = folate atagonist, inhibits DNA synthesis

6- mercaptopurine = inhibits purine syntheiss

Cytarabine (Ara-C)= pyrimidine analog, inhibits DNA polymerase

5-flurouracil = pyrimidine analog

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53
Q

Methotrexate adverse effects

A

N/V
Hepatitis (LFT derangement)
Photosensitive dermatitis
Myelosuppression
High dose- renal and CNS toxicity (lowers IQ)

can accumilate and cause toxicity in 3rd space fluid eg pleural/ascites
Need hyperhydration, urinary alkalinisation and folinic acid to avoid toxicity

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54
Q

Vinca alkaloids MoA

A

Inhibit microtubule assembly during MITOSIS, causing cell arrest
eg
Vincristine
Vinblastine

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55
Q

Vincristine side effects

A

constipation
abdo pain
neuropathy- peripheral, autonomic, cranial nerve
jaw pain
mucositis
phlebitis
Alopecia
VESICANT

IV only

Minimal myelosuppression
Not ematogenic

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56
Q

Topoisomerase MoA

A

Disrupts topoisomerase I and II –> inhibits DNA replication
Works in S + G 2 phase of cell cycle
eg
Etoposide

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57
Q

Etoposide side effects

A

N/V
Myelosuppression
Secondary leukemia
Type 1 hypersensitivity reactions

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58
Q

Anthacyclin MoA

A

increase oxygen free radicals–> cell apoptosis
Intercalates DNA
Not specific to any phase in cell cycle

eg
Doxorubicin
Danorubicin

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59
Q

Doxorubicin side effects

A

N/V
Cardiomyopathy
Radiation dermatitis
Myelosuppression
Arrythmia
*dexrazoxane reduces risk of cardiotoxicity**

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60
Q

Platinum agents MoA

A

Inhibit DNA synthesis by cross linking DNA
Not phase specific

eg
carboplatin
cispatin

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61
Q

Cisplatin side effects

A

HIGHLY EMATOGENIC including delayed N/V
Ototoxicity - very common
Nephrotoxicity
Hypomagnesemia
Myelosuppression, seizures, neuropathy- uncommon
THROMBOSIS

**sodium thiosulfate and amifostine otoprotective

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62
Q

L- asparaginase works at which phase of cell cycle

A

G1

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63
Q

L-asparaginase side effects

A

Anaphylaxis
Pancreatitis
Coagulopathy
Hyperglycemia
Cerebral sinus thrombosis

*give PEG asparaginase if become allergic to L-asparaginase

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64
Q

Bleomycin works at which phase of cell cycle

A

G2

65
Q

Bleomycin side effects

A

N/V
Pneumoatosis
Mucocutaneous reactions and dermatitis
Alopecia
Pulmunary fibrosis- less common
NO MYELOSUPPRESSION

65
Q

Bleomycin side effects

A

N/V
Pneumoatosis
Pulmunary fibrosis
Mucocutaneous reactions and dermatitis

NO MYELOSUPPRESSION

66
Q

Ciclosporin MoA

A

Calcineurin inhibitor
Reduce IL-2 and IL-2 receptor production

67
Q

Ciclosporin side effects

A

Hirsuitism
Gingival hyperplasia
Nephrotoicity
Tremor
Headache
Hyperglycemia
Elevated LFTs
HTN

68
Q

Which drugs cause no or minimal myelosuppression

A

Vincristine
Asparaginase
Bleomycin

69
Q

Which drugs cause alopecia

A

Vincristine
Cyclophosphamide

70
Q

Which drugs cause mucositis

A

antimetabolities
doxyrubicin
bleomycin
etoposide

71
Q

which chemo agent is most ematogenic

A

cisplatin

carboplatin
Cyclophophamide
High dose methotrexate
Cytarabine

72
Q

which chemo agents are minimally ematogenic

A

Vincristine
Asparaginase
Bleomycin
Mercaptopurine
Low dose methotrexate

low- flurouracil, busulfan, low dose cytarabine, doxorubicin, etoposide,

73
Q

which chemo agents have highest risk secondary malignancy

A

etoposide (AML)
Cyclophosphamide (AML)
Carboplatin/cisplatin

74
Q

which chemo agents are associated with SIADH

A

cyclophosphamide
ifosfamide
cisplatin

75
Q

which chemo drugs can be given intrathecally

A

methotrexate
cytarabine

76
Q

at which phase of cell cycle to topoisomerase inhibitors work

A

S and G2/M

77
Q

at which phase do vinca alkaloids work

A

M phase

eg vincristine (microtubule formation)

