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Pathology: Bone & Soft Tissue Sarcomas > Osteosarcoma > Flashcards

Osteosarcoma Flashcards

1
Q

What is the epidemiology of osteosarcoma?

A

Osteosarcoma is the most common primary bone tumor in children and young adults.

It comprises more than half of all pediatric primary skeletal malignancies.

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2
Q

What age group is most affected by osteosarcoma?

A

Osteosarcoma most often occurs in people under 20 years old, with teens being the most commonly affected age group.

Osteosarcomas can develop at any age.

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3
Q

How common is osteosarcoma in adults?

A

Osteosarcoma is the second most common type of primary bone tumor in adults.

It is most common in males.

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4
Q

What is osteosarcoma?

A

Osteosarcoma is a type of bone cancer.

The cause of most osteosarcomas is not clear.

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5
Q

What age group is at the highest risk for osteosarcoma?

A

The highest risk is for those between the ages of 10 and 30, especially during the teenage growth spurt.

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6
Q

How does age affect the risk of osteosarcoma?

A

The risk goes down in middle ages but rises again in older adults (over age of 60).

Osteosarcoma in older adults is often linked to another cause such as a long-standing bone disease.

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7
Q

What is a common characteristic of children with osteosarcoma?

A

Children with osteosarcoma are usually tall for their age.

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8
Q

Which gender is more likely to develop osteosarcoma?

A

Males are more likely to develop osteosarcoma.

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9
Q

Which races/ethnicities are at higher risk for osteosarcoma?

A

African-American and Latino individuals are at higher risk.

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10
Q

What previous medical exposure is a risk factor for osteosarcoma?

A

Previous radiation exposure is a risk factor.

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11
Q

What bone disease is associated with an increased risk of osteosarcoma?

A

Paget’s disease is associated with an increased risk.

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12
Q

What genetic conditions are linked to osteosarcoma?

A

Retinoblastoma, Li-Fraumeni syndrome, and Rothman-Thomson syndrome are linked to osteosarcoma due to mutations of the p53 gene.

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13
Q

What is the most common presenting symptom of osteosarcoma?

A

Pain

Pain may occur even without a mass that can be felt.

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14
Q

How do patients with osteosarcoma typically describe their pain?

A

Patients usually experience nonspecific pain and swelling that becomes progressively worse over several months.

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15
Q

What are other common presenting symptoms of osteosarcoma?

A

Mass, Decreased Range of motion, Fracture

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16
Q

Where does osteosarcoma most commonly occur?

A

In the long bones around the knee

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17
Q

What are the most common sites for osteosarcoma?

A
  1. Distal Femur (near metaphyseal plate)
  2. Proximal tibia
  3. Proximal humerus
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18
Q

What is important to determine the duration of symptoms in osteosarcoma?

A

A complete history of the patient is important.

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19
Q

What imaging is used at the primary tumor site for osteosarcoma?

A

X-ray of primary tumor site.

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20
Q

How does osteosarcoma appear on an X-ray?

A

It appears as a tumor permeating the medullary cavity with a classic ‘onion skin’.

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21
Q

What imaging is used to rule out lung metastasis in osteosarcoma?

A

Chest CT.

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22
Q

What is the key indicator of malignancy on a radiograph?

A

The characteristic of the margin.

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23
Q

What percentage of cases with well-defined sclerotical borders are malignant?

A

A legion with well-defined sclerotical borders is malignant in only 6% of cases.

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24
Q

Why is MRI used in instances of aggressive bone tumors?

A

Because of the accuracy in distinguishing healthy tissues and neurovascular structures from the tumor tissue.

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25
Q

What is essential in planning a surgical biopsy and treatment?

A

MRI is essential.

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26
Q

What does MRI demonstrate in relation to tumors?

A

MRI is highly sensitive, demonstrating the reactive zone of the tumor in the bone while differentiating marrow edema adjacent to tumor tissue.

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27
Q

What future imaging technique may be used in the diagnosis and staging of primary bone tumors?

