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Haem/Onc > Hepatic tumours, ASPHO > Flashcards

Hepatic tumours, ASPHO Flashcards

1
Q

liver tumours = what percent of tumours in kids?

A

1%

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2
Q

median age of liver tumours in kids?

A

19 mos…80% under 15 yrs

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3
Q

hepatoblastoma = ___ most common intra-abdo tumour in kids

A

3rd

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4
Q

incidence of hepatoblastoma?

A

11.2 per million

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5
Q

91% of hepatoblastoma cases occur before what age?

A

5

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6
Q

liver malig in adolescents?

A

hepatocellular carcinoma; >95% of liver tumours in people over age 15

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7
Q

hepatocellular ca is more common where? why?

A

asia and africa; endemic hep b

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8
Q

DDX for benign liver tumours in infancy? (3)

A

hemangioendothelioma, mesenchymal hamartoma, teratoma

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9
Q

ddx for malig liver tumours in infants? (4)

A

HEPATOBLASTOMA, rhabdoid tumour, yolk sac tumour, LCH, M7 AML, disseminated NBL

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10
Q

ddx of benign liver tumours age 1-3?

A

hemangioendothelioma, mesenchymal harmatoma

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11
Q

ddx for malig liver tumours age 1-3?

A

HBL, rhabdomyosarcoma, inflammatory myofibroblastic tumour

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12
Q

ddx for benign liver tumour age 3-10? (1)

A

perivascular epithelioid cell tumours (PE-comas)

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13
Q

ddx for malig liver tumours age 3-10? (4)

A

hepatocellular carcinoma, embryonal sarcoma, angiosarcoma, cholangiocarcinoma, endocrine (gastrin) carcinoma

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14
Q

ddx for benign liver tumours age 10-16? (3)

A

adenoma, focal nodular hyperplasia, biliary cystadenoma

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15
Q

ddx for malig liver tumours in age 10-16? (3)

A

conventional and fibrolamellar hepatocellular carcinoma, hodgkin lymphoma, leiomyosarcoma

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16
Q

describe bio of HBL

A
  • activation of WNT pathway, including activating mutations of CTNNB1 (or APC) in >90% of cases; upregulation of Wnt signaling pathway genes, like DKK1, APCDD1, AXIN2, CCND1
  • NFE2L2 (NRF2) mutation in 5%
  • Other alterations= mutations in epigenetic modifiers like MLL2, ARID1a or variable expression of Lin-28, AFP, Notch
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17
Q

give 5 congenital risk factors for HBL

A

“Genetics For Hepatic Glitches at Birth”

  • familial polyposis
  • Gardner syndrome
  • Beckwith-Wiedemann Syndrome
  • Hemihypertrophy
  • Glycogen storage disease
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18
Q

Give 5 enviornemntal RFs for HBL

A

“Other Features Predisposing GI Malignant Conditions”

  • Fetal alcohol syndrome
  • Prematurity and LBW
  • Oral contraceptives
  • Oral gonadotropins
  • Parental exposure to metal/petroleum/paint
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19
Q

BWS: 1) involves what chrom? 2)incidence?

3) give 5 features

A

1) 11p15.5
2) 1 in 10k
3) abdo wall defects, macroglossia, GU malformations, organomegaly, hyperinsulinemic hypoglycemia

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20
Q

BWS: malig risk in first decade of life?

A

8-10%

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21
Q

Give 5 tumours associated with BWS and the incidence of the 2 most common

A 
Wilms: 43%
HBL: 20%
Adrenocortical tumours (7%)
NBL
RMS
Pancreatoblastoma
Leukemia
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22
Q

Hemihypertrophy incidence?

A

1 in 86k

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23
Q

Hemihypertrophy can be ISOLATED or associated with which syndromes (give 3)?

A

BWS
Klippel-Trenaunay-Weber
McCune-Albright Syndrome

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24
Q

Malignancy risk with hemihypertrophy?…give 3 tumours that are associated

A

5%

Wilms, HBL, adrenocortical tumours

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25
Q

HBL: see disprorotionate increase in HBL with BW< what?

A

2500 g

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26
Q

which germline mutation is associated with familial adenomatous polyposis and gardner syndrome?

A

APC mutation

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27
Q

other than increased risk of HBL, 3 features/tumours associated with FAP?

A

gastric tumours, desmoids, congenital hypertrophy of retinal pigment epithelium

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28
Q

feature of gardner syndrome?