78
Q

which chemo agents are cell cycle indepenant

A

Platinum analgues - cisplatin, carboplatin
Anthracyclines- doxorubicin
Alkylating agents - cyclophosphamide, ifosfamide
busulfan

79
Q

which chemo agents work in G2

A

bleomycin
etoposide

80
Q

Cytarabine side effects

A

fever and flu like symptoms
mucositis
phlebitis
diarrhoea
GI ulceration
deranged LFT
neuro and pulmunary toxicity

81
Q

side effects busulfan

A

acute lung injury or intersitial lung fibrosis
N/V
Diarrhoea

82
Q

chemo agents associated with radiation enhancement/radiation recall

A

bleomycn
dactinomycin
doxorubicin
hydroxyurea
methotrexate

radiation recall only - arsenic, cyclophosphamide, cytarabine, etoposide,

83
Q

Tumor lysis syndrome

A

hyperkalemia
hyperphosphatemia
hyperuricemia
hypocalcemia (binds to phosphate)

84
Q

risk TLS highest in

A

acute leukemias WCC> 100
Burkitts lymphoma
Large tumors eg abdominal
patients with preexisitng renal disease/hyperuricemia

85
Q

timing of TLS

A

usually within 24-48 hours of starting treatment, up to 7 days after starting

86
Q

Consequences of TLS

A

hyperuricemia- precipitation uric acid in tubules – inflammation – AKI
hyperphosphatemia – precipitates calcium phosphate and uric acid deposition in kidneys
–> uric acid nephropathy (in acidic urine), or calcium phosphate nephropathy (at alkaline pH)
Hyperkalemia– arrythmias
Hypocalcemia- seizures, muscle cramps, tetany

87
Q

Treatment TLS

A

Hyperhydration
Alkalinization is usually necessary to maintain a urine pH of approximately 7.0. The urinary excretion of uric acid is reduced at an acidic pH, whereas the excretion of phosphorus is impaired by over-alkalinization (and they precipitate more at these pHs)

Allopurinol
- if low risk
BLOCKS XANTHINE OXIDASE
- blocks catabolism of hypoxanthine and xanthine (more soluable than uric acid)- but xanthine can still precipitate at high levels
-* contraindicated in combination with azathioprine or 6MP as causes severe bone marrow suppression

Rasburicase
- if high risk
- urate oxidase. Catalyses uric acid into water soluable allantoin

88
Q

Treatment of electrolyte derangements in TLS

A

Treatment of hyperkalaemia:
1. Cardiac membrane stabilisation -> IV calcium gluconate immediately
2. Shift of K+ intracellularly -> insulin/glucose infusion, sodium bicarbonate, beta-agonists (salbutamol)
3. Reduction of K+ load -> resonium, loop diuretics, dialysis (last resort)

Treatment of hyperphosphatemia:
1. Dietary restriction of phosphate
2. Phosphate binders
3. Dialysis

Treatment of hypocalcemia:
1. IV calcium gluconate with caution as can increase precipitation of calcium phosphate (Don’t give if hyperphosphatemia. Don’t treat if asymptomatic)

89
Q

Flow cytometry

A
  1. Immature cells = CD34, CD117, HLA-DR
    1. T cells = CD2, CD3, CD4, CD8
    2. B cells = CD10, CD19, CD20, CD22
    3. Myeloid = CD15, CD33
90
Q

