Section 2: Pediatric Cancers - Osteosarcoma

Front 1

Define osteosarcoma

Back 1

A primary malignant tumor of the bone

Front 2

How is osteosarcoma distinguished from other bone tumors?

Back 2

Production of osteoid substance (osteoblastic, chondroblastic, & fibroblastic differentiation)

Front 3

In what population is Osteosarcoma considered the most common bone tumor?

Back 3

Children

Front 4

T or F: After leukemia & lymphoma, osteosarcoma is the 3rd most frequent neoplasm in adolescents & young adults

Back 4

True - 5.6 cases per 1 million children of European ancestry <15yrs in US/yr

Front 5

During what period of growth is the peak incidence of osteosarcoma?

Back 5

in second decade of life, during adolescent growth spurt

Front 6

Does osteosarcoma occur earlier in boys or girls?

Back 6

Girls - they have growth spurt earlier

Front 7

Is Osteosarcoma more common in girls than boys?

Back 7

No, boys are more common d/t large bone volume

Front 8

What percentage of Osteosarcoma is identified in bone tumors in children & adolescents?

Back 8

56%

Front 9

What percentage of osteosarcomas result from irradiation?

Back 9

3% - applies to older population, 4 to 40yrs (median is 12-16yrs) after radiation

Front 10

In what premalignant condition will 2% of the patient's develop Osteosarcoma after age 50?

Back 10

Those w/Paget's disease

Front 11

What 2 other hereditary conditions have a predisposition to develop osteosarcoma?

Back 11

1. Hereditary Retinoblastoma
2. Carriers of p53 gene mutation

Front 12

What gene mutation does LiFraumini refer to?

Back 12

p53 gene mutation

Front 13

Those with LiFraumini have what percentage of developing a malignancy by age 30? and what percentage develop a cancer by age 70?

Back 13

By Age 30 = 50% chance
By Age 70 = 90% chance
(usu. bone or breast cancer)

Front 14

Read table on Page 37

Back 14

Table 2-10: Causes of Osteosarcoma

Front 15

What is the common presentation of Osteosarcoma?

Back 15

Dull, aching, and constant pain.
Pain is worse at night
Average duration of pain is 3 months BEFORE diagnosis

Front 16

T or F: Patients will always have soft tissue-mass or swelling

Back 16

False: They may or may not have soft-tissue mass or swelling

Front 17

What bones are most commonly affected by Osteosarcoma?

Back 17

Long bones (distal femur, proximal tibia, humerus)

Front 18

About 15-20% of patients present with what lesions?

Back 18

Metastatic lesions (macrometastatic) majority in the lungs

Front 19

What prognosis is given to those with bone metastasis at time of diagnosis?

Back 19

Extremely poor prognosis

Front 20

Along with H&P and PE, what other diagnostic tests should be included in the evaluation of a patient w/Osteosarcoma?

Back 20

1. X-ray
2. Bone scan
3. CT of chest
4. MRI of area w/tumor
5. Labs (LDH & Alk Phos)
6. Arteriography
7. Biopsy of tumor

Front 21

What is a common radiologic finding in an Osteosarcoma tumor?

Back 21

1. Sunburst pattern = mixed regions of sclerosis & lytic lesions of bone
2. Codman's triangle

Front 22

What does a Codman's triangle refer to?

Back 22

An isolated cuff of reactive subperiosteal new bone at the boundary of any benign or malignant mass that rapidly elevates the periosteum

Front 23

What diagnostic test is always recommended when considering Osteosarcoma?

Back 23

Fine-needle, core, or open biopsy
(open is preferred in order get the best specimen)

Front 24

What type of diagnostic test can assess for increased uptake of radioisotope at tumor sites or areas of healing bone?

Back 24

Bone scan

Front 25

Why is a CT of the chest performed when Osteosarcoma has been diagnosed?

Back 25

Assess for pulmonary metastasis and their extent of disease

Front 26

What diagnostic test is used to assess for soft-tissue, nerve, and vessel involvement & tumor boundaries, when Osteosarcoma has been diagnosed?

