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solids Flashcards

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1
Q

males get what kind of brain tumours

A

medulloblastoma and ependymoma

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2
Q

diencephalic syndrome =

A

FTT + emaciation despite normal caloric intake + hyper alert/happy/kinetic, a/w hypothal/thal

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3
Q

types of brain tumours

A

Glial
1) astrocyte: astrocytoma/ ependydoma
2) oliodendrocyte

Non-glial
1) craniopharyngioma
2) germ cell
3) embryonal e.g. medulloblastoma, ATRT
4) other e.g. meningioma

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4
Q

most common malignant brain tumour in kids

A

medulloblastoma, and 20% of all CNS tumours

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5
Q

what is posterior fossa syndrome?

A

Delayed neurological changes after PF tumour resection, 1-5 days post-op:
irritable, nystagmus, mutism

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6
Q

LGG vs HGG prognosis

A

LGG (I-II) 90% at 20y
HGG (III-IV) 30% at 3y

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7
Q

optic pathway + glioma =

A

juvenile astrocytomas

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8
Q

key features of LGG

A

BRAF pathway problem
Rosenthal fibres on histo
good outcome, better in NF1

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9
Q

most common type of LG astrocytoma

A

pilocytic - locally aggressive but thassabout it. cerebellum most common

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10
Q

Pilocytic astrocytoma + optic pathway = what condition

A

NF1

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11
Q

types of high grade astrocytoma (3)

A

WHO III – anaplastic astrocytoma
WHO IV – glioblastoma multiforme
DIPG

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12
Q

ependymoma - key features

A
  • SLOW growing
  • from ependymal lining of ventricular wall or from spinal canal
  • 60% survival, worse if younger, localised to PF, or disseminated
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13
Q

PNET includes what cancers?

A

embryonal tumours = Primitive Neuroectodermal Tumour (PNET) inc. MB, ATRT. all grade IV WHO tumours.

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14
Q

why are many MBs metastatic at presentation

A

1/3 met at presentation bc they spread along the neuraxis

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15
Q

favourable and not favourable molecular subtyping for medulloblasoma

A

favourable = WNT = CTNNB1mutation, rarely mets

unfavourable = MYC+++

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16
Q

ATRT key features

A

S. Nardella:
- round blue cell on histo with loss of INI1 nuclear staining (SMARCB1)
- poor prognosis

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17
Q

sellar mass - what imaging modality?

A

MRI!

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18
Q

DDx for a sellar mass

A
  1. infection e.g. abscess
  2. cystic lesion
  3. pituitary: adenoma
  4. benign mass: craniopharyngioma, meningioma
  5. AV fistula
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19
Q

craniopharyngioma: key features

A
  • from Rathke’s pouch
  • peak 5-14y
  • 3rd most common (after MB, glioma)
  • MRI: cystic calcified parasellar lesion (from cholesterol crystals)
  • bitemporal hemianopia
  • endo problems ** (GH!!> GnRH >TSH> ACTH)**
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20
Q

craniopharyngioma vs pituitary adenoma, and vs rathke’s cyst

A

PA:
- hyperprolactinaemia (cf CPh)
- cystic, but not calcified

Rathke cleft cyst:
- no solid /calcification
- most intrasellar

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21
Q

pituitary adenoma key features

A

a/w MEN1
ACTH > GH, PRL

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22
Q

most common extracranial solid tumour in children

A

NB

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23
Q

key features of NB

A
  • from primordial neural crest cells
  • any site where the SNS is: esp. adrenals, abdomen
  • common met sites: long bones, skull
  • rare after 6y
  • sweating + HTN from catechols (NB HTN could be from RAS)
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24
Q

NB a/w what syndromes??

