Histo: Neoplastic Bone Disease Flashcards

1
Q

Which part of the body is most commonly affected by neoplastic bone disease?

A

Around the knee joint

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2
Q

Outline some presenting features of neoplastic bone disease.

A
  • Pain
  • Swelling
  • Deformity
  • Fracture
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3
Q

How are bone tumours diagnosed

A

X-ray

Biopsy

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4
Q

What type of biopsy is often used for diagnosing neoplastic bone disease?

A

Needle biopsy using a Jamshidi needle under CT or US guidance
Open biopsy for sclerotic or inaccessible lesions

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5
Q

List some tumour-like conditions of the bone.

A
  • Fibrous dysplasia
  • Metaphyseal fibrous cortical defect/non-ossifying fibroma
  • Reparative giant cell granuloma
  • Ossifying fibroma
  • Simple bone cyst
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6
Q

What is fibrous dysplasia? Which age does it affect?

A
  • Condition in which fibrous tissue replaces normal bone tissue
  • Can occur in any bone but ribs and proximal femur is most common
  • Tends to affect adolescents
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7
Q

Which eponymous syndrome is characterised by polyostotic fibrous dysplasia?

A

McCune Albright Syndrome: polyostotic fibrous dysplasia + endocrine problems + rough border café-au-lait spots

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8
Q

Which mutation causes fibrous dysplasia?

A

GNAS mutation Chr 20 q13 (mutation in a G-protein)

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9
Q

Describe the histological appearance of fibrous dysplasia.

A

The marrow is replaced by fibrous stroma with rounded trabecular bone (‘Chinese letters’)

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10
Q

Describe the deformity seen on X-ray of fibrous dysplasia of the femoral head. What other lesion might you see?

A

Shepherd’s crook deformity

Soap bubble appearance of lytic lesions

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11
Q

List three types of cartilaginous benign bone tumour.

A
  • Osteochondroma
  • Enchondroma
  • Chondroblastoma
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12
Q

List three types of bone-forming benign bone tumour.

A
  • Osteoid osteoma
  • Osteoma
  • Osteoblastoma
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13
Q

What are osteochondromas and which bones tend to be affected?

A
  • A benign overgrowth of cartilage and bone that tends to happen at the ends of long bones
  • Most common site is distal end of femur
  • They mimic normal tubular bone as they have a cartilaginous surface overlying normal trabecular bone
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14
Q

What is an enchondroma and which bones tend to be affected?

A
  • A cartilaginous proliferation within the bone
  • Most tend to be found in the hands and can cause pathological fractures
  • X-ray may show popcorn calcification
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15
Q

What are two macroscopic features of benign bone tumours?

A
  • Well demarcated
  • May erode through the cortex of bone but does not burst through the cartilaginous surface
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16
Q

What are giant cell tumours? Where do they tend to be found and what is their histological appearance?

A
  • Benign tumour of the bone characterised by the presence of lots of osteoclast giant cells
  • They tend to be found at the ends of long bones (epiphysis)
  • It has a lytic appearance on X-ray
  • Histology shows many osteoclasts on a background of spindle/ovoid cells
  • Borderline malignancy - locally aggressive, can metastasise
17
Q

What is the most common type of malignant bone tumour?

A

Metastases

18
Q

Which cancers in adults tend to spread to the bone?

A
  • Breast
  • Prostate
  • Lung
  • Kidney
  • Thyroid
19
Q

Which cancers in children tend to spread to the bone?

A
  • Neuroblastoma
  • Wilm’s tumour
  • Osteosarcoma
  • Ewing’s sarcoma
  • Rhabdomyosarcoma
20
Q

List three types of malignant primary bone tumour. Which is the most common?

A
  • Osteosarcoma (most common)
  • Chondrosarcoma
  • Ewing’s sarcoma/PNET
21
Q

What is an osteosarcoma, what age group is it most common in, and where does it tend to affect?

A
  • Malignant bone-forming tumour of the bone that mainly occurs at the ends of long bones
  • Tends to occur at age 10-30 years
  • 60% affect the knee
22
Q

Describe the X-ray appearance of osteosarcoma. What is the name of the pathognomic X-ray sign?

A
  • Usually metaphyseal
  • Lytic
  • Elevated periosteum (Codman’s triangle)
23
Q

Describe the histological appearance of osteosarcoma.

A
  • Lots of malignant osteoblast cells with or without bone and cartilage formation

NOTE: this can be stained for using ALP

24
Q

How can osteosarcoma be classified?

A
  • Site within the bone (intramedullary, intracortical, surface)
  • Degree of differentiation (high, intermediate, or low grade)
  • Multicentricity
  • Primary or secondary
25
Q

What is a chondrosarcoma? Which age group does it affect?

A
  • Malignant cartilage-producing tumour
  • Tends to occur in patients aged > 40 years
26
Q

Describe the X-ray appearance of chondroscarcoma.

A

Lytic with fluffy calcification

27
Q

Describe the histology of chondrosarcoma

A

Malignant chondrocytes +/- chondroid matrix

28
Q

List the histological subtypes of chondrosarcoma.

A
  • Conventional (myxoid or hyaline)
  • Clear cell (low grade)
  • Dedifferentiated (high grade)
  • Mesenchymal

NOTE: myxoid = composed of clear, mucoid substance

29
Q

What is an Ewing’s sarcoma? What ages does it predominantly affect?

A
  • Highly malignant tumour arising from neuroectodermal cells
  • Occurs in people < 20 years old
30
Q

Describe the X-ray appearance of Ewing’s sarcoma.

A
  • Onion skinning of the periosteum
  • Lytic with or without sclerosis
31
Q

Describe the histological appearance of Ewing’s sarcoma.

A

Sheets of small round cells

32
Q

Which genetic abnormality is associated with Ewing’s sarcoma?

A

Chromosomal translocation 11;22 (EWSR1/FLI1) (q24:q12)

33
Q

Describe the prognosis of osteosarcoma, chondrosarcoma, and Ewing’s sarcoma

A

Osteosarcoma: 60% 5 year survival
Chondrosarcoma: 70% 5 year survival
Ewing’s: 75% 5 year survival

34
Q

Define soft tissue tumour.

A

Mesenchymal proliferations which occur in the extra-skeletal, non-epithelial tissues of the body - excluding meninges and lymphoreticular system

35
Q

Where do soft tissues occur and which age group do they predominantly affect?

A

Anywhere - majority occur in large muscles of extremeites, chest wall, mediastinum, retroperitoneum

Predominantly affect >55 years

36
Q

List three types of soft tissue tumour.

A
  • Liposarcoma
  • Spindle cell sarcoma
  • Pleomorphic sarcoma
37
Q

List some special diagnostic techniques used to diagnose soft tissue tumours.

A
  • Immunohistochemistry
  • Electron microscopy
  • Cytogenetic
  • FISH
  • RT-PCR
38
Q

List some bad prognostic factors for soft tissue tumours.

A
  • > 5 cm size
  • Depth involving deep fascia
  • High grade
  • Involved excision margin
  • Vascular invasion
  • Ploidy (aneuploid/hyperdiploid)
  • High proliferation index
  • Absent tumour suppression genes
  • Present tumour promotor genes
39
Q

Which conditions have these following X-ray features:
- soap bubble lytic lesions
- shepherd’s crook
- popcorn calcifications
- Codman’s triangle
- lytic with fluffy calcifications
- periosteal onion-skinning

A
  • fibrous dysplasia, giant cell tumour of bone
  • fibrous dysplasia
  • enchondroma
  • osteosarcoma
  • chondrosarcoma
  • Ewing’s sarcoma