MSK rheumatology summary Flashcards
autoantibody for mixed CTDs
anti RNP
are ganlion cysts true cysts? why?
no epithelial lining
histology of synovial cysts
space with myxoid material
which drugs may cause dupytrens
anticonvulsants
features of embryonal rhabdomyosarcoma
cildhood
central tract, GI tract, GU tract, H+N, common bile duct
deletion xp11,15
features of alveolar skeletal muscle tumours
young adults
many sites eg H+ N
PAX translocation (t2,5)
features of pleomorphic skeletal muscle tumour
rarest form of older age group
IHC - myogi, myogenin
what is the difference between liposarcoma and lipoma
lipoblasts (purple blobs) in liposarcoma
who are rhabdomyosarcomas found in
children
where are leiomyomas found?
uterus
what is an osteoma
simple - cranial bones
multiple - gardners syndrome
paediatric leisons - nidus
what is an osteosarcoma
childrens long bones
codmans triangle
any malignant osteoid producing tumour is osteosarcoma until proven otherwise
what is ewings sarcoma
translocation of chromosomes 11+12
small, round, blue cells
describe nodular fascitis
a rare, non-cancerous tumour of soft tissue which mimics cancer cells
which cells surround the terminal bouton?
schwann cells
what are the 5 key steps of neuromuscular transmission?
- synthesis of ACH in cytoplasm of bouton
- uptake of ACH into synaptic vesicles for concentration + storage
- Ca+ dependent release of ACH INto synaptic cleft by exocytosis
- brief activation of nicrotinic ACH receptors by reversible binding of ACH
- rapid transmission of neurotransmittion action by ACHe within synaptic cleft
what is the role of ACHe?
it hydrolyses ACh to choline + acetate
- choline is taken up by choline transporter
- acetate diffuses from synaptic cleft
what is neuromytonia / issacs syndrome?
cramping, stiffness + muscle twitches
prolonged epp
how to treat isaacs syndrome?
carbimazepine (blocks voltage gates Na+ channels)
what is lambert eaton myasthenia syndrome?
muscle weakness in limbs
associated with SCLC
decreased release of ACH
how to treat lambert eaton mtasthenia syndrome?
pyridostigmine (increases duration of action of ACH in synaptic cleft + K+ channel blockers
what is myasthenia gravis
increasing muscle weakness during periods of activity
weakness in eyelid and muscles
decreased epp
how to treat myasthenia gravis
pyridostigmine
what it botulinum toxin
irreversively inhibits ACh release
used to remove wrinkes
what is an osteochondroma
most common cartilaginous outgrowth local pain 1% chance of malignancy multiple = MEN
what is an enchondroma
intramedullary
metaphyseal cartilaginous tumour
what causes enchondroma
failure of normal enchondral ossification at the growth plate (growing cartilage is replaced by bone)
what does enchondroma look like
a leucent lesions which can undergo mineralisation –> patchy sclerotic appearance
what is a simple bone cyst
fluid fillled cyst in bone
growth defect from physis
where are simple bone cysts usually seen?
proximal humerus and femur
what is a giant cell tumour
locally agressive / painful
involves epiphysis
where are giant cell tumours most commonly seen
knee and distal radius
soap bubble appearance
what is the met stat for giant cell tumor?
5% met to lung
how to treat giant cell tumour
intralesional excision with phenol, bone cement or liquid nitrogen to destroy remaining tumour
what if fibrous dysplasia?
genetic mutation which causes lesions of fibrotic tissue + immature bone
what is xray sign for fibrous dysplasia?
extensive involvement of femur = shephards crook deformity
what does a bone scan show in fibrous dysplasia?
increased uptake during development but usually becomes inactive
what is the treatment for fibrous dysplasia?
bisphosphonates
what is osteoid osteoma?
small nidus of immature bone surrounded by an intense sclerotid halo
features of osteoid osteoma?
intense constant pain which is worse at night
NSAIDS
what is an osteosarcoma?
produced bone in adolescents
60% around knee
met spread - haematogenous