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Haem/Onc > Neuroblastoma, ASPHO > Flashcards

Neuroblastoma, ASPHO Flashcards

1
Q

NBL originates from wehre

A

neural crest tissue of the sympathetic nervous system

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2
Q

spectrum of neuroblastic tumours?

A

ganglioneuroma>ganligoneuroblastoma (nodular vs. intermixed…intermixed= favourable)>NBL

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3
Q

2 features of NBL on path

A

SRBCT

homer-wright rosette pattern= tumour cells around neuropil

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4
Q

3 drivers of disease in NBL

A

NYMC amplificaiton/overexpression
ATRX inactivation
TERT rearrangements

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5
Q

chrom changes in NBL and outcome?

A

hyperdiploid= excellent outcome

segemental chrom aberrations/diploid DNA content= inferior outcome

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6
Q

most common somatic chrom aberrations/loss of genetic material in NBL?

A

chrom 1p36 (MYCNA); 11q23 (TERT, ATRX)

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7
Q

other somatic mutations in NBL?

A

aberations and activating mtuations of ALK; ch17q gain; ARID1a/1b deletions and point mutations, p53 mutations, PTPN11 (noonan), mtuations in genes of Ras/MAPK signaling pathway

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8
Q

msot common extracranial solid tumour in childhood?

A

neuroblastic tumours

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9
Q

sex predom in neuroblastic tumours?

A

male

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10
Q

neuroblastic tumour= most common solid tumour in kids __ __ __

A

under 1 year

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11
Q

CPSs in NBL?

A

familail NBL (alk germline mutation)
PHOX2B…neurocristopahty syndrome: central hypovent, hirschsprung’s, nbl
-Neurofibromatosis
-BWS
-Li Frameni
-Noonan
-germline deleation at 1p36 o 11q14 locus

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12
Q

prog features of NBL?

A

age <1 = BETTER
stage
path
presence of unfavourabe geentic changes (Mycn, alk, atrx, segmental chrom aberration/diploid)

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13
Q

old international NBL staging system based on what?

A

surgical resection

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14
Q

waht staging system do we use now for NBL? what does it indicate?

A

INRGSS=international nbl risk group staging system…based on pre-op features

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15
Q

what are the diff stages of INRGSS?

A

L1, L2, M, MS

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16
Q

Stage L1=?

A

lcoalized tumour not involving vital structures (no image defined risk factors) adn confied to one body compartmetn

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17
Q

stage L2=?

A

locoregional tumour with 1 or more IDRFs

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18
Q

stage M=?

A

distants mets (except MS)

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19
Q

stage MS?

A

mets in kids<18 months with disease confined to skin, liver, and/or BM

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20
Q

waht are the image defined risk factors?

A
  • tumor encases vessels in neck/thorax/abdo
  • encasing brachial plexus
  • extending across compartments
  • invading porta hepatis or renal pedicle
  • infiltrating organs
  • compressing spinal cord
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21
Q

what is the international neuroblastoma pathology classification?

A

INPC; determines if histo features are favourable or not favourable, priro to therapy

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22
Q

waht does the INPC look at?

A

amnt of Schwannian stroma
degree of tumour NBL maturation
mitosis-karyorrhexis index= MKI

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23
Q

INPC evaluated within context of what?

A

age…<18 mos best…>5 yrs worst

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24
Q

INPC = sep prog factor…how do neuronal diff and MKI fit in?

A 
Bad= less diff
Bad= high MKI
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25
Q

who qualitifes as low risk disease? (4)

A
  • INSS stage 1, 2, 4s
  • INRGSS L1, MS
  • Any age for 4S; MS<18 months
  • No MYCN amplification
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26
Q

who qualifies for HR disease?

A
  • MYCN amplificaiton
  • INSS stage 4
  • INRGSS M AND ageat least 18 months, or 12-18 months iwth unfavouragle histo or genomics
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27
Q

intermed diseaes?

A

INSS stage 3, INRGSS L2
INSS stage 4, INRGSS M AND age<18 mos
No Myc N amp

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28
Q

NBL accounts for what % of childhood caner mort?

A

10%

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29
Q

Inernational NBL risk group is a ___ classification schema

A

pretreatment

30
Q

features of pres in NBL?