Risk factors for ALL

A

T21
NF1
Bloom syndrome
Ataxia telangectasia

91
Q

Signs of extramedullary disease in ALL

A

i. Lymphadenopathy + hepatosplenomegaly
ii. Bone pain +++ – particularly lower extremities, can be severe and wake patient at night
iii. T cell disease: mediastinal adenopathy causing major airway compression with stridor and/or superior vena cava obstruction
iv. Signs of CNS involvement are INFREQUENT at diagnosis:
1. Headache, nausea and vomiting
2. Irritability, nuchal rigidity and papilledema
3. Cranial nerve involvement – most frequently involving CNVII, CNIII, CNIV, CNVI
v. Testicular involvement at diagnosis is rare – appears as painless testicular enlargement and is usually unilateral
vi. Fever- inflammatory cascade from tumor cells

92
Q

Risk stratification in ALL

A

i. Age (1-10 years favourable, <1 yr, >10 yrs unfavourable)
ii. WCC at presentation (<50 favourable)
iii. Cytogenetic/molecular eg Ph+ = risk factor
iv. Response to induction (= MRD) – biggest prognostic factor
v. Testicular disease, CNS disease poorer prognosis
b. Low risk
i. High risk features not present
ii. Favourable cytogenetics
1. Hyperdiploidy (>50)
2. Trisomies 4, 10
3. ETV-RUNX protein (t12;21)
c. Standard risk
i. High risk features not present
ii. No favourable genetics
d. High risk/ very high risk
i. Age = <1 or >10 years
ii. Presentation
1. WCC > 50 at diagnosis
2. CSF involvement
3. Testicular involvement
iii. Additional cytogenetic + molecular features
1. Hypodiploidy (<44)
2. KMT2A/MLL genetic rearrangements - translocations of 11q23 (eg. t4;11)
3. iAMP21 amplification
4. Philadelphia chromosome t(9;22) BCR-ABL fusion protein –> very poor prognosis

93
Q

Which feature on blood film is characteristic of AML

A

Aurer rod

94
Q

Immunohistochemistry in AML

A

CD15 or CD33 positive
Negative for CD3 or CD 19 (as not B or T cells)
Myeloblasts will stain positive with myeloperoxidase

95
Q

which cytogenetic factors give a favourable prognosis in AML

A

t (8,21), t (8,17), inv (16)
NPM1

95
Q

which cytogenetic factors give a favourable prognosis in AML

A

t (8,21), t (8,17), inv (16)
NPM1

96
Q

Acute promyeloocytic leukemia (APML)

A

t(15;17)= PML- RARA

Chemo= all trans retnoic acid + anthrayclines + cytarabine

risk differentiation syndrome and DIC

97
Q

T21 and leukemia

A

500 x more likely to develop AML (but better survival than other kids)
30 x more likely to develop ALL
GATA1 mutations

98
Q

Chronic myeloid leukemia

A

Most due to Philadephia chromosome t(9,22) BCR-ABL fusion protein
encodes oncogenic protein
Philadelphia chromsome on FISH

Rx: tyrosine kinase inhibitors (imatinib, dasatinib)
- inhibits BSR-ABL tyrosine kinase
Hydroxyurea
Allogenic SCT

99
Q

Juvenile Myelomonocytic Leukaemia (JMML)

A

rare cancer of infancy
Associated with mutations in RAS/MAP kinase pathway
Only curative rx is stem cell transplant

Risk factors:
- NF1
-Noonan syndrome (mutations in PTPN11, NRAS, KRAS)
Ix: monocytosis, thrombocytopenia, anemia, BM= hypercellular myeloid cells, <20% myeloblasts, molecular analysis

100
Q

Staging non hodgkins lymphoma

A

Murphy
I: Tumor at one site (nodal or extranodal – “E”)
II: Two or more sites; same side of body (or resectable GI primary)
III: Both sides of body
IV: CNS or marrow involvement

101
Q

Staging hodgkins lymphoma

A

Ann Arbor
I: Tumor at one site (nodal or extranodal – “E”)
II: Two or more sites; same side of body (or resectable GI primary)
III: Both sides of body
IV: Lung, liver, or bone mets

A- asymptomatic
B- B symptoms present

102
Q

Hodgkins lymphoma key points

A

a. A malignancy of the germinal centre B-Cells that affects the reticuloendothelial and lymphatic systems
b. Characterised by the presence of Reed-Sternberg cells (histopathologically)
c. Spread: slow, predictable, with extension to contiguous lymph nodes
Haematogenous spread also occurs (LESS COMMON)  liver, spleen, bone, bone marrow or brain
d. Associated with B symptoms (which are a poor prognostic factor)
e. usually presents with painless, firm rubbery cervical /supraclavicular lymphadenopathy. Mediastinal mass of HSM.

