3. Tumours of Bone and Soft Tissue Flashcards

(52 cards)

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
Where does Ewings Sarcoma present?
Any bone / any location
26
Describe the histological appearance of Ewings sarcoma?
* Small Round Cells (like lymphocytes) * No Differentiation * CD 99 (immunohistochemistry) * Resemble Primitive Neuroectoderm Cells
27
By what name is Ewing's Sarcoma also known as?
AKA Malignant small cell blue round tumour.
28
What is the Classic Molecular Abnormality associated with Ewings Sarcoma?
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
What signs of Ewing's Sarcoma may be present on X-Ray?
* Destructive Lytic | * "Onionskin" (not specific)
30
What is the Ddx for Ewing's sarcoma and how are they distingushed?
(DDX Osteomyelitis) | Histology required to distinguish
31
What is the histological appearance of osteogenic sarcoma?
* Anaplastic Spindle Cells * Osteoid + Bone * Rare Low Grade Variant * Parosteal (surface) OGS
32
In whom is OGS common?
Young
33
Where is OGS common?
Metaphysis (calcified)
34
What tissue is OGS a malignancy off?
Bone
35
What tissue is chondrosarcoma a malignancy off?
Cartilage
36
In malignant bone tumours, what is used to give a prognosis regarding 5 year survival?
Resected bone will be sent to lab and the histological findings re necrosis etc will give prognosis re 5yr survival.
37
What treatments are available for bone tumours?
* Surgery (Amputation or Limb Sparing, appropriate prosthesis replaces portion of the tibia/fidia) * Chemotherapy * Radiotherapy
38
Give an example of a primary bone tumour which can be benign or malginant?
Giant Cell Tumour (GCT) (Tumour of osteoclasts)
39
What are the two broad categories of soft tissue tumours?
Visceral | Non-Visceral
40
What are the main types of benign/malignant soft tissue tumours?
* Fibroma/Sarcoma * Lipoma/Sarcoma * Leiomyoma/Sarcoma (Uterus) * Rhabdomyoma/Sarcoma * Neurofibroma/Sarcoma
41
Name the 3 types of soft tissue sarcoma studied?
* Malig fibrous histiocytoma * Synovial Sarcoma * Ewing’s Sarcoma
42
What is the commonest type of non-visceral soft tissue tumour?
Stis Sarcoma | Mid Age or Elderly
43
What are the histological appearance of Malignant Fibrous Histiocytoma (MFH)?
Primitive Mesenchyme
44
What are the histological variants of MFH?
``` o Pleomorphic o Myxoid o Giant Cell o Inflammatory o Angiomatoid ```
45
In whom does synovial Sarcoma usually occur and where?
* Young Adults * Close to but almost never within joint cavities * Recapitulates Primitive Synovium * High Grade Tumour
46
What factors give a prognosis in synovial sarcoma?
o Location: Deep (bad) v Superficial o Size: 5cm o Grade: 1-3 (cellularity, atypia, mitoses)
47
What are Desmoid Tumours?
Aggressive Fibromatosis/Grade 1-2 fibrosarcoma
48
What invasion patterns do Desmoid tumours exhibit?
* Locally Invasive/Destructive | * Does not Metastasise
49
What type of fibromatosis is commonly seen after trauma?
Multicentric Fibromatosis (rapid proliferation)
50
What chromosomal rearrangement is associated with Synovial Sarcoma?
t(X:18)
51
What chromosomal rearrangement is associated with Ewings Sarcoma?
t(11:22)
52
What chromosomal rearrangement is associated with Rhabdomyosarcoma?
T(2:13)