solids Flashcards

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
Q

familial NB a/w what mutation??

A

PHOX2B and ALK genes

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26
Q

horner syndrome and NB

A

ptosis, miosis, anhidrosis
DONT resolve with resection

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27
Q

what is ROHHAD?

A

rapid onset
obesity
hypothal dysfunction
hypoventilation
autonomic
dysregulation

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28
Q

Ptosis and periorbital ecchymoses = what?

A

orbital mets

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29
Q

specific Ix in NB

A

urine catechols (HVA/VMA)

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30
Q

NB + opsoclonus + myoclonus =

A

opsoclonus (super chaotic non rhythmic movements)- myoclonus -ataxia:
- immune mediated
- actually a/w favourable tumour, but resection won’t improve OMA Sx

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31
Q

wilm’s tumour peak age

A

2-3y, rare >5y

32
Q

wilm’s a/w what syndromes

A
  1. BWS
  2. hemi-hypertrophy
  3. NF1
  4. overgrowth syndromes e.g. sotos
  5. WAGR
  6. denys-drash
33
Q

WAGR key features

A

Wilms tumour, aniridia, genitourinary abnormalities, and mental retardation (WAGR)
- Del 11p13

34
Q

most important predictor of Wilm’s prognosis

A

histology - anaplastic is bad

35
Q

which is worse in Wilm’s - anaplastic or bilat?

A

anaplastic worse- 50% 5y
bilat 70% 5y

36
Q

overall wilm’s survival

A

60%

37
Q

Most common solid renal tumour identified in the neonatal period /benign renal tumour

A

Mesoblastic nephroma, dx much earlier than WT

38
Q

intussception a/w what cancer

A

NHL

39
Q

key features of germ cell tumour

A

Girls: abdominal pain, vaginal bleeding
Boys: testicular mass, new onset hydrocele, sacrococcygeal mass/dimple

+ve bHCG/ AFP (in serum and CSF) and Asians

40
Q

wilm’s vs NB

A

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
Q

most soft tissue sarcomas are?

A

rhabdomyosarcoma

42
Q

rhabdomyosarcoma a/w what syndromes??

A

Li-Fraumeni
NF1

43
Q

two types of RMS, and which is better

A
  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
Q

RMS occurs where?

A

head and neck (40%), genitourinary tract (20%), extremities (20%), and sx come from obstruction/displacement

45
Q

FRACP: RMS and basically most other sarcomas get mets where

A

pulmonary = SOB, pain

46
Q

most common primary malignant bone tumour in kids

A

<10y: OS > Ewing’s
>10y: Ewings greater

47
Q

OS vs Ewings

A

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
Q

benign conditions with possible malignant transformation into OS

A

Paget disease, endochondromatosis, fibrous dysplasia

49
Q

what is the good kind of osteosarcoma?

A

parosteal - low grade, well differentiated, surgical intervention only usually

50
Q

most important prognostic factor of OS

A

histologic response to chemotherapy - necrosis bad after chemo

51
Q

most common benign lesion of bone

A

osteochondroma - usually metaphyseal in long bones

52
Q

RB - how common unilat vs bilat

A

unilat = 75%
bilat = 25%

53
Q

classification of RB

A
  1. hereditary: younger, multifocal, bilat
  2. sporadic: older, unifocal, unilat
54
Q

RB genetics

A

loss of function of RB1
two hit mutation
most germline, but some AD inherited

55
Q

histology in RB

A

biopsies CONTRAINDICATED

56
Q

poor prognostic factors of RB

A

metastatic/extra-ocular disease
trilateral

57
Q

most common cause of death for patients with heritable retinoblastoma is ?

A

secondary malignancy and not the initial primary retinoblastoma

58
Q

screening for children with germline mutation?

A

regular until 7yo

59
Q

what is trilateral RB?

A

pineal tumour + hereditary + bilateral retinoblastoma

60
Q

risk factors for germ cell tumours

A

Klinefelters!
T21
any undescended testes

61
Q

location of germ cell tumours

A

extra-gonadal - most sacrococcygeal, if mediastinal, think klinefelters

gonadal

62
Q

types of germ cell tumours we care about

A

1) gonadoblastoma (occurs with gonadal dysgenesis)
2) teratoma

63
Q

key features: teratoma

A

germ cell tumour with multiple cell types
usually in gonads, most common are ovarian, or sacrococcygeal

64
Q

which cancers commonly metastasise to the liver?

A

NB, Wilm’s, and lymphoma

65
Q

HB - a/w what syndromes??

A

FAP
BWS
hemihypertrophy
GSD1
Li-Fraumeni
Goldenhar
T21

66
Q

super rare hepatoblastoma paraneoplastic syndrome FRACP style

A

isosexual precocious puberty in boys from beta-hcg

67
Q

monitoring biochem marker for hepatoblastoma

A

AFP

68
Q

HCC … think - who could it happen to?

A

when your liver gets fucked up e.g. hep B/C, or diseases like galactossaemia, A1AT etc.

69
Q

hepatoblastoma vs HCC

A

HB:
<3y
a/w prematurity and genetics
platins + resections

HCC:
>3y
a/w liver injury

70
Q

thyroid nodules - bad or not?

A

75% are benign in kids

71
Q

classic risk factors for CRC in kids

A
  1. Lynch (HNPCC, AD) - also endometrial Ca, much fewer polyps cf FAP
  2. FAP (APC, AD) > 100 poylps
  3. polyposis
  4. Peutz-Jeghers
72
Q

BWS - three cancers NTBM

A
  1. Wilm’s
  2. HB
  3. NB
73
Q

Langerhans histiocytosis age group

A

1-3y

74
Q

what is LCH?

A

langerhans are APCs of the skin > overgrowth of these cells. LCH has differents recognised patterns of presentation.

75
Q

diff ways LCH can present

A
  1. bone 80% - lytic lesions
  2. skin 40% - eczematous / ulcerated / papules
  3. lymphadenopathy 20%
  4. liver/spleen
  5. CNS
  6. endo - esp. DI
76
Q

bad prognostic factor for NB

A

n-MYC