Most commonly affects teens, rare <5 years
Less common than non Hodgkins lymphoma

103
Q

Non Hodgkins lymphoma key points

A

a. Accounts for 60% of lymphomas in children
b. 2nd most commonly malignancy in those 15-35 years old
c. Burkitt lymphoma most common in children 0-14 years
d. DLBCL most common in adolescents/young adults
2. Key points
a. Malignant solid tumour characterised by undifferentiated lymphoid cells
b. Spread: aggressive, diffuse, unpredictable
c. Involves lymphoid tissue and can infiltrate the BM and CNS
d. Characterised by a high growth fraction and doubling time
e. Early diagnosis and treatment is critical
Rapid chemotherapy response can occur, therefore there is a higher risk for tumour lysis3. Risk factors
a. Inherited or acquired immunodeficiency (eg. SCID, Wiskott-Aldrich, HIV)
b. Viruses (eg. HIV, EBV)
c. Part of genetic syndrome (eg. ataxia-telangiectasia, Bloom syndrome)
d. Radiation therapy/ exposure
e. Post T cell depleted HSCT
Post solid organ transplan

104
Q

Non Hodgkins lymphoma staging

A

a. Stage I: Involvement of single tumour or single anatomic area excluding the mediastinum or abdomen
b. Stage II: Two or more lymph node regions on the same side of the diaphragm or resectable primary abdominal
c. Stage III: Involvement of lymph node regions on both sides of the diaphragm; all primary mediastinal, paraspinal or extensive intra-abdominal disease
Stage IV: Any of the above and initial involvement of the CNS, BM or both

105
Q

Infratentorial brain tumors

A

aka cerebella
present as truncal and gait ataxia, CN palsy
Usually CN 6 first (abducens)- LR affected, unable to move gaze laterally
CN4 (trochlear, SO affected)- double vision
CN3- oculomotor- down and out position, dilated pupil, ptosis
Nystagmus

106
Q

Brainstem tumors

A

CN palsies
Nystagmus
Gait and coordination difficulties

UMN signs- hemiparesis, hyperreflexia, clonus

107
Q

Supra tentorial tumors

A

Lateralized deficits (focal motor weakness, focal sensory changes, language disorders, focal seizures, reflex asymmetry

108
Q

Supra-sellar tumors

A

most common= craniopharyngioma, astrocytoma
Visual field defects
neuroendocrine defects = obesity, abnormal linear growth velocity, diabetes insipidus, galactorrhoea, precious puberty, delayed puberty and hypothyroidism
1. Di-encephalic syndrome = failure to thrive + emaciation despite normal caloric intake + inappropriately normal or happy affect(tumor in diencephalon (hypothalamus, optic chiasm), most commonly pilocytic astrocytoma)

109
Q

Diencephalon

A

structures that are on either side of the third ventricle, including the thalamus, the hypothalamus, the epithalamus and the subthalamus

Arises from the prosencephalon

110
Q

Perinaud syndrome

A

Pineal gland tumor/midbrain infarction or haemorrhage/obstructive hydrocephalus
Paralysis of upward gaze
Pupils not reactive to light (pseudo Argyll RObertson pupil)
Nystagmus to convergence
eyelid retraction

111
Q

What brain tumors come from supporting cells?