A

-neck mass
-abdo dist
-horner syndrome
-GU symptoms
-paralysis for SC compression
-htn
features if mets:
-constittuional sx
-boney pain
-proptosis if mets at orbit
-racoon eyes
-resp distress
-skin nodules

31
Q

paraneoplastic syndromes in NBL?

A

opsoclonus myoclonus ataxia syndorme (OMAS)

Kerner morriosn syndrome

32
Q

features of OMAS?

A
  • myoclonic jerking and random eye movment, with or without cerebellar ataxia
  • associatd with favourable NB features
  • can have longeterm neruo and cog defs
33
Q

describe kerner-morrison syndrome

A

-tumour secretes VIP–> secretory diarrhea that rsolves after tumour resection

34
Q

staging for NBL?

A

CT/MRI of piramry
I-123 MIBG to look for mets
FDG-PET if tumour NOT MIBG-avid (10%)
—Tc99 bone scan = outdated

35
Q

work up for NBL?

A

tumour bx
bilateral BMA, bx
serum or urine catehcoalmines: HVA, VMA…secreted in 90%
nonspecfici markers: NSE, ferritin, LDH

36
Q

immunohistochem for NBL

A

S100, CD56, PGP9.5, NSE, synaptophysin

37
Q

EFS/OS for LR NBL?

A

> 90% for both

38
Q

how to tx LR NBL?

A
  • mainstay of tx= surgery…dealyed surgery for symptomatic 4S/MS…complete resesction NOT mandatory
  • OR, can do observation alone without bx! if inant with small stage 1/L1 adrenal tumour; ongiong trials to assess observation for additional non-adrenal small L1 tumours
  • chemo if acute life/organ threatening sx or recurrence
  • rads if acute life/organ threatening sx
39
Q

Intermed risk NBL: EFS and OS?

A

> 85% EFS

>90% OS

40
Q

how to tx intermed risk NBL?

A
  • can be surgery alone, chemo alone, or both
  • SURGERY upfront biopsy for genetic and histo..deleayed resection to achieve at least 50% reduction in primary tumour…complete resection NOT mandatory
  • response-directed chemo (2-8 cycles) of Vcr, carboplatin, cyclophos, doxorubicin, with goal of reducing primary tumour by at least 50% and resolving mets…longer chemo duration if unfavorable histo, tumour SCA or tumour diploid (at lesat 4, up to 8 cycldes if combined with stage 4/INRGSS M disease)
  • radiation only for acute life/organ threatneing disease
41
Q

Main parts of HR NBL tx?

A

INduction
Consolidation
Post-consolidatoin

42
Q

What happens during indcution for HR NBL?

A

-multi-agent chemo x 5 cycles
-stem cell collection (after cycle #2)
-surgery (usually between cycles 4-5)
in some cases, no surg

43
Q

what happens during HR NBL consoldiation?

A

tandem HD chemo adn auto transplant; external beam radiotherapy

44
Q

what happens during post-consolidation NBL tx?

A

Isotretinoin, anti-GD2 immunotherpay

45
Q

what is isotretinoin?

A

Retinoic acid, biologic response modifier

46
Q

CCG 3891 showed?

A

1) pts randomized to receiving RA had improved EFS to those not receiving it
2) also randomized continuation chemo after induction vs. 1 round of MA chemo adn SCT…3 yr EFS: 33% for MA chemo vs. 22% for continuation chemo–> standard of care MA chemo

47
Q

ANBL0032 showed?

A

randomized to anti-GD2 with (dinutuxuimab) IL2, GMCSF with cis-RA, or JUST cis-RA alone after consolidation: 2 yr EFS better with anti-GD2 of 66% vs. 46%….since then, found IL2 doesn’t improve outcomes but does increase toxicity..don’t give IL2 anymore

48
Q

ANBL 0532 did what?

A

randomized single round of MA chemo and transplant vs tandem…3 yrs EFS 73% for tandem vs 54% for single, most evident for kids who got immunotherapy

49
Q

goal for LR/IR NBL?

A

goal to reduce pirmary tumour by 50%…via chemo alone or surg alone or both

50
Q

goal for HR NBL?

