slow-growing, silent from cortical bone skull sinuses, tubular bones chronic sinusitis, headaches, ocular disturbances, exophthalmos female x3

Neoplastic lesions of bone & cartilage Flashcards by Danielle Mercier

List the developmental and congenital anomalies of the cervical spine

occipitalisation
posterior ponticle
odontoid anomalies
block vertebra
cervical ribs

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List the tumour-like neoplastic lesions of bone and cartilage?

Tumour-like:
• paget’s disease
• fibrous dysplasia

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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)

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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

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What is Paget’s disease?

abnormal destruction of bone with abnormal reparative process
results in deformities
unknown aetiology
familial and maybe viral
4 phases

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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

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What is the epidemiology of Paget’s disease?

> 50yrs
3% of >40s have it
men x2
familial and environmental factors

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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

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What area is most commonly affected by Paget’s disease?

pelvis
lumbar spine
femur

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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

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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

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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

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What is solitary enchondroma?

bening, < 30s
islands of cartilage in metaphysics of a bone
hands & feet
asymptomatic unless path. fracture
long bones more symptomatic

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What is the most common benign bone tumour of the hand?

solitary enchondroma

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What is multiple enchondromatosis?

< 30s
hands, feet, femur, tibia, iliac crest (bilateral)
asymptomatic unless path. fracture
if large: deformity, loss of function

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What is an osteoma?

slow-growing, silent
from cortical bone
skull sinuses, tubular bones
chronic sinusitis, headaches, ocular disturbances, exophthalmos
female x3

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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