A

PET/MRI.

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28
Q

What is the benefit of PET/MRI in osteosarcoma?

A

It is an excellent tool for evaluation of tumor size before surgery and can be used to monitor progress after neoadjuvant therapy.

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29
Q

What is the sensitivity of bone scans in detecting tumors?

A

Bone scans are extremely sensitive and can detect tumors in bone that are not yet visualized on diagnostic radiographs.

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30
Q

What is the chance of an osteolytic lesion with less defined edges being malignant?

A

An osteolytic lesion with less defined edges on a radiograph has a 50% chance of being malignant.

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31
Q

What is the most important step in confirmation of a pathologic diagnosis?

A

Biopsy for confirmation of diagnosis.

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32
Q

What must be discussed with the surgeon and radiation oncologist before a biopsy?

A

The biopsy site and approach.

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33
Q

What are the preferred methods for biopsy?

A

Typically, a core needle biopsy or open biopsy is the preferred method.

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34
Q

What is one disadvantage of open biopsy?

A

The risk of tumor cell seeding.

35
Q

What is osteosarcoma?

A

Osteosarcoma is a type of primary bone tumor.

36
Q

What is the significance of pathologic staging and grading in osteosarcoma?

A

Pathologic staging and grading are closely linked to anatomic staging systems and are significant prognostic factors.

37
Q

What are the grades of osteosarcoma tumors?

A

Tumors are classified as either low grade (G1) or high grade (G2).

38
Q

How do low grade and high grade tumor cells differ?

A

Low grade tumor cells resemble normal cells and are less likely to grow and spread quickly, while high grade tumor cells appear more abnormal.

39
Q

What factors classify osteosarcoma tumors?

A

Tumors are classified based on grade, tumor size (greater or less than 8cm), and the presence or absence of distant metastases.

40
Q

What are the histologic subtypes of osteosarcoma?

A

The histologic subtypes include osteoblastic, chondroblastic, fibroblastic, and mixed.

41
Q

What is the Enneking staging system?

A

The Enneking staging system was previously considered the standard classification system.

42
Q

What has updated the Enneking staging system?

A

It has been updated by the AJCC to replace compartmentalization with tumor size.

43
Q

How are osteosarcomas generally classified?

A

Osteosarcomas are generally classified as poorly differentiated high grade tumors.

44
Q

What is the most aggressive subtype of osteosarcoma?

A

The osteoblastic subtype is the most aggressive and is considered high grade.

45
Q

What is the histologic classification of the chondroblastic subtype?

A

The chondroblastic subtype is considered intermediate grade and includes periosteal lesions.

46
Q

How is the fibroblastic subtype classified?

A

The fibroblastic subtype is a low grade subtype that commonly presents as a parosteal tumor.

47
Q

What is the staging system for osteosarcomas?

A

The Enneking staging system

48
Q

How are osteosarcomas generally classified?

A

Osteosarcomas are generally classified as poorly differentiated high grade tumors.

49
Q

What are the histologic subtypes of osteosarcoma?

A

The histologic subtypes of osteosarcoma include:
1. Osteoblastic - most aggressive subtype and is considered high grade.
2. Chondroblastic - considered intermediate grade in which periosteal lesions are histologically classified.
3. Fibroblastic - low grade subtype which commonly presents as a parosteal tumor.

50
Q

What is the most common site of metastasis in osteosarcoma?

A

The lungs.

51
Q

What percentage of patients with high-grade osteosarcoma develop lung metastases within 2 to 3 years?

A

Approximately 90%.

52
Q

What are skip metastases in osteosarcoma?

A

A second, smaller focus of osteosarcoma in the same bone or a second bone lesion on the opposing side of a joint space.

53
Q

What contributes to the phenomenon of skip metastases?

A

The extensive spread by the lesion into the marrow cavity of the bone.

54
Q

Why is control of bone tumors difficult?

A

Due to the overall aggressiveness of bone tumors.