A

Gliomas- further divided into:
astrocytomas
ependmoma
oligodendrocytomas
mixed glial/neuronal

112
Q

what brain tumors come from primitive tissue

A

medulloblastoma
neuroblastoma
pNET
Atypical teratoid/ rhabdoid tumors (ATRT)

113
Q

Ependymoma

A

arise from lining of ventricles/spinal cord (most in posterior fossa)
bimodal- in kids <5 years
NF2 have increased risk
MRI: calcifications common, arise at ventricles, can look very nasty and heterogenous
Histology: pseudorosettes/ependymal rosettes
Rx: surgery + radiation, not usualy chemosensitive
Worse prognosis if cant do GTR, posterior fossa disease, diseemniated disease

114
Q

Astrocytoma

A

Subset of glioma
Can be low grade (eg pilocytic) or high grade (DIPG/GBM)
optic pathway gliomas are low grade astrocytomas

Pilocytic astrocytoma typically in cerebellum
NF1= better prognosis
MRI: contrast enhanging nodule within wall of cystic mass
Histology: Rosenthal fibres (corkscrew pink bundles)

115
Q

Pilocytic astrocytoma

A

Most common type of low grade astrocytoma
Typically in cerebellum, optic pathway, or tectal
Commonly in NF1
Locally aggressive but rarely invasive
May stabilise or even regress without intervention
MRI: contrast enhanging nodule within wall of cystic mass, in posterior fossa or optic tract, often associated with hydrocephalus
Histology: Rosenthal fibres (corkscrew pink bundles)
Molecular: change in BRAF (RAS/MAPK) pathway (can use BRAF/MEK/mTORinhibitors)
Rx: slow growing so dont respond well to chemo.
Can do nothing, just watch if normal visual acuity
Surgery is first line
If progression- small amount of chemo (carboplatin, vincristine, vinblastine)
Survival 90%

116
Q

High grade astrcytomas include:

A

Anaplastic astrocytoma
Glioblastoma multiforme
DIPG (midline GBM)- very agressive, 90% mortality within years

117
Q

Primitive neuroectodermal tumors (PNET)

A

Embryonal tumors
Include medulloblastoma and ATRT

118
Q

Medulloblastoma

A

most common childhod malignant brain tumor
Arise from cerebellum- usually vermis
1/3 have mets at presentation - usually to CNS
M>F
Peak age 3-9 years

Prognostic factors:
Good= WNT1, age >3 years, localised disease
Bad= MYC amplification (Group 3) , p53
Intermediate = Sonic hedgehog (better prognosis in infants; but SHH +p53 are very bad), group 4, >1.5 cm2 residual tumor, large cell anaplastic
Babies <3 years automatically high risk - cant do radiation

Rx: surgery, chemotherapy, radiation
Craniospinal irradiation with a boost to the posterior fossa followed by intensive adjuvant chemotherapy
MRI of spine + lumbar CSF obtained at least 10 days after surgery

119
Q

Atypical teratoid/rhabdoid tumors (ATRT)

A

highly malignnat, fast growing
can appear anywhere in brain or spine
usually in kids <3 years
short clinical hx
mets in 20% at presentation
small round blue cells + rhabdoid tumor cells deletion/inactivation of INI1/SMARCB1
Poor prognosis, median survival 12 months
Increased risk for renal and soft tissue tumors as well

120
Q

Craniopharyngioma

A

A craniopharyngioma develops from remnant of Rathke’s pouch in sella turcica.

Clinically often presents with headache/vomiting, visual disturbances due to the compression of the optic nerves or the optic chiasm (bitemporal hemianopia, optic atrophy, visual loss)
About 50% of childhood craniopharyngiomas extend upward into the third ventricle and result in hydrocephalus.
Bimodal, age 10-14 yo
Endocrine: growth hormone deficiency (75%); thyroid-stimulating hormone deficiency (25-64%); adrenocorticotropic hormone deficiency (25-56%); luteinising hormone or follicle-stimulating hormone deficiency (40-44%); diabetes insipidus (9-17%, but almost 100% post-op).
MRI: cystic. most calcified. suprasellar location