A

BWS + hemihypertrophy
Turner
NF1
ROHHAD

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25
familial NB a/w what mutation??
PHOX2B and ALK genes
26
horner syndrome and NB
ptosis, miosis, anhidrosis DONT resolve with resection
27
what is ROHHAD?
rapid onset obesity hypothal dysfunction hypoventilation autonomic dysregulation
28
Ptosis and periorbital ecchymoses = what?
orbital mets
29
specific Ix in NB
urine catechols (HVA/VMA)
30
NB + opsoclonus + myoclonus =
opsoclonus (super chaotic non rhythmic movements)- myoclonus -ataxia: - immune mediated - actually a/w favourable tumour, but resection won't improve OMA Sx
31
wilm's tumour peak age
2-3y, rare >5y
32
wilm's a/w what syndromes
1. BWS 2. hemi-hypertrophy 3. NF1 4. overgrowth syndromes e.g. sotos 5. WAGR 6. denys-drash
33
WAGR key features
Wilms tumour, aniridia, genitourinary abnormalities, and mental retardation (WAGR) - Del 11p13
34
most important predictor of Wilm's prognosis
histology - anaplastic is bad
35
which is worse in Wilm's - anaplastic or bilat?
anaplastic worse- 50% 5y bilat 70% 5y
36
overall wilm's survival
60%
37
Most common solid renal tumour identified in the neonatal period /benign renal tumour
Mesoblastic nephroma, dx much earlier than WT
38
intussception a/w what cancer
NHL
39
key features of germ cell tumour
Girls: abdominal pain, vaginal bleeding Boys: testicular mass, new onset hydrocele, sacrococcygeal mass/dimple +ve bHCG/ AFP (in serum and CSF) and Asians
40
wilm's vs NB
WT: - calcification uncommon - won't cross midline - well circumscribed - claw sign - invades renal vein, IVC - mets to lung, liver NB: - calcifies, crosses midline, mets to bone
41
most soft tissue sarcomas are?
rhabdomyosarcoma
42
rhabdomyosarcoma a/w what syndromes??
Li-Fraumeni NF1
43
two types of RMS, and which is better
1. embryonal 70% 2. alveolar 30% - older, more invasive, mets everywhere not just lung, metastatic disease is incurable - HAVE Translocations!! t(1;13) or t(2;13)
44
RMS occurs where?
head and neck (40%), genitourinary tract (20%), extremities (20%), and sx come from obstruction/displacement
45
FRACP: RMS and basically most other sarcomas get mets where
pulmonary = SOB, pain
46
most common primary malignant bone tumour in kids
<10y: OS > Ewing's >10y: Ewings greater
47
OS vs Ewings
OS: bone Only Metaphysis (O closer to M) sclerotic, sunburst on XR, Codman triangle a/w RB, P53 (Li-Fraumeni) treatment - NO RT ES: bone AND soft tissue primary Diaphysis (E closer to D) lytic on XR with onion skins! t(11;22) in 90%!! CAN get RT often a/w constitutional Sx
48
benign conditions with possible malignant transformation into OS
Paget disease, endochondromatosis, fibrous dysplasia
49
what is the good kind of osteosarcoma?
parosteal - low grade, well differentiated, surgical intervention only usually
50
most important prognostic factor of OS
histologic response to chemotherapy - necrosis bad after chemo
51
most common benign lesion of bone
osteochondroma - usually metaphyseal in long bones
52
RB - how common unilat vs bilat
unilat = 75% bilat = 25%
53
classification of RB
1. hereditary: younger, multifocal, bilat 2. sporadic: older, unifocal, unilat
54
RB genetics
loss of function of RB1 two hit mutation most germline, but some AD inherited
55
histology in RB
biopsies CONTRAINDICATED
56
poor prognostic factors of RB
metastatic/extra-ocular disease trilateral
57
most common cause of death for patients with heritable retinoblastoma is ?
secondary malignancy and not the initial primary retinoblastoma
58
screening for children with germline mutation?
regular until 7yo
59
what is trilateral RB?
pineal tumour + hereditary + bilateral retinoblastoma
60
risk factors for germ cell tumours
Klinefelters! T21 any undescended testes
61
location of germ cell tumours
extra-gonadal - most sacrococcygeal, if mediastinal, think klinefelters gonadal
62
types of germ cell tumours we care about
1) gonadoblastoma (occurs with gonadal dysgenesis) 2) teratoma
63
key features: teratoma
germ cell tumour with multiple cell types usually in gonads, most common are ovarian, or sacrococcygeal
64
which cancers commonly metastasise to the liver?
NB, Wilm’s, and lymphoma
65
HB - a/w what syndromes??
FAP BWS hemihypertrophy GSD1 Li-Fraumeni Goldenhar T21
66
super rare hepatoblastoma paraneoplastic syndrome FRACP style
isosexual precocious puberty in boys from beta-hcg
67
monitoring biochem marker for hepatoblastoma
AFP
68
HCC ... think - who could it happen to?
when your liver gets fucked up e.g. hep B/C, or diseases like galactossaemia, A1AT etc.
69
hepatoblastoma vs HCC
HB: <3y a/w prematurity and genetics platins + resections HCC: >3y a/w liver injury
70
thyroid nodules - bad or not?
75% are benign in kids
71
classic risk factors for CRC in kids
1. Lynch (HNPCC, AD) - also ***endometrial Ca***, much fewer polyps cf FAP 2. FAP (APC, AD) > 100 poylps 3. polyposis 4. Peutz-Jeghers
72
BWS - three cancers NTBM
1. Wilm's 2. HB 3. NB
73
Langerhans histiocytosis age group
1-3y
74
what is LCH?
langerhans are APCs of the skin > overgrowth of these cells. LCH has differents recognised patterns of presentation.
75
diff ways LCH can present
1. bone 80% - lytic lesions 2. skin 40% - eczematous / ulcerated / papules 3. lymphadenopathy 20% 4. liver/spleen 5. CNS 6. endo - esp. DI
76
bad prognostic factor for NB
n-MYC

Oncology (4 decks)

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