Neoplastic lesions of bone & cartilage Flashcards
List the developmental and congenital anomalies of the cervical spine
- occipitalisation
- posterior ponticle
- odontoid anomalies
- block vertebra
- cervical ribs
List the tumour-like neoplastic lesions of bone and cartilage?
Tumour-like:
• paget’s disease
• fibrous dysplasia
List the benign neoplastic lesions of bone and cartilage?
- solitary osteochondromas
- hereditary multiple exostoses
- solitary enchondroma
- multiple enchondromatosis
- osteoma
- osteoid osteoma
- bone island
- other (bone cyst, giant cell tumour, fibrous xanthoma, chondroblastoma, osteoblastoma, haemangioma)
List the malignant neoplastic lesions of bone and cartilage?
Primary Multiple myeloma • Osteosarcoma • chondrosarcoma • Ewing's sarcoma • fibrosarcoma • non-hodgkin's lymphoma of bone
Metastatic
What is Paget’s disease?
- abnormal destruction of bone with abnormal reparative process
- results in deformities
- unknown aetiology
- familial and maybe viral
- 4 phases
What are the 4 phases of Page’ts disease pathology?
1) Resorptive -osteoclastic activity predominates
2) Mixed -osteoblsatic réponse to osteoclasts
3) Sclerotic -osteoblastic predominates
4) Malignant degeneration (<2% of cases)
• during active phase: rate f transfer of calcium in and out of the skeleton x7 but balance is close to zero
What is the epidemiology of Paget’s disease?
- > 50yrs
- 3% of >40s have it
- men x2
- familial and environmental factors
What are the clinical features of Paget’s disease?
- lesions seldom painful
- weakened, deformed thickened skeleton (path. fractures)
- skull enlarges (basilar invagination)
- shunting of blood (hypertension, arteriosclerosis)
- <2% malignant changes at 70-80 yrs
- 80-95% remain undiagnosed due to small symptoms
What area is most commonly affected by Paget’s disease?
pelvis
lumbar spine
femur
What is fibrous dysplasia?
- bone marrow replaced with fibro-osseous tissue
- also effects skin and endocrine (precocious sex development)
- 8-14yrs
- asymptomatic unless fracture
- bowing
- café au lait
- from: mutation in gene for G-alpha protein
What is solitary osteochondromas?
- benign, male x2
- exostoses from cortical surface with a hyaline-lined cartilage cap
- femur, humerus, tibia
- asymptomatic
- discovered by 20yrs incidentally
- common -50% of all bone tumours
What is hereditary multiple exostoses? (HME) (tissue, symptoms, sites)
- metaphysical overgrowth with multiple osteochondromas
- average 10 lesions
- asymptomatic
- pain if malignant degeneration (rare)
- autosomal dominant
- less severe in females
- femur, tibia, humerus, radius
What is solitary enchondroma?
- bening, < 30s
- islands of cartilage in metaphysics of a bone
- hands & feet
- asymptomatic unless path. fracture
- long bones more symptomatic
What is the most common benign bone tumour of the hand?
solitary enchondroma
What is multiple enchondromatosis?
- < 30s
- hands, feet, femur, tibia, iliac crest (bilateral)
- asymptomatic unless path. fracture
- if large: deformity, loss of function
What is an osteoma?
- slow-growing, silent
- from cortical bone
- skull sinuses, tubular bones
- chronic sinusitis, headaches, ocular disturbances, exophthalmos
- female x3
What is an osteoid osteoma?
- benign, < 30’s, males x2
- fibrous CT surrounded by sclerotic bone, < 1cm
- femur, tibia, spine
- insidious severe, aching pain, worse at night, relieved by aspirin
- sweating, hot
What is a bone island?
- focal lamellar bone in normal spongiosa
- incidental finding (asymptomatic)
- adults
- 30% show growth active growth
- some shrink
What is Gardner’s syndrome?
trio of growths involving:
- numerous osteomas
- polyps in colon
- soft tissue fibromas
What is an enostoma?
other name for bone island
What is multiple myeloma?
- monoclonal malignant, of plasma cells from bone marrow
- non-Hodgkin’s lymphoma, B cell
Destroys osseous tissue: • pain, fractures, deformities • anaemia, leucopenia, thrombocytopenia • osteoporosis • Bence Jones proteins in urine, renal tubular damage, hypercalcaemia • amyloidosis
What is osteosarcoma?