Back 26

MRI

Front 27

What purpose does an arteriography have when someone is diagnosed with Osteosarcoma?

Back 27

May help determine extent of blood or vascular flow to tumor

Front 28

What is the most important prognostic factor in Osteosarcoma diagnosis?

Back 28

Extent of disease at diagnosis

Front 29

What do pulmonary metastasis indicate at the time of diagnosis?

Back 29

1. Greatly decreases survival time
2. Impact the mgmnt of disease

Front 30

What dictates the surgical procedure of an Osteosarcoma tumor?

Back 30

Location, location, location
Limb salvage vs resection

Front 31

Why does Spinal Osteosarcoma typically have a poor prognosis?

Back 31

Usu. cannot resect entire tumor

Front 32

What size of tumor has been associated with dismal prognosis?

Back 32

Any tumor >15cm

Front 33

What other demographic qualities are typically assoc. w/poor prognosis?

Back 33

<10 years of age
Males
Elevated Alk Phos in blood & tumor

Front 34

What response to preoperative chemotherapy typically have a more favorable outcome?

Back 34

>98% necrosis of tumor

Front 35

Why is chemotherapy & surgery an important combination in the treatment of osteosarcoma?

Back 35

Presume all patients have microscopic metatstases
- 80% pts w/only surgery WILL develop metastasis

Front 36

What chemotherapy is used to treat Osteosarcoma?

Back 36

1. High Dose MTX (Mexate)
2. Doxorubicin (Adriamycin)
3. Cisplatin (platinol)
4. Ifosfamide (Ifex)
5. Etoposide (VP16)

Front 37

After initial chemotherapy, what is the next step in treatment?

Back 37

Surgery for tumor resection & reconstruction of limb
More chemo after surgery

Front 38

What is the overall survival rate of Osteosarcoma?

Back 38

65-70%

Front 39

Is osteosarcoma a radiosensitive or radioresistant tumor?

Back 39

Radioresistant
- radiation can be used for metastatic disease or palliation

Front 40

How are lung metastases treated?

Back 40

Resection and possible radiation to areas of tumors

Front 41

What is the Event-Free-Survival (EFS) rate for a pt. w/lung metastases?

Back 41

20-30%

Front 42

What is the goal with surgical resection of the tumor?

Back 42

Obtain wide margin = a zone of 5mm or more of normal healthy tissue - around the tumor

Front 43

Is the limb functional outcome the same with limb salvage surgery vs amputation?

Back 43

Yes - they achieve similar functional outcomes

Front 44

What is involved in the limb salvage surgical procedure?

Back 44

Endoprosthetic device is implanted after diseased bone is removed
(i.e., total knee or hip joint replacement)

Front 45

What will 10% of limb salvage patients have as a result of the surgery?

Back 45

Mechanical failure d/t regular use, and need for replacement procedures

Front 46

What is an arthrodesis surgery?

Back 46

Fusion of the joint by replacing joint with allograft or cadaver bone

Front 47

What diseased bones are considered for amputation surgery?

Back 47

Expendable bones (fibula, rib, toe, finger, or ulna)

Front 48

What does rotationplasty surgery entail?

Back 48

Excision of the femur w/preservation of lower leg.
Lower leg is rotated 180 degrees and foot become knee joint.
Similar to a below-the-knee amputation

Front 49

What are 2 new therapies that are being tested for treatment to improve outcomes?

Back 49

1. Monoclonal antibodies (Herceptin/Trastuzumab)
2. Inhaled GMCSF for lung mets

Front 50

What routine monitoring is needed to detect recurrent disease early?

Back 50

Routine X-ray, chest & affected limb

Front 51

What time frame does recurrent disease typically present itself?

Back 51

Within 3 years from initial diagnosis

Front 52

If a recurrence appears in less than how many years, is there less possibility of a cure?

Back 52

< 2years after therapy is discontinued

Front 53

What percentage of patients have recurrent disease that presents in the lungs?

Back 53

85%

Front 54

Read page 38

Back 54

Table 2-4 : Osteosarcoma 5-year Relative Survival Rates