NEOPLASMS OF BONE AND CARTILAGE Flashcards

1
Q

State the pathogenesis of osteosarcoma.

A
  • Mutation to retinablastoma (RB) gene: a proto-oncogene that regulate cycle
  • Mutation to p53, a gene whose product regulate DNA repair
  • CDKN2A inactivation
  • MDM2 and CDK4 overexpression.
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2
Q

List the risk factor of osteosarcoma.

A
  • Bone infarcts
  • Paget disease
  • Post radiation
  • Chronic osteomyelitis
  • Metal prostheses
  • Retinoblastoma and Li- Fraumeni syndrome (mutations in the genes encoding RB & p53)
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3
Q

Desc the clinical manifestations of osteosarcoma.

A
  • Painful
  • Progressively enlarging masses
  • Decrease range of motion of the involved joint
  • Systemic symptoms
  • Respiratory symptoms
  • Lymphadenopathy
  • Arise in the metaphyseal region of the long bones of the knee
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4
Q

Desc the morphology of osteosarcoma

A
  1. RADIOLOGY
    - Large destructive
    - Mixed lytic and blastic mass with infiltrate margins
    - Sun- burst appearance
    - Codman triangle
  2. MACROSCOPY
    - Irregular tan-white mass arising within the metaphysis of proximal tibia
    - Firm and tan-white
    - Infiltrating/ extending into surrounding soft tissue
    - Bulky tumour at distal femur
    - Metaphysis and diaphysis region extend to surrounding soft tissue
    - Heterogenous pale white cut surface
    - Haemorrhage
    - Necrosis
  3. MICROSCOPY
    - Cells vary in size and shape(pleomorphic)
    - Have large hyperchromatic nuclei
    - Bizarre tumour giant cell
    - Necrosis
    - Mitosis
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5
Q

Desc the clinical manifestations/ complications of osteosarcoma.

A
  • Painful, progressively enlarging masses
  • Decreased range of motion of the involved joint
  • Pathological fracture
  • Systemic symptoms: Fever, loss of weight,
  • Respiratory symptoms if tumour metastasise to lungs
  • Lymphadenopathy if tumour metastasise to lymph node
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6
Q

State the radiology, gross and microscopy findings of osteosarcoma.

A

RADIOLOGY FINDING:

  • Large destructive
  • Sun burst appearance
  • Codman triangle

GROSS:

  • Irregular tan-white mass arising within the metaphysis of proximal tibia
  • Firm and tan- white
  • Infiltrating/ extending into surrounding soft tissue
  • Necrosis, haemorrhage
  • Bulky tumor at distal femur

MICROSCOPY:

  • Pleomorphic
  • Hyperchromatic
  • Bizarre tumor giant cells
  • Necrosis
  • Mitosis
  • The formation of bone (osteiod) bu the tumor cells is characteristics coarse, lace like architecture.
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7
Q

What is the clinical features of osteiod osteomas?

A
  • Metaphysis or diaphysis of the long bones
  • Occur in the bone cortex
  • Painful
  • Can be relieved by aspirin
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8
Q

State the morphology of osteoid osteomas.

A

Resected tumour:
The actual tumour knows as the nidus. Based or radiology findings, it shows a small round lucency that may be centrally mineralised.

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9
Q

State the microscopy of osteoid osteoma and osteoblastoma.

A

Composed of randomly interconnecting trabeculae of woven bone which prominently rimmed by osteoblasts

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10
Q

State the difference of osteoid osteoma and osteoblastoma.

A

Osteoid osteoma:

  • Small, < 2 cm in dimension
  • Mainly in the cortex of femur @ tibia
  • Severe nocturnal pain and can be relieved by aspirin

Osteoblastoma:

  • > than 2cm
  • Involved the spine more frequently
  • The pain is dull, achy, and unresponsive to salicylates.
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11
Q

What is chondrosarcomas?

A

It is a malignant cartilage forming tumour. Mainly, it occur in their 40s or older. The tumor affect men twice as frequently as women. The risk factor of chondrosarcomas is arise from a preexisting chondroma or osteochondroma.

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11
Q

What is the clinical features of chondrosarcomas?

A

These tumors usually present as painful, progressively enlarging masses. It mainly arise in the central portions of the skeleton, including the pelvis, shoulder and ribs. In contrast to chondroma, chondrosarcoma rarely involved the distal extremities.

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12
Q

State the radiology findings and the biologic behavior in chondrosarcomas.

A

RADIOLOGY FINDINGS

  • Prominent endosteal scalloping due to nodular growth pattern of the cartilage.
  • Foci of flocculent densities due to calcified matrix
  • Reactive thickening of the cortex in a slow-growing, low- grade tumor.
  • Destroyed cortex with soft tissue mass in a more aggressive high grade neoplasm

BIOLOGIC BEHAVIOR
- Directly correlates with the grade of the tumor

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13
Q

State the macroscopy and microscopy of chondrosarcoma.

A

MACROSCOPY

  • large bulky tumor
  • Nodule of gray white, somewhat translucent glistening tissue
  • Tumor grows with broad pushing fronts into the surrounding soft tissue
  • Central necrosis
  • Spotty calcifications
  • The adjacent cortex is thickened or eroded

MICROSCOPY

  • Atypical chondrocyte: hyperchromatic nuclei, binucleated, multinucleated
  • Chondromyxoid background
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13
Q

State the macroscopy and microscopy of chondrosarcoma.