55
Q

When is lymphatic spread of bone tumors a concern?

A

When the tumor arises in the trunk of the body.

56
Q

What increases the chance of lymphatic invasion by bone tumors?

A

The prominence of lymph vessels and nodes in the trunk of the body.

57
Q

What can happen if tumor cells invade the lymphatic system?

A

Microscopic tumor cells can be carried to other parts of the body through the lymphatic system.

58
Q

What is the treatment approach for osteosarcoma?

A

The treatment requires a multidisciplinary approach due to its chemo-sensitivity and radioresistance.

59
Q

What does the current accepted treatment for osteosarcoma consist of?

A

It consists of neoadjuvant, multiagent chemotherapy, surgical resection of the primary tumor, followed by additional chemotherapy.

60
Q

What was the historical treatment for the primary lesion in osteosarcoma?

A

Historically, the treatment was amputation, which achieved excellent local control but had unsatisfactory outcomes for life and functionality.

61
Q

How have amputation rates changed over the years for osteosarcoma treatment?

A

Amputation rates have declined due to the evolution of new chemotherapy treatments, leading to a shift towards limb-sparing surgery.

62
Q

What is limb-sparing surgery in the context of osteosarcoma?

A

In limb-sparing surgery, the bone involved with the tumor is removed and reconstructed with an implant.

63
Q

Is radiation part of the standard treatment regimen for osteosarcoma?

A

No, radiation is not part of the standard treatment regimen for patients with osteosarcoma.

64
Q

What is the treatment overview for Osteosarcoma?

A

Multidisciplinary approach including neoadjuvant chemotherapy, limb-sparing surgery, and adjuvant chemotherapy.

65
Q

What are the key components of adjuvant chemotherapy for Osteosarcoma?

A

Cisplatin, Doxorubicin, Methotrexate with Leucovorin.

66
Q

What is a characteristic of Osteosarcoma regarding radiation treatment?

A

It is radioresistant with no definitive radiation treatment.

67
Q

What is advised for patients with positive surgical margins and partial resections who are nonsurgical candidates?

A

External beam therapy is advised for these patients.

68
Q

What is the postoperative dose for high-risk anatomic sites in osteosarcoma treatment?

A

64-68 Gy to the high-risk anatomic site.

69
Q

What is the dose for unresectable tumors in osteosarcoma treatment?

A

70 Gy.

70
Q

What are common side effects of radiation therapy?

A

Skin reactions, reduced blood counts, and fractures.

71
Q

What complications can arise from the treatment of osteosarcoma?

A

Nephrotoxicity and neurotoxicity from chemotherapy.

72
Q

When are more side effects typically experienced in osteosarcoma treatment?

A

More side effects are normally experienced when several modalities are used in combined sequence.

73
Q

What is the most important prognostic indicator for osteosarcoma?

A

The presence or absence of metastases at the time of diagnosis.

74
Q

What percentage of osteosarcoma patients have distant metastasis at presentation?

A

Approximately 15-20% of patients.

75
Q

How does gender affect the prognosis of osteosarcoma?

A

Prognosis is typically worse in males.

76
Q

What age group has a worse prognosis for osteosarcoma?

A

Males younger than 10 years of age.

77
Q

How does the duration of symptoms affect prognosis?

A

Prognosis is typically worse if the duration of symptoms is less than 6 months.

78
Q

What factors are considered in the prognosis of osteosarcoma?

A

Grade and stage at time of diagnosis, duration of symptoms, and interval between chemotherapy and surgery.

79
Q

What is the overall 5-year survival rate for osteosarcoma?

A

63%

80
Q

What is the 5-year survival rate for males with osteosarcoma?

A

59%

81
Q

What is the 5-year survival rate for females with osteosarcoma?

A

70%

82
Q

What is the 5-year survival rate for patients with no metastasis?

A

70%

83
Q

What is the 5-year survival rate for patients with metastasis?

A

30%

Pathology: Bone & Soft Tissue Sarcomas (14 decks)

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