3. Tumours of Bone and Soft Tissue Flashcards

1
Q

Which is the more common source of bone tumours, primary or metastatic?

A

Metastatic

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2
Q

Malignant tumours in bone tend to metastasise from which areas?

A

Lung, Prostate, Breast, (2/3’s collectively, and most common cancers overall)

Also Kidney, Thyroid

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3
Q

Name the 3 main types of benign Primary Bone Tumours

A
  • Osteoma
  • Osteoid Osteoma
  • Osteoblastoma
Other
o	Non-ossifying fibroma
o	Cysts simple
o	Aneurysmal Bone Cyst (has a lining)
o	Fibrous dysplasia (proliferation of benign bone)
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4
Q

What are the clinical signs of and osteoid osteoma?

A
  • Pain is relived by Aspirin
  • ~ 1cm
  • Radiolucency with Central Nidus of bone,
  • Histology = benign bone + plump osteocytes
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5
Q

What distinguishes a osteoid osteoma from an osteoblastoma?

A

Size

If > 2cm = Osteoblastoma

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6
Q

What are the 4 types of (benign) cartilage-forming bone tumours?

A
  • Osteochondroma (surface)
  • Enchodroma (syndromes)(inside)
  • Chondroblastoma
  • Chondromyxoid fibroma
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7
Q

What is an osteochondroma also known as?

A

• AKA Cartilaginous Exostosis (any bone)

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8
Q

What are the clinical features of osteochondroma?

A

Very common primary bone lesion.
At 2-3cm presents as a lump.
Technically the term applies only exostosis is just bone but these are cartilaginous.
Cartilage Cap (if >2cm in depth the risk of malignancy increases)
Rarely Becomes Malignant (<1% become malignant)

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9
Q

What cartilaginous tumour is common in the fingers?

A

Enchondroma

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10
Q

What are the clinical features of Enchondroma?

A
  • Fingers commonly (and also feet?)
  • Islands of Cartilage
  • Rarely Becomes Malig.
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11
Q

What presentation of cartilaginous tumours most often becomes malignant? In which conditions is this most often seen?

A
Multiple Chondromas 
(Ollier's Disease, Maffucci's Syndrome [autosomal dominant])
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12
Q

What is the rate of malignant transformation in multiple chondromas?

A

Malignant 20%-40%.

By 50yo=50% (to do with the genetics of the syndromes)

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13
Q

Name the 3 main types of Malignant Primary Bone Tumours. Name the other, less common type.

A
  1. Osteosarcoma
  2. Chondrosarcoma
  3. Ewing sarcoma
    (Also Giant Cell Tumours ‘GCT’s’)
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14
Q

Which is the most common malignant primary bone tumour?

A

Osteosarcoma

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15
Q

What are some risk factors for osteosarcoma?

A

Pagets Dis Bone
Post Radiation Therapy
Retinoblastoma (5-10% of paediatric patients develop osteosarcoma*)
(Very High incidence)

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16
Q

Where does osteosarcoma present in younger patients?

A

90% occur in the metaphysis (active growing area) of long bones

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17
Q

How does osteosarcoma present in elderly patients?

A

Usually as a result of Pagets Disease
Presents in the Flat Bones
Extraskeletal Osteogenic Sarcoma - V.Rare

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18
Q

Where does osteogenic sarcoma (ogs) typically present?

A

Near the joints such as the distal femur or proximal tibia

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19
Q

What signs of osteosarcoma are can be present on x-ray?

A

Medulla
o Combination of Lytic and Sclerotic appearance. Depending on ratio of deposit/dest
o Mixed (10-20% require 2nd biopsy to diagnosis, especially if Rx/Path don’t match)
o “Codman’s Triangle” (not specific) (Codman triangle (previously referred to as Codman’s triangle) is the triangular area of new subperiosteal bone that is created when a lesion, often a tumour, raises the periosteum away from the bone.)
o Destroys Adjacent Bone

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20
Q

What are the main causes of chondrosarcomas?

A
  • De Novo 80%

* Multiple Chondromas 20%

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21
Q

Where do chondrosarcomas typically appear?

A
  • Central Skeleton
  • Flat Bones (Metaphysis)
  • Rare in hands or feet
22
Q

What are the signs of chondrosarcoma on X-ray?

A

o “Ring” Calcification
o Large
o Destroys Adjacent Bone

23
Q

What is the histological appearence of chondrosarcoma?