A

MACROSCOPY

  • large bulky tumor
  • Nodule of gray white, somewhat translucent glistening tissue
  • Tumor grows with broad pushing fronts into the surrounding soft tissue
  • Central necrosis
  • Spotty calcifications
  • The adjacent cortex is thickened or eroded

MICROSCOPY

  • Atypical chondrocyte: hyperchromatic nuclei, binucleated, multinucleated
  • Chondromyxoid background
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14
Q

What is osteochondroma?

A

Osteochondroma also known as exostosis is a benign cartilage capped tumor that is attached to the underlying skeleton by a bony stalk. It also can caused by hereditary which is mutation at the EXT1 @ EXT2. Mutation at EXT1 @ EXT2 will cause the defective endochondral ossification leading to abnormal growth.

15
Q

The risk factor and the clinical features of osteochondroma.

A

RISK FACTOR:

  • Usually occur at late adolescence and early adulthood by multiple osteochondroma become apparent during childhood.
  • Mainly in men

CLINICAL FEATURE:

  • Slow growing mass
  • Can be painful if they impinge on a nerve or if the stalk is fractured
  • Multiple hereditary exostosis: underlying bones may be bowed and shortened reflecting an associated disturbance in epiphyseal growth
  • Usually stop growing at the time of growth plate closure
16
Q

State the radiology, microscopic, and gross findings of osteochondromas.

A

RADIOLOGY

  • Bony protrusion, pedunculated
  • In continuous with femur bone

MICROSCOPIC
- Cartilage cap: disorganised growth plate- like cartilage

GROSS

  • Sessole or muschroom shaped
  • The cap is composed of benign hyaline cartilage varying in thickness
17
Q

State the clinical presentations of chondroma

A
  • Benign tumors of hyaline cartilage
  • Small bones of hands and feet
  • Arise within the medullary cavity (enchondroma)
  • Arise on the cortical surface (juxtacortical chondroma)
  • Occur in 20 to 50 years old
  • Solitary lesion
  • Ollier disease and Maffucci syndrome
17
Q

State the clinical presentations of chondroma

A
  • Benign tumors of hyaline cartilage
  • Small bones of hands and feet
  • Arise within the medullary cavity (enchondroma)
  • Arise on the cortical surface (juxtacortical chondroma)
  • Occur in 20 to 50 years old
  • Solitary lesion
  • Ollier disease and Maffucci syndrome
18
Q

State the clinical presentations of chondroma

A
  • Benign tumors of hyaline cartilage
  • Small bones of hands and feet
  • Arise within the medullary cavity (enchondroma)
  • Arise on the cortical surface (juxtacortical chondroma)
  • Occur in 20 to 50 years old
  • Solitary lesion
  • Ollier disease and Maffucci syndrome
19
Q

State the radiology and morphology findings of chondroma.

A

RADIOLOGY:

  • Circumscribed lucency with central irregular calcification
  • Sclerotic rim
  • Smaller than 3cm
  • Circumscribe nodules of hyaline cartilage containing benign chondrocytes
  • Gray-blue and translucent
  • Peripheral endochondral ossification
  • Central infarction and calcification
19
Q

State the radiology and morphology findings of chondroma.

A

RADIOLOGY:

  • Circumscribed lucency with central irregular calcification
  • Sclerotic rim
  • Smaller than 3cm
  • Circumscribe nodules of hyaline cartilage containing benign chondrocytes
  • Gray-blue and translucent
  • Peripheral endochondral ossification
  • Central infarction and calcification
19
Q

State the radiology and morphology findings of chondroma.

A

RADIOLOGY:

  • Circumscribed lucency with central irregular calcification
  • Sclerotic rim
  • Smaller than 3cm
  • Circumscribe nodules of hyaline cartilage containing benign chondrocytes
  • Gray-blue and translucent
  • Peripheral endochondral ossification
  • Central infarction and calcification
19
Q

State the radiology and morphology findings of chondroma.

A

RADIOLOGY:

  • Circumscribed lucency with central irregular calcification
  • Sclerotic rim
  • Smaller than 3cm
  • Circumscribe nodules of hyaline cartilage containing benign chondrocytes
  • Gray-blue and translucent
  • Peripheral endochondral ossification
  • Central infarction and calcification
20
Q

What is metastatic bone tumor and where does it effects?

A

Metastatic bone tumor usually develop in later stages of tumor progression.The pathway of spread include direct extension, lymphatic or hematogenous dissemination and intraspinal seeding. Mostly, it will affect the axial skeleton, proximal femur, and humerus in descending order of frequency.

21
Q

Briefly desc the radiology of metastatic bone tumour.

A

Skeletal metastases are typically multifocal. However, carcinomas of the kidney and thyroid are solitary lesion. It may be purely lytic, purely blastic or mixed both lytic and blastic. In lytic lesions, the metastatic cells secrete substances such as PG, Cytokines, and PTH- related protein that upregulate RANKL on osteoblast and stromal cells to stimulate osteoclastic bone resorption. Lysis of bone tissue rich in growth factors help the tumor cell to grow. However, blastic lesion secrete WNT proteins that stimulate osteoblastic bone formation.

22
Q

State the morphology of metastatic tumor.

A
  • Mainly occur at prostate, breast, kidney, thyroid, lung
  • Multiple irregular nodules within vertebrae
  • Destruction of vertebral bodies
  • Haemoorhage
  • Necrosis
  • Large tan mass with adjacent small nodule
  • Heterogenous cut surface
  • Glandular formation and nest
  • Bone trabecula seen
  • Atypical cells lining the gland
  • Hyperchromatic, mildly pleomorphic nuclei
  • Prominent nucleolus
  • High N:C ratio
  • Mitosis