A

osteomas

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29
Q

what % of HBL cases have FAP syndrome?

A

5-10%! important to do fam hx!…APC testing recommended in sporadic HBL!

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30
Q

if no FAP, what’s risk of HBL? If FAP?

A

1 in 100k…1 in 250

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31
Q

presentation of HBL?

A

-asymptomatic abdo mass
-wt loss, anorexia, emesis, abdo pain (advanced disease)
=elevated AFP in 80-90%
-distant mostly mets in 20% (mostly to lung)
-thrombocytosis (HB cells secrete IL-1B–> induces fibroblasts and endothelial cells to produce IL-6–> hepatocyte GF secretion adn thrombopoietin secretion
-htn (rare) if secrete renin
-precocious puberty (rare) if secrete beta-hcg

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32
Q

where does HBL met?

A

LUNG=#1

intraperitoneal, LN, brain, tumour thrombus

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33
Q

evaluation for HBL?

A
  • US with doppler’
  • CT C/A/P and MRI
  • AFP
  • biopsy
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34
Q

half life of AFP?

A

5-7 days

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35
Q

if doing needle biopsy for HBL, what’s recommended?

A

MULTIPLE passes because multiple histologic types can be present and intermingled

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36
Q

Types of hbl histo and incidence?

A

Epithelial 85-90%
pure fetal histology 7-10%
small cell undifferentiated histo 5%

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37
Q

epithelial histo in HBL includes what?

A

fetal + embryonal

38
Q

prog in pure fetal histo for HBL?

A

excellent outcome! if stage 1, may only need surgery for cure

39
Q

small cell undifferentiated histo in HBL: outcome?

A

very poor

40
Q

small cell undifferentiated histo in HBL: AFP tends to be high or low?

A

LOW…<100 ng/ml

41
Q

small cell undifferentiated histo associated with what genetic change?

A

22q11 aberations (hSNF5/INI1 gene)= malig rhabdoid…get NEGATIVE INI1 immunostain

42
Q

AFP high in what % of HBL? what % of HCC?

A

80-90% of HBL

60-70% of HCC

43
Q

Other than HBL and HCC, AFP also high in what 2 tumours? also mildly elevated in what 2 benign tumours?

A

germ cell tumours (liver primary), pancreatoblastoma….infantile hemangioendothelioma, mesenchymal hamartoma

44
Q

in what variant of HCC is AFP low or normal?

A

fibrolamellar variant

45
Q

In HBL, LOW AFP (<100 ng/ml) is associated with ___ prog and wiht ___ ___ ___ histo

A

worse; small cell undifferentiated

46
Q

surgical criteria for staging HBL?

A 
1= Complete resection
2= microscopic margins
3= gross residual or nodal involvement 
4= met disease
47
Q

SIOPEL staging based on what? (for preop chemo in all pts)

A

radiographic criteria; PRETreatment EXTent of disease

48
Q

Describe PRETEXT staging

A

PRETEXT 1: 3 contiguous sectors free

2: 2 continguous sectors free
3: 1 contiguous sector free
4: all 4 sectors involved:(

49
Q

what 2 things determine HBL tx?

A

stage and path

50
Q

what is the mainstay of curative therapy in HBL?

A

surgical resection

51
Q

what % of pts with HBL have resectable disease at presentation?

A

20-30%

52
Q

surgery ONLY is curative for what % of pure fetal HBL?

A

> 90%

53
Q

Is HBL considered to be very chemosensitive?

A

YES

54
Q

HBL chemo is ___-based

A

cisplatin

55
Q

in HBL, chemo increases the proportion of ___ ___ ___ ___

A

pts achieving complete resection

56
Q

in HBL, chemo is used in what settings?

A

both neoadjuvant and adjuvant

57
Q

is radiation indicated in upfront tx of HBL?

A

NO

58
Q

COG chemo for HBL?

A

Cisplatin + 5FU+ Vcr (c5v) +/- dox (C5VD)

59
Q

what does SIOPEL (europe) use to tx hbl?

A

cisplatin alone or with doxo (PLADO)

60
Q

what additional agents are used for tx for high risk/met HBL pts?

A

irinotecan, carboplatin, etoposide

61
Q

how do COG and siopel approaches to HBL difer?

A

COG: surgery when feasbile, followed by chemo
SIOPEL: always preop chemo

62
Q

what is the AHEP1531 trial?

