PAMS: Ortho- Bone Tumors Flashcards

1
Q

What are the origins of Primary Malignant Bone tumours?

A
  • Hematopoietic > 40%
  • Chondrogenic > 20%
  • Osteogenic > 20%
  • Unknown 10%
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2
Q

What are the 3 Most Common Malignant tumours of
Bone ?

A
  1. Osteosarcoma
  2. Chondrosarcoma
  3. Ewing sarcoma
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3
Q

What are the risk factors for osteosarcoma ?

A
  • Retinoblastoma
  • Hx of radiation tx
  • Hx of high bone turnover (e.g., Paget Disease of Bone)
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4
Q

What are the most common locations of osteosarcoma ?

A

Distal femur and proximal tibia = 60%
Proximal femur =15%
Proximal humerus = 10%
Mandible = 8%

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5
Q

What is the epidemiology of osteosarcoma ?

A

It is more common in boys than girl. It accounts for 50% of bone cancers in those <20 years. It is also the 5th most common cancer between 15-19 year olds.

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6
Q

What are the symptoms of osteosarcoma?

A

Dull aching pain over the involved area, night pain and growing pain with or without B symptoms.

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7
Q

What are the signs of Osteosarcoma ?

A

Patients often have tenderness to palpation on the involved area. Limited AROM of adjacent joints. They may also present with or without swelling, mass, deformity or Spontaneous fracture.

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8
Q

What are the imaging studies in osteosarcoma?

A
  • Regional MRI and CT helpful for
    guiding surgical repair.
  • Evaluation for metastatic pulmonary
    disease
  • Pre-op: All patients get chest CT
  • Post-op: Repeat chest CT q 3-6 mo x 2 yr.
  • Bone scintigraphy: May show distant metastatic lesions or local invasion.
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9
Q

What is the significance of biopsy in osteosarcoma?

A

Percutaneous needle biopsy is sufficient to make the diagnosis and staging.

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10
Q

What is the Enneking System for Staging Osteosarcomas?

A

Stage 1: Low grade no mets,
1A- intra compartmental and 1B extra compartmental.
Stage 2: High grade no mets, IIA intra compartmental and IIB extra compartmental.
Stage 03: Low or High grade with Mets.

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11
Q

What is the Tx of osteosarcoma ?

A

Methotrexate, Doxorubicin, and Cisplatin for 10 week, Surgery on the 11th week, followed by same chemo protocol for a year.

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12
Q

What should be the post surgical follow up in osteosarcoma patients ?

A

There is 70 to 85% long-term survival rate in osteosarcoma. However relapses are common therefore monitoring as follows is essential:
* In the first year blood work and imaging every 3 months.
* Same screening continued every 6 months for the next 2 years.
* Then yearly.
* Chest CT must be done every 6 months for the first 2 years due to the risk of mets into the lungs.

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13
Q

What is the prognosis of Osteosarcoma?

A
  • 5-Year survival – 70%
  • Influenced by metastases at time of diagnosis
  • Response to neoadjuvant chemo is major prognostic indicator
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14
Q

What is the pathophysiology of osteosarcoma?

A

The pathogenesis of osteosarcoma consist of RB and tp53 mutations triggering uncontrolled proliferation of pleomorphic osteoblasts around the metaphysis of the long bones such as tibia and fibula leading to the symptomatology and radiological sunburst appearance and Codman’s triangle.

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15
Q

What is a chondrosarcoma ?

A

It is a cartilaginous tumor and is the 3rd most common bone malignancy after multiple myeloma and osteosarcoma. 90% are conventional chondrosarcomas followed by dedifferentiated, clear cell, mesenchymal, and juxtacortical subtypes. Secondary chondrosarcoma can arise from enchondroma or osteochondromas.

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16
Q

what are the grades of Chondrosarcoma?

A

Grade I (low grade)
* Grade II (intermediate grade)
* Grade III (high grade)

17
Q

What is the epidemiology of Chondrosarcoma?

A

It is equally seen in both genders between the age of 50 and 70.

18
Q

What is the clinical presentation of Chondrosarcoma?

A
  • Deep, dull, achy pain
  • Chronic (> 1 year)
  • Pain at night
19
Q

what is the distribution of chondrosarcoma ?

A

27% on Pelivs, 13% on ribs, 12% on proximal femur, 7% on proximal humerus, 6% on distal femur and 4% on proximal tibia.

20
Q

What is the Tx and prognosis of Chondrosarcoma ?

A
  • Surgical resection
  • Does not respond well to chemo or radiation
  • Prognosis
  • Grade I tumours - 90% survival at 5 years
  • Grade II tumours - 81% survival at 5 years
  • Grade III tumours - 29% survival at 5 years
21
Q

What is the epidemiology and pathophysiology of Ewing’s sarcoma ?

A

It is seen in patients between the age of 5-30. The oncogenesis is mediated by the fusion gene formed from t11,22 translocation.

22
Q

What is the presentation of Ewing sarcoma ?

A

Bone pain and B symptoms with tender palpable mass on examination.

23
Q

What are the imaging findings in Ewing’s sarcoma ?

A

Sun burst sign, Onion skin sign and moth eaten pattern.

24
Q

What is the diagnostic work-up in Ewing’s sarcoma ?

A

Blood works will show elevated WBC, ESR and LDH.
Biopsy for histopathological confirmation and MRI for staging.

25
Q

What is the Tx of Ewing’s sarcoma ?

A

The Tx is surgery, radiation and chemo. The 5 year survival in local disease is 70% and metastatic disease is 30%.

26
Q

What is the pathogenesis and clinical Hx of ganglion cyst?

A

It is caused by the destruction of mucoid connective tissue. Patient’s will typically complaint of joint instability, weakness and limited ROM.

27
Q

What are the physical examination findings in Ganglion cysts?

A
  • Normal overlying skin
  • Compressible, movable mass
  • Transilluminates
  • May be adherent to bone, joint, or tendon
28
Q

What is the Tx of Ganglion cyst?

A

It often doesn’t require any Tx. If needs to be treated :
* Noninvasive
* Compression
* Aspiration
* Surgical
* Open excision
* Requires post-op immobilization x7 days
* Recurrence
* Ranges from 4% to 40%

29
Q
A
30
Q

What is the genetic mutation in Ewing sarcoma?

A

EWSR1- FLI1 fusion gene.

31
Q

What is the histopathological appearance of Ewing sarcoma?

A

Small round blue cells