A

o Atypical Cartilage Cells

o Grade I-III (v. v. important) Again Rx/Path must correlate as typical signs N/P

24
Q

In whom is Ewings Sarcoma Common?

A

Young <25

Typically 8-10yo

25
Q

Where does Ewings Sarcoma present?

A

Any bone / any location

26
Q

Describe the histological appearance of Ewings sarcoma?

A
  • Small Round Cells (like lymphocytes)
  • No Differentiation
  • CD 99 (immunohistochemistry)
  • Resemble Primitive Neuroectoderm Cells
27
Q

By what name is Ewing’s Sarcoma also known as?

A

AKA Malignant small cell blue round tumour.

28
Q

What is the Classic Molecular Abnormality associated with Ewings Sarcoma?

A

Long arm reciprocal 11/22 translocation
Fusion gene EWS/FLI – gives rise to a high grade proliferation
Molecular abnormality is required to deliver precise Dx

29
Q

What signs of Ewing’s Sarcoma may be present on X-Ray?

A
  • Destructive Lytic

* “Onionskin” (not specific)

30
Q

What is the Ddx for Ewing’s sarcoma and how are they distingushed?

A

(DDX Osteomyelitis)

Histology required to distinguish

31
Q

What is the histological appearance of osteogenic sarcoma?

A
  • Anaplastic Spindle Cells
  • Osteoid + Bone
  • Rare Low Grade Variant
  • Parosteal (surface) OGS
32
Q

In whom is OGS common?

A

Young

33
Q

Where is OGS common?

A

Metaphysis (calcified)

34
Q

What tissue is OGS a malignancy off?

A

Bone

35
Q

What tissue is chondrosarcoma a malignancy off?

A

Cartilage

36
Q

In malignant bone tumours, what is used to give a prognosis regarding 5 year survival?

A

Resected bone will be sent to lab and the histological findings re necrosis etc will give prognosis re 5yr survival.

37
Q

What treatments are available for bone tumours?

A
  • Surgery (Amputation or Limb Sparing, appropriate prosthesis replaces portion of the tibia/fidia)
  • Chemotherapy
  • Radiotherapy
38
Q

Give an example of a primary bone tumour which can be benign or malginant?

A

Giant Cell Tumour (GCT) (Tumour of osteoclasts)

39
Q

What are the two broad categories of soft tissue tumours?

A

Visceral

Non-Visceral

40
Q

What are the main types of benign/malignant soft tissue tumours?

A
  • Fibroma/Sarcoma
  • Lipoma/Sarcoma
  • Leiomyoma/Sarcoma (Uterus)
  • Rhabdomyoma/Sarcoma
  • Neurofibroma/Sarcoma
41
Q

Name the 3 types of soft tissue sarcoma studied?

A
  • Malig fibrous histiocytoma
  • Synovial Sarcoma
  • Ewing’s Sarcoma
42
Q

What is the commonest type of non-visceral soft tissue tumour?

A

Stis Sarcoma

Mid Age or Elderly

43
Q

What are the histological appearance of Malignant Fibrous Histiocytoma (MFH)?

A

Primitive Mesenchyme

44
Q

What are the histological variants of MFH?

A
o	Pleomorphic
o	Myxoid
o	Giant Cell
o	Inflammatory
o	Angiomatoid
45
Q

In whom does synovial Sarcoma usually occur and where?

A
  • Young Adults
  • Close to but almost never within joint cavities
  • Recapitulates Primitive Synovium
  • High Grade Tumour
46
Q

What factors give a prognosis in synovial sarcoma?

A

o Location: Deep (bad) v Superficial
o Size: 5cm
o Grade: 1-3 (cellularity, atypia, mitoses)

47
Q

What are Desmoid Tumours?

A

Aggressive Fibromatosis/Grade 1-2 fibrosarcoma

48
Q

What invasion patterns do Desmoid tumours exhibit?

A
  • Locally Invasive/Destructive

* Does not Metastasise

49
Q

What type of fibromatosis is commonly seen after trauma?

A

Multicentric Fibromatosis (rapid proliferation)

50
Q

What chromosomal rearrangement is associated with Synovial Sarcoma?

A

t(X:18)

51
Q

What chromosomal rearrangement is associated with Ewings Sarcoma?

A

t(11:22)

52
Q

What chromosomal rearrangement is associated with Rhabdomyosarcoma?

A

T(2:13)