A

HBL trial with consensus COG/SIOPEL approach using both regimens

63
Q

Define Low Risk, Intermed Risk, High Risk HBL

A

Low Risk: Stage 1 and non-small cell undifferentiated
Intermed: Stage 1 and 2 small cell undiff + stage 3
High riskL stage 4 and all pts with AFP<100

64
Q

treatment for low risk HBL?

A

= stage 1 and 2 (non SCU): give upfront surgery adn adjuvant chemo (2-4 cycles of CDDP=cisplatin- based therapy)

65
Q

treatment for intermed risk HBL?

A

= stage 3= early transplant evaluation; preop chemo (2-4 cycles CDDP-based therapy)–> surgery–> postop chemo (2-4 cycles of CDDP based therapy)

66
Q

high risk HBL tx?

A

= stage 4 and any patients with AFP<100 ….early transplant eval, preop chemo (2-4 cycles of cisplat-based hterapy)–> surgery–> postop chemo (2-4 cycles of high risk CDDP based therapy)

67
Q

outcome for low risk HBL?

A

90%+ survival; pure fetal histo curable with surgery only!

68
Q

intermed risk HBL: outcome?

A

70-90% survival

69
Q

high risk HBL: outcome?

A

40-60% survival

70
Q

what % of HBL pts need transplant?

A

10%

71
Q

who needs liver transplant with HBL?

A
  • unifocal PRETEXT 4
  • multifocal PRETEXT 4
  • unifocal, centrally-located tumours involving main hilar structures or main hepatic veins
72
Q

if considering liver transplant, imp to do what?

A

refer early!!!!

73
Q

2 factors associated with improved survival in liver trasnplant HBL pts?

A

complete resection ASAP after induction therapy (4 cycles), avoiding excessive pre-transplant chemo & good response to chemo

74
Q

are lungs mets a contraindication to liver transplant in HBL?

A

no, can still transplant if the mets can be resected:)

75
Q

HCC: makes up ___ % of liver cancer in pts over the age of ___

A

95%; 15

76
Q

median age for hepatocellular ca?

A

12 yrs

77
Q

2 categories of hepatocellular ca?

A

1) in context of underlying liver disease

2) de novo

78
Q

what are the types of de novo HCC? 3

A
  • conventional HCC
  • fibrolamellar HCC
  • HCC with elements of HBL (transitional cell tumour or hepatocellular neoplasm otherwise not specified
79
Q

which signaling pathways are activated in HCC?

A

WNT/CTNNB1, EPHB2, TGFB/MTOR signaling pathways

80
Q

type of fusion seen in fibrolamellar HCC?

A

DNAJB1-PRKACA

81
Q

congenital RFs for HCC? 5

A

Hepatic Glitches At Birth: Hcc

  • Hereditary Tyrosinemia
  • Biliary cirrhosis
  • Glycogen storage diseases
  • alpha 1 antitrypsin def
  • Hemochromatosis
82
Q

envrionmental RFs for HCC? 5

A

crazy habits and alternative afflictions

hepatitis b and c
alcohol consumption
anabolic steroids
aflatoxin
carcinogens (pesticides, vinyl chloride, thorotrast)
83
Q

HCC: fibrolamellar= what % of cases?

A

25%

84
Q

HCC: fibrolamellar: AFP is __ to ___

A

low to normal (90%<20)

85
Q

HCC:
% with mets?
% with extrahepatic extension or vasc invasion?
% with multifocality?

A

30%; 40%; 50-60%

86
Q

what % of HCC is associated with liver cirrhosis?

A

30%

87
Q

response to chemo in HCC? add waht?

A

usually poor:( <50%…usually add sorafenib

88
Q

waht % of HCC pts can have complete resection? may use what? role of tarnsplnat?

A

only 30%…low:(…may use chemoembolization…almost always send pts to transplant, unless they have extrahepatic disease

89
Q

HCC: 5 yrs OS and EFS?

A

OS:28%; EFS 17-19%

90
Q

long term effects for pts with liver tumours?

A 
CHEMO-->
hearing loss
therapy-related AML
cardiotox
renal tox

SURGERY–>
post-transplant immuno supp

Also colon ca in APC+ pts

91
Q

surveillance guidelines for APC+ pts?

A

yearly sigmoidoscopy from age 13-15 onwards

ppx colectomy between ages 16-20

upper GI study every 3 years from age 30 to watch for small bowel ca (5-10% risk)

Haem/Onc (30 decks)

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