121
Q

cytarabine syndrome

A

fever
malaise
myalgia/arthralgia
after cytarabine

122
Q

Subependymal giant cell astrocytomas (SEGA) are associated with …

A

tuberus sclerosis

bengin, slow growing, arise in wall of lateral ventricles
Treated with mTOR inhibitors (eg everolimus) if unresectable

123
Q

Klinefelter syndrome associated with which tumor

A

Extragonadal germ cell tumors (esp mediastinal)
Breast cancer

124
Q

Turners syndrome associated with which tumor

A

Gonadoblastoma

125
Q

NF1 asssociated with which tumors

A

Neurofibromas (cutaneous, plexiform)
Optic pathway gliomas low-grade Other brain tumors- astrocytomas, brainstem gliomas, and high-grade gliomas
Malignant peripheral nerve sheath tumors
Rhabdomyosarcoma

126
Q

NF2 associated with which tumors

A

Vestibular schwannomas
Meningiomas
Spinal ependymomas

127
Q

Posterior fossa syndrome

A

neurological changes 1-5 days after PF tumor resection

difficulty verbalising
irritability
nystagmus
mutism
emotional lability

mutism usually resolves but can take a year

128
Q

Diffuse midline glioma (DIPG)

A

most common high grade glioma in kids
usually pons/brainstem
Presentation: multiple CN palsies, raised ICP, cerebellar signs, long tract signs (hyperreflexia, increased tone, clonus, Babinski +, motor deficit)
Often have basilar artery encasement
Clinical + radiological disgnosis as cant biopsy a brainstem
Most have histone H3.3 mutation
Rx: palliative radiation
Prognosis: most survive <1 year. bad

129
Q

Intracranial germ cell tumors

A

Arise in suprasellar (pituitary, infundibulum) or pineal region
More common in Japanse
Presentation: pituitary:hormone deficiencies, DI
Pineal: usually malignant, short hx, raised ICP, diplopia, parinaud syndrome, hydrocephalus
Ix: AFP/bHCG (serum + CSF), MRI brain + spine
High AFP= non germinomatous germ cell tumor
Rx: chemo + radiation. germinomas are very radiosensitive. NGGCT- chemo + CS radiation.

Germinomas + teratomas: AFP + HCG neg
Yolk sac- AFP +
Choriocarcinoma: HCG +

130
Q

Bladder masses in children

A
  • Rhabromyosarcoma
  • Other bladder cancers very rare
  • Associated with haematuria, obstruction, urinary symptoms
  • Usually embryonal subtype which is a small round blue cell tumor
131
Q

what do you need to avoid in germline RB1 mutations

A

radiation/Xrays
due to high risk of other cancers

132
Q

osteochondroma

A

most common benign bone cyst
metaphysis of long bone/tendon insertion site
Bony, non painful mass
XRay- stalk or projection of bone

133
Q

osteoid osteoma

A

most commonly on legs
unremitting pain often worse at night and relieved by aspirin/NSAIDS
exam: limp, atrophy, weakness
xray: lucency surrounded by sclerotic bone

134
Q

osteoblastoma

A

benign, aggressive osteogenic bone lesions commonly found in the posterior elements of the spine. Patients typically present between ages 10 and 30 with regional pain with only partial response from NSAIDs.
Diagnosis is made radiographically by a characteristic lesion that is > 2 cm in diameter with a sclerotic margin and radiolucent nidus.

135
Q

Features of aspergillus infection

A

Can occur any time when severely immunosuppressed
Mostly LUNGS
- halo sign and air crescent sign on CT
Can also occur in sinuses, brain

Rx: voriconazole, caspofungin second line
Inherently resistant to fluconazole

136
Q

Features of candida infection

A

Mostly in neutropenic phase, or with prolonged antibiotics in leukemia/lymphoma
Mostly LIVER

137
Q

Promyelocytic leukaemia

A

T15,17 RaRa gene
Complications- differentiation syndrome from all trans retinoic acid
DIC

138
Q

presentation of Wilms tumor

A

Abdominal mass
Hematuria
HTN
fever

139
Q

Syndromes associated with Wilms tumor

A

Denys Drasha
WAGR
Fanconi anemia
BWS

140
Q

where does Wilms metastsise to ?

