Skeletal Tumours

Question 1

Metastatic lesions in bone are commonly from what primary tumours?

Answer 1

breast 
lung 
prostate 
thyroid
kidney

Question 2

Describe an osteochondroma.

Answer 2

benign cartilage capped bony projection arising on external surface of bone containing a marrow cavity that is continuous with that of underlying bone.

Question 3

In who do osteochondromas arise and what do they present with?

Answer 3

Young <20
Male = female
develop near epiphyses of long bones
pain and irritation

Question 4

What is the treatment for osteochondromas?

Answer 4

surgical removal

Question 5

A chondroma is another benign skeletal tumour. Describe it.

Answer 5

Benign hyaline cartilage tumour arising in the medullary cavity of bones of hands and feet - if breaks through into bone cortex then is malignant

Question 6

Are chondromas more common in young men or young women?

Answer 6

young men

Question 7

What is Maffucci’s syndrome?

Answer 7

Multiple enchondromatosis with soft tissue and visceral haemangiomas of viscera.

Question 8

What is Ollier’s disease?

Answer 8

Enchondromas

Tumours are typically unilateral and involving one extremity

Question 9

Is the risk of malignant transformation higher in Ollier’s disease or Maffucci’s syndrome?

Answer 9

Maffucci’s syndrome

Question 10

What is an osteoid ostemoa?

Answer 10

Benign osteoblastic tumour composed of a central core of vascular osteoid tissue and a peripheral zone of sclerotic bone.

Question 11

What is the typical presentation of an osteoid ostemoa?

Answer 11

Dull pain that is worse at night and disappears within 20-30 mins of treatment with aspirin or NSAID.
may be accompanied by soft tissue swelling and tenderness, synovitis and limitation of movement.

Question 12

How long does an osteoid osteoma take to spontaneously resolve?

Answer 12

33 months without treatment

Question 13

What is a chondroblastoma.

Answer 13

Benign cartilage tumour arising in bone. If more aggressive may extend beyond bone.

Question 14

Where are chondroblastomas found?

Answer 14

Epiphysis of long bones

Question 15

Describe the radiological appearance of a chondroblastoma.

Answer 15

Spherical and well defined osteolytic foci, sometimes extending into the subarticular bone, joint, space or metaphysis.

Question 16

Describe the histological appearance of a chondroblastoma.

Answer 16

distinct cytoplasmic borders with foci of “chicken-wire” calcification

Question 17

What treatment is required for a chondroblastoma.

Answer 17

Biopsy and curettage plus adjuvant liquid nitrogen

Question 18

Name the 3 benign but locally aggressive skeletal tumours.

Answer 18

Chordroma
Giant cell tumour
Osteoblastoma

Question 19

What cell is presumed to be the origin of giant cell tumour?

Answer 19

Osteoclast

Question 20

Where are giant cell tumours often found?

Answer 20

Long bones - often around the knee

Question 21

Radiologically, what is key to differentiate a giant cell tumour from an osteoblastoma?

Answer 21

Giant cell tumour - radiolucent with increasing density towards periphery
Osteoblastoma - radiolucent defect with a central density due to ossification. May have surrounding sclerosis.

Question 22

What are the treatment options for a giant cell tumour?

Answer 22

Curettage or resection

Question 23

Why should a giant cell tumour not be radiated?

Answer 23

If radiated it may undergo malignant transformation

Question 24

Describe an osteoblastoma.

Answer 24

Solitary, benign and self-limited tumour that produces osteoid and bone.

Question 25

What are the common symptoms of osteoblastoma?

Answer 25

Pain of long duration, swelling and tenderness. | Tumours of the spine may cause scoliosis and neurological symptoms

Question 26

What are the treatment options for am osteoblastoma?

Answer 26

Curettage or intra-lesional excision or en-bloc resection

Question 27

What is a Chordroma?

Answer 27

Very rare tumour arising from notochord remnants. It is locally destructive and invasive.

Question 28

Where are chordromas found?

Answer 28

Midline tumour often in the sacral region - management is hard

Question 29

Who do chordromas present in?

Answer 29

older adults 40+ years | M:F 1:2

Question 30

Where do chordromas often invade into?

Answer 30

lesion often tracks along nerve roots in the sacral plexus or out the sciatic notch in planes of least resistance. Also soft tissue planes between muscles.

Question 31

What is used for treatment of a Chordroma?

Answer 31

difficult to resect (usually in sacral region) radiation may be helpful Chemotherapy for late stage disease

Question 32

Metastatic bone lesions are described as what?

Answer 32

Osteolytic lesions | Prostate mets - osteoslerotic giving hardening of bony lesions

Question 33

Less commonly what other tumours can metastasise to bone?

Answer 33

gastric malignant melanoma neuroblastoma

Question 34

What is multiple myeloma?

Answer 34

Malignant primary bone tumour that occurs in old age. Malignant proliferation of plasma cells in bone marrow giving bone destruction of axial skeleton and often causes renal failure.

Question 35

What doe multiple myeloma produce and what does this mean for the patient?

Answer 35

Produces monoclonal IgG therefore patient is susceptible to infection

Question 36

What is the characteristic appearance of multiple myeloma?

Answer 36

rounded "punched out" lesions

Question 37

What is Ewing's sarcoma?

Answer 37

Highly malignant peripheral primitive neuroectodermal tumour (PNET)

Question 38

Where does ewing's sarcoma arise?

Answer 38

metaphysis and diaphysis of femur, tibia and humerus.

Question 39

In who does Ewing's sarcoma present?

Answer 39

Most common in second decade of life | M:F 3:2

Question 40

What is the most common and second most common primary bone tumour?

Answer 40

1 - Osteosarcoma | 2 - Chondrosarcoma

Question 41

What causes an osteosarcoma and where does it usually occur?

Answer 41

Malignant osteoblasts forming osteoid, mineralizes poorly | Usually occurs in the ends of long bones particularly distal femur, proximal tibia and proximal humerus

Question 42

What is essential for diagnosis of Osteosarcoma?

Answer 42

Osteoid production from the tumour

Question 43

What Tx is required for osteosarcoma?

Answer 43

Pre op chemo (tumour debulking) surgical resection post op chemo (residual tumour or mets)

Question 44

What is specific about a chondrosarcoma?

Answer 44

Exhibits pure hyaline cartilage differentiation no bony elements - composed of malignant chondrocytes

Question 45

What happens in a chondrosarcoma?

Answer 45

Nodules of tumour erode and extend outward from bone giving areas of bone destruction accompanied by partial calcification. Tumour often extends into soft tissue.

Question 46

What treatment is required for chondrosarcoma?

Answer 46

Wide surgical excision