- malignant, < 30’s, male x2
- undifferentiated osteoid CT
- long bones (KNEE, close to metaphysis)
- pain, swelling, increasing
- poor prognosis
- mets to lung, bone, kidney
- Lab: high alkaline phosphatase
What are the most common primary malignant bone tumours in order of most to least?
1) Multiple myeloma
2) Osteosarcoma
3) Chondrosarcoma
4) Ewing’s sarcoma
List the types of osteosarcoma?
- central (medullary)
- multi centric (multiple lesions)
- parosteal (juxtacortical)
- secondary (transformation of benign lesion, Paget’s, osteochondroma)
- extraosseous
What is chondrosarcoma?
- malignant, <30s, male x2
- cartilaginous tissue
- pelvis, proximal femur, ribs, humeurs, scapula, femur, tibia
- highly aggressive
- mets to lung
List the types of chondrosarcomas
- central (medullary)
- peripheral (juxtacortical)
- extraosseous (rare)
- secondary (osteochondroma or enchondroma)
What carcinomas DO NOT metastasize to bone?
CNS tumours and basal cel carcinomas
What are the pathways of metastatic spread of metastatic bone tumours?
1) Direct extension -tumours from peritoneal cavity
2) Lymphatic dissemination -rare cus no lymphatic channels in bone marrow
3) Hematogenous dissemination
- most common
- veins vulnerable to tumour penetration
- lung, liver, axial skeleton
What are the clinical features of bone metastasis?
- unexplained weight loss, cachexia
- anaemia, fever (later)
- skeletal pain insidious and at night
- pathological fracture
What investigations do we do for bone metastasis?
• high ESR
• maybe high serum calcium
• elevated alkaline phosphatase with elastic lesions
high acid phosphatase and Prostate specific antigen (PSA) with prostatic carcinoma when spread to bone
How can Paget’s disease be fatal?
- complications of pathological fracture -> fat embolism, haemorrhage
- cardiovascular issues (bones require lots of blood supply -> huge strain on heart)
- malignancies -lung, brain
How can osteoporosis be fatal?
- hip fracture -fat embolus from bone marrow -> pulmonary embolus
- rib fracture
- tearing arteries
- sever kyphosis (interrupt internal organs -lungs, digestion)
Explain why hyperparathyroidism has a major effect on the skeleton
- extreme activity of the PTH in its job to increase serum calcium
- will increase resorption of calcium from the bones to increase serum calcium
- increase intestinal absorption of calcium
Describe the most likely mechanism by which a child may develop acute osteomyelitis.
- most often haematogenous spread
- staphylococcus aureus is most likely, localised collection under skin (boil)
- *same as for adult
When would you suspect a child to have acute osteomylteitis. How could this be confirmed?
- redness, swelling, heat, tenderness, pain (rest) -any inflamed tissue
- rapid onset of local tenderness, soft tissue swelling and pyrexia
- examined by radiograph examination or laboratory blood culture
- order whole blood check: maybe leucocytes
- x-ray, CT scan, MRI
- ESR increased
Describe metastatic bone tumours?
- > 40yrs
- marrow rich bones
- poor prognosis
- Hodgkin’s lymphoma, Ewing’s, osteosarcoma, neuroblastoma
What are 70% of all bone tumours? metastatic or primary?
metastatic
What is Ewing’s sarcoma?
- undifferentiated, aggressive (5% survival)
- <30’s, male x2
- from bone marrow, round cell
- diaphysral; femur, pelvis
- looks like infection (pain, swelling, hot, tender)
- slight fever
- mets to lungs
What are the clinical features of chondrosarcomas?
- swelling, pain (late)
- peripheral lesions asymptomatic
- if early detection and treatment -> 90% 5-year survival rate
Where are metastatic bone tumours from?
- breast,
- prostate
- lung
- kidney (most from epithelial tissue)
Where are located most metastatic bone tumours?
- spine
- ribs
- sternum
- pelvis
- sacrum
- skull
- long tubular bones
What is the difference between lytic and blastic metastases?
Lytic:
• destruction from expanding tumour on trabeculae & cortices
• Trabeculae may be absorbed
• begin in the medullary cavity
Blastic:
• increase bone density
• deposition of non-neoplastic bone (reactive sclerosis) -futile attempt at bone repair