A

compelte resection of primary tumour while preserving critical organs and structures

51
Q

do we think of neg margins in NBL?

A

not really

52
Q

role of rads in NBL and timing?

A

LR/IR: only for life/organ threatening symptoms

HR: 2160 cGy to primary tumour to pre-surgical tumour and margin..boost to residual gross tumour NOT found to increase local control…can irrad met soft tissue and bone sites…timing is after tandem ASCT

53
Q

revised international nbl rsponse criteria: based on what 3 components? does it include VMA/HVA levels?

A

-response of primary
-response of met sites (soft tissue and bone)
-bone marrow response
uses MIGB, PET

….no

54
Q

responses options for INRC overall resposne?

A

Complete response, partial, minor, stable disease, progressive disease…pretty much base overall rating on worst response for any given component…but minor response = at least 1 PR or CR and at least 1 SD (with no progressive disease)

55
Q

list 5 complciations /late effects of NBL treatment

A
  • renal damage from surgery
  • neural damage from surgery
  • altered msk growth from rads
  • infert from rads
  • secondary malig from rads
  • organ dysfunction from rads (renal, heart, lungs, pituitary, neurocog, etc)
  • otoxicity from platinum
  • cardio tox from athracyclin
  • infert from alkylator
  • secondary malig from alkylator, etoposide
  • decreased bone density from isotretinoin
56
Q

pheochromocytoma and paraganglioma: describe origin

A

neuroendocrine tumours arising from chromaffin cells of either sympathetic tissue of adrenal or extraadrenal abdo locations or parasympathetic tissue in the thorax or head and neck

57
Q

% of pheochromocytomas that are malig?

A

10%

58
Q

% of paragangliomas that are malig?

A

25%

59
Q

subtypes of pheochromocytoma and paraganglioma?

A
  • pseudohypoxia
  • wnt-signaling
  • kinase-signaling pathway (PI3kinase/AKT, RAS/RAF/ERK, mTOR)
60
Q

pseudohypoxia phenotype of PPGL–> what subtype? associated with what germline mut?

A

noradrenergic subtype; von Hippel-Lindau…get elevated norepi/normetanephrine

61
Q

kinase singaling tumour associated with which phenotype? germine mut?

A

adrenergic, secrete metaneprhine…germline mut in FET, NF1, TMEM127

62
Q

dopaminergic phenotype related to germline muts where? tumours are were?

A

SDHB and SDHD, secrete dopamine, head and neck tumours

63
Q

PPGLs: if you have a patient with this, should do what?

A

refer to cancer genetics!

64
Q

RFs for met disease in PPGLs?

A
  • SDHB germline mut
  • extra-adrenal location
  • primary>5 cm (or >3.5 cm in tumours related to SDHB)
  • young age of initial dx
  • elevated 3-methoxytyramine (MT) levels
65
Q

PPGLs: dx how?

A
  • excessive catecholamines
  • lcoalization of tumour by CT or MRI
  • measure metaneprhines (normetanephrine and metaneprhine separately measured) in urine or plasma
  • F-FDOPA or Ga-DOTATE PET (somatstatin analog)…or FDG-PET
66
Q

PPGLs met where?

A

lungs, liver, bones, LN

67
Q

role of MIGB-SPECT in PPGLs?

A

if considering MIBG therapy for met diseaes

68
Q

PPGLs pres?

A 
pain
nerve palsies
headache (90%)
diaphoresis
tachycarida
htn
hypotension/postural hypotension/alternating periods of high and low BP
anxiety, panic attacks
69
Q

tx localized PPGL how?

A

surgical resection]

-alpha blockade BEFORE beta blockage prior to surgery

70
Q

tx metastatic PPGL how?

A
  • poor prog if not resectable or mets
  • chemo: cyclophos, vcr, dacarbazine
  • sunitinib= TKI and everolimus= mTOR inhibitor
71
Q

radioistope therapy for NBL and PPGLs?

A
  • 131I-MIBG for relapsed and refractory NBL and PPGLs

- Lu 177 Dotatate = oral, no admission needed, radiolabeled somatostatin analog

Haem/Onc (30 decks)

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