solids Flashcards
males get what kind of brain tumours
medulloblastoma and ependymoma
diencephalic syndrome =
FTT + emaciation despite normal caloric intake + hyper alert/happy/kinetic, a/w hypothal/thal
types of brain tumours
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
most common malignant brain tumour in kids
medulloblastoma, and 20% of all CNS tumours
what is posterior fossa syndrome?
Delayed neurological changes after PF tumour resection, 1-5 days post-op:
irritable, nystagmus, mutism
LGG vs HGG prognosis
LGG (I-II) 90% at 20y
HGG (III-IV) 30% at 3y
optic pathway + glioma =
juvenile astrocytomas
key features of LGG
BRAF pathway problem
Rosenthal fibres on histo
good outcome, better in NF1
most common type of LG astrocytoma
pilocytic - locally aggressive but thassabout it. cerebellum most common
Pilocytic astrocytoma + optic pathway = what condition
NF1
types of high grade astrocytoma (3)
WHO III – anaplastic astrocytoma
WHO IV – glioblastoma multiforme
DIPG
ependymoma - key features
- SLOW growing
- from ependymal lining of ventricular wall or from spinal canal
- 60% survival, worse if younger, localised to PF, or disseminated
PNET includes what cancers?
embryonal tumours = Primitive Neuroectodermal Tumour (PNET) inc. MB, ATRT. all grade IV WHO tumours.
why are many MBs metastatic at presentation
1/3 met at presentation bc they spread along the neuraxis
favourable and not favourable molecular subtyping for medulloblasoma
favourable = WNT = CTNNB1mutation, rarely mets
unfavourable = MYC+++
ATRT key features
S. Nardella:
- round blue cell on histo with loss of INI1 nuclear staining (SMARCB1)
- poor prognosis
sellar mass - what imaging modality?
MRI!
DDx for a sellar mass
- infection e.g. abscess
- cystic lesion
- pituitary: adenoma
- benign mass: craniopharyngioma, meningioma
- AV fistula
craniopharyngioma: key features
- 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)**
craniopharyngioma vs pituitary adenoma, and vs rathke’s cyst
PA:
- hyperprolactinaemia (cf CPh)
- cystic, but not calcified
Rathke cleft cyst:
- no solid /calcification
- most intrasellar
pituitary adenoma key features
a/w MEN1
ACTH > GH, PRL
most common extracranial solid tumour in children
NB
key features of NB
- 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)
NB a/w what syndromes??
BWS + hemihypertrophy
Turner
NF1
ROHHAD
familial NB a/w what mutation??
PHOX2B and ALK genes
horner syndrome and NB
ptosis, miosis, anhidrosis
DONT resolve with resection
what is ROHHAD?
rapid onset
obesity
hypothal dysfunction
hypoventilation
autonomic
dysregulation
Ptosis and periorbital ecchymoses = what?
orbital mets
specific Ix in NB
urine catechols (HVA/VMA)
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