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imed3003 > Musculoskeletal Neoplasms and Metastatic Disease > Flashcards

Musculoskeletal Neoplasms and Metastatic Disease Flashcards

1
Q

What is the metaphysis?

A

The area between the diaphysis (shaft) and the epiphysis where the bone widens

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2
Q

What is the inside of bone and outside layer of bone called?

A

Compact bone = cortical bone = Outer layer of bone

Spongy bone = Trabecular bone = Cancellous bone = Inside layer of bone

Spongy bone + bone marrow = medullary cavity

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3
Q

What is lamellar bone and what is woven bone?

A

Lamellar bone = mature bone

Woven bone = osteoid = immature/new bone (can be produced by tumours)

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4
Q

What are the most common bone tumours?

A

Metastatic tumours from other parts of the body

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5
Q

What are the classes of bone tumours (classification by aetiology)?

A

Metastatic disease

Haematolymphoid malignancies (Plasma cell myeloma and Primary bone lymphoma)

Primary bone tumours (rare in comparison to metastases and haematolymphoid malignancies)

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6
Q

Which cancers most commonly metastasize to bone? (in order of incidence)

A

Lung, breast, prostate, colorectal adenocarcinoma

Thyroid adenocarcinoma

Renal Cell Carcinoma

Malignant melanoma

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7
Q

What are the types of bone metastases?

A

Osteolytic metastases: Bone is destroyed by tumour cells these are called osteolytic metastases. Cytokines stimulate osteoclastic bone resorption.

Osteoblastic metastases: Cytokines stimulate osteoblastic activity and new bone

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8
Q

Which cancers typically result in osteoblastic metastases?

A

Prostate and breast carcinomas

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9
Q

What are the most common types of haematolymphoid malignancies?

A

Plasma cell (multiple) myeloma

Primary bone lymphoma (haematology)

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10
Q

What is plasma cell myeloma?

A

Previously called multiple myeloma, bone marrow-based malignant neoplasm of plasma cells. Forms multiple tumours throughout skeletal system

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11
Q

What is the incidence of plasma cell myeloma?

A

Most common primary malignancy of bone. (usually in >50yo

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12
Q

What are the clinical symptoms of plasma cell myeloma?

A

Bone pain, pathological fracture (tumours in bone)

Hypercalcaemia (osteoclastic bone destruction)

Monoclonal immunoglobulin in serum and urine (clonal proliferation of plasma cells)

Renal failure (paraprotein deposits in kidney)

Susceptibility to infection (abnormal immunoglobulin)

Anaemia (Chronic disease)

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13
Q

What is an M protein/paraprotein?

A

Protein produced by abnormal plasma cells that have proliferated.

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14
Q

How are bone tumours identified?

A

Their clinicopathological features are relatively constant:

Age of patient

Bone and area affected

Radiological and pathological features (multidisciplinary bone tumour meeting)

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15
Q

How are bone tumours managed?

A

Several disciplines are involved because it can affect relatively young people.

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16
Q

What are the types of tumours and how are they further divided?

A

Bone forming (eg osteosarcoma)

Cartilaginous

Fibro-osseus

Fibrous/fibrohistiocytic

Giant cell rich

Cystic lesions

Miscellanious

These tumours are further divided into benign and malignant categories

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17
Q

What type of tumour is the osteosarcoma?

A

Bone forming malignant mesenchymal neoplasm (sacoma) producing bone matrix.

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18
Q

How common is osteosarcoma?

A

Most common primary malignant tumour of bone and most commonly affects children.

Males are slightly more commonly affected and there is a bimodal age distribution (10 - 20s and then >20 years old)

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19
Q

What are the risk factors for primary bone cancer?

A

Adolescents

Majority idiopathic

Hereditary retinoblastoma

Li-Fraumeni syndrome (p53 mutation)

Ionising radiation

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20
Q

What are the risk factors for secondary bone cancer?

A

Older adults

Underlying benign bone disease: Paget’s disease, benign bone tumours, chronic osteomyelitis

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21
Q

What are the clinical symptoms of osteosarcoma?

A

Nocturnal local bone pain

Palpable mass

Pathological fracture

Constitutional symptoms (fever, anorexia, weight loss, metastatic disease)

Destructive mass in medullary cavity invading through cortex that can be identified using Codman’s triangle on x-ray or sunburst pattern.

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22
Q

What is the prognosis for osteosarcoma?

A

80% 5 year survival rate in localised disease

30% 5 year survival rate in metastatic disease

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23
Q

What do osteosarcomas look like on histology?

A

Malignant tumour cells produce osteoid matrix in a delicate pericellular lacelike pattern. This is most prominent in osteoblastic variant.

24
Q

What is an osteoid osteoma?

A

A benign bone-forming tumour.

25
Q

How do osteoid osteoma present clinically?

A

Presents as nocturnal pain that is responsive to NSAIDs

26
Q

Where are osteoid osteomas most commonly seen?