A

Lungs
IVC tumor thrombus via renal vein

141
Q

anti GD2 monoclonal antibody = DINUTUXIMAB

A

used for high risk neuroblastoma
Kills neuroblasts
Antibodies bind to antigens on neuroblastoma cells –> active killing of antibody bound tumor cells by NK cells and macrophages

GD2 also found on peripheral + central nerves and skin
Severe pain when giving this drug- need concurrent morphine infusionAcute toxicity can present with neuropathic pain, hypotension, hypoxia, fever, capillary leak syndrome and hypersensitivity reactions.

142
Q

Which of the following is the most common long-term complication of cranial irradiation?

A

GH deficiency

143
Q

acute Graft versus Host

A

Present within 100 days post HSCT and with classic features of skin involvement (maculopapular rash), gastrointestinal involvement (diarrhoea and abdominal pain) and/or liver involvement (rising serum transaminases and bilirubin).

can do a skin biopsy if diagnosis unclear

144
Q

Etoposide

A

Topoisomerase inhibitor
inhibits the enzyme topoisomerase II, which unwinds DNA, and by doing so causes DNA strands to break.
Work in S and G2 phase

145
Q

which tumor is associated with coagulopathy and thrombosis

A

Wilms tumor

146
Q

posterior reversible encephalopathy syndrome (PRES)

A

hemotherapeutic agents and hypertension are both risk factors for PRES. Vision loss suggests the occipital lobes are involved. The lack of diffusion restriction indicates vasogenic oedema rather than cytotoxic oedema that is seen in watershed infarcts.

147
Q

most common cause of fungal meningitis in immunocompramised patients

A

cryptococcus neoformans

india ink stain - cells w surrounding halo

148
Q

Blinutumomab

A

anti-CD19+ and CD-3+ monoclonal antibody with bi-specific T-Cell engagers (BITE) that is used to treat B-cell acute lymphoblastic leukaemia

149
Q

Brentuximab

A

anti-CD30+ monoclonal antibody indicated for treatment of CD30+ lymphomas.

150
Q

Which genetic marker is the strongest prognostic factor in predicting overall survival in patients with neuroblastoma

A

MYCN amplification

151
Q

cytokine release syndrome

A

systemic response caused by the release of cytokines as a response to some form of immunotherapy. The exact pathophysiology is unknown, but symptoms can range from mild (fever, arthralgia, nausea) to severe (acute renal failure, seizures and disseminated intravascular coagulopathy). There is a risk of Cytokine Release Syndrome in any form of immunotherapy, but it occurs most commonly in CAR-T cell therapy (for B-ALL). The current theory is that CAR-T cells produce interleukin-1 and interleukin-6 perpetuating a pro-inflammatory response. Monoclonal antibodies and immune checkpoint inhibitors are all forms of immunotherapy and therefore have the same risks.

Tocilizumab (interleukin-6 antagonist) is used to treat cytokine release syndrome.

152
Q

Auer rods seen in which cancer type

A

AML

Immunohistochemistry is negative for CD3 and CD19 in AML. Myeloblasts will most commonly stain positive with myeloperoxidase

153
Q

B-ALL

A

B-lineage lymphoblasts are positive for cell marker CD19 and cytoplasmic markers CD79a and CD22. Although these markers on their own is not specific for B-ALL, high intensity positivity strongly supports the diagnosis. B-ALL must be negative for CD3 and negative on myeloperoxidase staining.

154
Q

Differentiation syndrome

A

Diagnosis of Differentiation Syndrome is by the presence of three of more of the following features in the absence of another explanation:
Fever ≥38° C
Weight gain >5 kg
Hypotension
Dyspnoea
Radiographic opacities
Pleural or pericardial effusion
Acute renal failure

155
Q

Drugs that cause minimal/no myelosuppression

A

○ vinca alkaloids
○ enzymes (asparaginase)
○ bleomycin
steroids

156
Q

which chemo agents are given intrathecally

A

MTX
cytarabine

RACP paeds Qld Group (46 decks)

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