Pathology & Tumour Flashcards
Causes of vertebral plana?
EG
Infection - especially TB
Ewing’s sarcoma
Metastatic tumour
What are the three forms of Langerhan’s Cell Histiocytosis?
- EG (localised form) 70%
- Hand-Schuller-Christian disease (chronic recurring form) 20%
- Lettered-Siwe disease (fulminant form) 10%
Differential diagnosis of osteosarcoma?
Osteomyelitis
Ewings
Trauma
Myosotis ossification
Combined vascular malformation sand hypertrophy syndromes
Klippel-Trenaunay - capillary/lymphatic/venous
Parkes-Weber syndrome - AVN malformation
Maffuci- enchondroma todos with vascular malformation
DD of exquisitely tender lesion
Haemangioma Synovial sarcoma Neuroma Abscess Glomus tumour Fibrosarcoma Acute inflammation
Macro and micro Characteristics of Tuberculosis
Centrally necrotic, caseous cavity surrounded by multi-uncleared giant cells (epitheloid) and lymphocytes
Calcified late
Thickened synovium, effusion and pannus
Increased vascularity - osteopaenia
Absence of - sclerosis and periosteal reaction
Two Differential diagnoses of TB
Brucellosis - unpasteurised milk - Mx tetracycline
Rheumatoid
Abx Treatment of TB
RIPE
Rifampicin & Isoniazid for 9 months
Pyramidazole for 2 months
Ethambutol- retrobulbar neuritis
Features of congenital syphilis?
Treponema pallidum crosses placenta in second half pregnancy
Sick and irritable infant
Hepatosplenomegaly
Joint swelling and pseudoparalysis at several weeks old.
Swelling and tenderness at ends of long bones
What is the organism of syphilis? What is pathology
Treponema pallidum - penicillin
Spirochete
Angitis of vasa vasorum or small arterioles. Endarteritis produces vessel wall necrosis and tissue infaction supplied by vessel.
Primary - chancre
Secondary - mucocutaneous lesions and lymphadenopathy
Tertiary - untreated cases. Antibiotics ineffective
Orthopaedic manifestations of syphilis
Tabes dorsalis -
Posterior column degeneration
Lightening pains in lower limbs
Sensory ataxia, joint instability and CHARCOT
Gummata -
Punched out lesions in medulla
Destructive lesions in cortex
Pathological fractures
Sabre tibia
Xray
Periostitis- can be onion peel
Metaphysitis-
Trabecular erosion in juxta-epiphyseal region
Lucent band near physis followed by frank destruction
Similar to leukaemia and neuroblastoma
Dd Scurvy Multifocal OM Battered baby syndrome Caffey's disease - infantile cortical hyperostosis
Define synovial chondromatosis
Monoarticular Benign proliferation disorder of the synovium
Chondroid metaplasia of synovium resulting in production of MULTIPLE INTRA-ARTICULAR LOOSE BODIES
Primary
Secondary - degenerative arthritis with cartilage breaking off and growing in joint
Xray feature of synovial chondromatosis and DD
Stippled calcification of soft tissues around a joint
unless lesions large and ossified
Degenerative changes if SC is secondary
DD
Tumeral calcinosis
Soft tissue chondroma
Hypercalcaemia
Genetic association for enchondroma (MCQ)
IDH1 and IDH2 mutations
Especially Ollier disease and Mafucci syndrome
Define a hamartoma
Name some examples
A hamartoma is a benign, focal malformation that resembles a neoplasm in the tissue of its origin. This is not a malignant tumor, and it grows at the same rate as the surrounding tissues. It is composed of tissue elements normally found at that site, but which are growing in a disorganized mass.
Osteochondroma
Genetics for MHE
EXT1 & EXT2 proteins & genetic loci
Biosynthesis of heparin sulfate
Decreased expression leads to osteochondromas
What are the syndromes associated with non-ossifying fibromas
Familial multi-focal
Neurofibromatosis
Jaffe-Campanacci syndrome
Congenital with cafe-au-lait, mental retardation, non-skeletal abnorms - heart, eyes, gonads
Define fibrous dysplasia
Common developmental abnormality characterised by Hamartomatous proliferation of fibro-osseus tissue within bone
Genetics of fibrous dysplasia
Dominant activating mutations of GS alpha on chromosome 20q13
Produce a sustained adenylate cyclase- cAMP activation
High expressions of FGF-23
Cause of hypophosphataemia in McCune-Albright
Syndromes with fibrous dysplasia
McCune Albright - polyostotic FD, precocious puberty and Coast of Maine spots
Oncogenic osteomalacia- renal phosphate wasting due to FGF 23
Mazabraud syndrome - polyostotic FD, multiple intramuscular myxomas
Genetics of ABCs
Express TRE17/USP6 translocation
Chemotherapy for osteosarcoma
Adriamycin / doxorubicin - Blocks DNa/RNA synthesis via topoisomerase II
Cisplatinum - DNa disruption by covalent bonding
Methotrexate - inhibits dehydrofolate reductase
Ifosfamide - DNA alkylating agent
Genetics of osteopetrosis
Carbonic anhydrase II
TCIRG1 (ATP6i) gene mutation
Chloride channel 7
What are the seronegative arthropathies?
Reiter's Ankylosing spondylitis Psoriatic Enteropathic spondylitis Lupus associated arthritis
HLA-B27
Cellular Characteristics of rheumatoid synovitis
High presence of T-cells in inflamed tissue
Paucity of lymphocytes at invasion front and in synovial fluid
B-cells produce RF - IgM to the Fc of IgG
T-cells regulate inflammatory response
RF complexes activates complement cascade
What are DMARDS?
Synthetics
Methotrexate - give with folic acid
Biological
Infliximab- TNF alpha antagonist. Monoclonal antibody
4 causes of back kneeing
Polio
Lax posterior capsule
PCL deficiency
Fracture Malunion - femur extension, tibia recurvatum
5 causes of hip pain in Pagets
Fracture Osteoarthritis Malignant transformation - 2nd Osteosarc Active disease Spinal stenosis
Causes of Erlenmeyer flask deformity
Gauchers Osteopetrosis Niemann-Pick disorder MHE Lead poisoning Cranio Metaphyseal dysplasia Haemoglobinopathies- sickle / thalassaemia Metaphyseal dysplasia -Pyle disease
Two benign tumours that can metastasise
GCT
Chondroblastoma
Same as only 2 epiphyseal
3 tumours more common in women
Fibrous dysplasia
Parosteal osteosarcoma
GCT
5 mimickers
Big Five Mice
Fibrous dysplasia Metastatic bone disease Infection Cartilage lesions EG
Small round blue cell tumours
Infants
- neuroblastoma
- nephroblastoma - Wilm’s
- retinoblastoma
Kids
- EG / LCH
- Wilm’s
Adults
- Ewings
- MM
- Lymphoma
- Rhabdomyosarcoma