A

Affects people from 5 - 20 years of age and males twice as often as females.

It is seen on the long bones most often within the cortex.

27
Q

How common is the osteoid osteoma?

A

It makes up 15% of all primary benign bone tumours

28
Q

What does osteoid osteoma look like on histology and macroscopically?

A

It is a small circumscribed cortical-based lesion

Trabeculae of woven bone

Plump osteoblastic rimming

29
Q

What is an enchondroma?

A

Benign intramedullary cartilaginous neoplasm

30
Q

Where are enchondromas commonly seen?

A

In small tubular bones of hands and feet (phalanges) > long bones

31
Q

How common are enchondromas?

A

25% of primary benign bone tumours

32
Q

Who is most commonly affected by enchondromas?

A

Males and females equally in their 20s and 30s

33
Q

What are the risk factors for enchondroma?

A

Multiple lesions are formed in Ollier’s syndrome

34
Q

What is the clinical presentation of enchondroma?

A

Asymptomatic; it is often an incidental radiological finding.

Can cause pain with pathological fracture in some cases.

35
Q

What is the prognosis of enchondromas?

A

Benign and can be treated with local curettage.

36
Q

What does enchondroma look like histologically and macroscopically?

A

Radiologically:

Circumscribed cartilaginous lesion

Expanding medullary cavity of tubular bones

Histologically:

Nodules of hyaline cartilage

Sharp interface with trabecular bone

37
Q

What is an osteochondroma?

A

Benign pedunculated tumour of bone with cartilage cap overlying bony stalk

Commonly called exostosis

38
Q

How common are osteochondromas? Who are they most commonly seen in?

A

Most common benign bone tumours (50%)

Seen more in males than females that are 10 - 20 years of age.

39
Q

Which part of the bone do osteochondromas most commonly affect?

A

Metaphyseal surface of long bones

40
Q

What are the risk factors for ostechondroma?

A

Most common one is multiple hereditary exostosis which is an autosomal dominant condition.

41
Q

What is the clinical presentation of osteochondroma?

A

Usually asymptomatic and seen on radiology indidentally (unless impinging on a nerve or if there is fracture of stalk)

42
Q

What is the prognosis of osteochondroma like?

A

Benign and can be treated with conservative management

Symptomatic cases cured with simple excision

43
Q

What does an osteochondroma look like radiologically and histologically?

A

Radiologically:

Pedunculated (mushroom-like) lesion arsing from metaphysis that points away from joint space

Stalk is continuous with medullary cavity

Histologically:

Cartilage cap (neoplastic part of the tumour)

Merges with underlying bony stalk through endochondral ossification

44
Q

What is fibrous dysplasia?

A

A benign fibro-osseus tumour of bone.

45
Q

Who most commonly gets fibrous dysplasia?

A

7% of primary benign tumours

Early to mid adulthood and affects axial skeleton more often than long bones.

46
Q

What are the risk factors for fibrous dysplasia?

A

Usually it is monoastotic but can also be polyastotic (McCune-Albright syndrome)

It is non-inherited

47
Q

How does fibrous dysplasia present clinically?

A

Most are incidental radiological findings.

48
Q

What does fibrous dysplasia look like radiologically and histologically?

A

Radiologically:

Well-defined ground glass opacity within medullary cavity

Varus deformity in femur (shepherd’s crook)

Histologically:

Fibro-osseus tumour

Curvilinear arrays of woven bone (resembles chinese writing)

Fibrous stroma with bland spindle cells.

49
Q

What is Ewing sarcoma?

A

A highly malignant small, round, blue cell tumour.

It is a primitive ectodermal tumour (PNET)

50
Q

How common is ewing sarcoma and who is it most common in?

A

It makes up 10% of primary malignant bone tumours, affects children from 10 - 15 years of age and affects males more than females.

Primarily affects the long bones and pelvis

51
Q

How does ewing sarcoma typically present?

A

Painfully enlarging mass

It is usually detected only after it becomes metastatic

52
Q

What is the prognosis of ewing sarcoma?

A

5 year survival rate is 75%

Long term cure only in 50%

53
Q

How is ewing sarcoma diagnosed?

A

Prototypical small round blue cell tumour with rosettes on histology (due to cells being almost completely nuclei)

EWSR1-FLI1 t(11;22) translocation on FISH

Diffuse CD99 staining immunohistochemistry

54
Q

What is a sarcoma?

A

A malignant soft tissue tumour. (very rare)

55
Q

How do soft-tissue tumours present clinically?

A

Palpable mass for extremity tumours

Abdominal distension for abdominal/retroperitoneal tumours

Things that increase suspicion for sarcomas:

Tumour size >5cm or enlarging mass

Deep-seated (deep to fascia/intramuscular)

Painful

Multiple lesions

56
Q

How are soft tissue tumours classified?

A

WHO classification:

> 140 different subtypes

Histological classification:

Line of differentiation

Mature mesenchymal tissue which the tumour most closely resembles

imed3003 (48 decks)

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