Ewing Sarcoma Flashcards

1
Q

It is the most common primary malignant bone tumor in children.

a. Ewing sarcoma
b. Osteosarcoma
c. Rhabdomyosarcoma
d. Chondrosarcoma

A

B

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2
Q

It causes a dramatic shrinkage of the soft tissue mass and rapid significant pain relief.

A

Chemotherapy

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3
Q

It is the standard chemotherapy for osteosarcoma.

a. cyclophosphamide, etoposide, doxorubicin
b. vincristine, ifosfamide, doxorubicin
c. methotrexate, doxorubicin, cisplatin
d. doxorubicin, ifosfamide, cisplatin

A

C

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4
Q

It is a cell surface marker for Ewing sarcoma.

a. p30/32
b. p51/53
c. CD4
d. CD8

A

A - p30/32

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5
Q

It is a characteristic of Ewing’s sarcoma cells which is detected by staining with periodic acid-Schiff.

A

glycogen-filled cytoplasm

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6
Q

The classic cytogenic abnormality associated with Ewing sarcoma.

a. chromosomal amplification of region 12q13-q15
b. reciprocal translocation of the long arms of chromosomes 11 and 22
c. chromosomal anomalies in 9p21, 10, 13q14, and 17p13
d. inactivated CDKN2A which encodes tumor suppressors, p16 and p14

A

B

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7
Q

It has a predilection for metaphyses of long bones wherein the most common sites of involvement are the distal femur, proximal tibia, and proximal humerus.

a. Ewing sarcoma
b. Chondrosarcoma
c. Osteosarcoma
d. Rhabdomyosarcoma

A

C

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8
Q

The following are benign tumors of the bone of cartilage origin except:

a. osteoid osteoma
b. osteochondroma
c. enchondroma
d. chondroblastoma

A

A.

Common benign tumors of the bone
Cartilage origin: enchondroma, osteochondroma, chondroblastoma, chondromyxoid fibroma
Bone origin: osteoid osteoma and osteoblastoma
Fibrous tissue origin: fibroma and desmoplastic fibroma
Vascular origin: hemangioma
Unknown origin: giant-cell tumor

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9
Q

(TRUE or FALSE) The most common malignant tumors of bone are plasma cell tumors.

A

TRUE

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10
Q

The four most common malignant nonhematopoietic bone tumors are the following, except:

a. rhabdomyosarcoma
b. osteosarcoma
c. Ewing’s sarcoma
d. chondrosarcoma

A

A

UPS: Undifferentiated pleomorphic sarcoma

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11
Q

Based on the Musculoskeletal Tumor Society Staging System, at what stage is a bone sarcoma described as high grade and intracompartmental?

a. Stage IA
b. Stage IIIB
c. Stage IIA
d. Stage IVB

A

C

Musculoskeletal Tumor Society Staging System:
Stage I - low grade
Stage II - high grade
Stage III - any grade that have lymph node or distant metastases
A: intracompartmental (same soft tissue compartment as the initial tumor)
B: extracompartmental (extending into adjacent soft tissue compartment or into bone)

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12
Q

A radiograph finding of a destructive lesion with moth-eaten appearance, a spiculated periosteal reaction and a cuff of periosteal new bone formation at the margin of the soft tissue mass is a characteristic of:

a. Ewing sarcoma
b. Chondrosarcoma
c. Osteosarcoma
d. Rhabdomyosarcoma

A

C

sunburst appearance: periosteal reaction
Codman’s triangle: cuff of periosteal new bone formation at the margin of the soft tissue mass

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13
Q

It is used to get the image of the metastases to the bony skeleton.

a. bone scan
b. CT scan
c. FDG-PET
d. two of the options
e. all of the options

A

D

bone scan and fluorodeoxyglucose positron emission tomography

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14
Q

It is the standard management for osteosarcoma.

a. chemotherapy - limb-sparing surgery - chemotherapy
b. limb-sparing surgery - chemotherapy - radiation therapy
c. chemotherapy - limb-sparing surgery - radiation therapy
d. radiation therapy - limb-sparing chemotherapy

A

A

osteosarcoma is radioresistant; radiation therapy has no role in the routine management

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15
Q

It has a predilection for the flat bones, especially the shoulder and pelvic girdles, but can also affect the diaphyseal portions of long bones.

a. Ewing sarcoma
b. Chondrosarcoma
c. Osteosarcoma
d. Rhabdomyosarcoma

A

B

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16
Q

A radiograph finding of a lesion that may have a lobular appearance with mottled or punctate or annular calcification of the cartilaginous matrix.

a. Ewing sarcoma
b. Chondrosarcoma
c. Osteosarcoma
d. Rhabdomyosarcoma

A

B

17
Q

It is the mainstay of therapy for chondrosarcoma.

a. chemotherapy
b. radiation therapy
c. surgical resection of tumor
d. two of the options
e. all of the options

A

C

most chondroblastomas are resistant to chemotherapy

18
Q

Type of chondrosarcoma that responds to chemotherapy:

a. differentiated chondrosarcoma
b. mesenchymal chondrosarcoma
c. low-grade chondrosarcoma
d. two of the options

A

D

Dedifferentiated chondrosarcoma has a high-grade osteosarcoma or a malignant fibrous histiocytoma component that responds to chemotherapy.
Mesenchymal chondrosarcoma, a rare variant composed of a small-cell element, also is responsive to systemic chemotherapy and is treated like Ewing’s sarcoma.

19
Q

It typically involves the diaphyseal region of long bones and also has an affinity for flat bones.

a. Ewing sarcoma
b. Chondrosarcoma
c. Osteosarcoma
d. Rhabdomyosarcoma

A

A

20
Q

It is a type of sarcoma that is associated with virus, specifically caused by the human herpes virus 8 (HHV8)

a. Undifferentiated pleomorphic sarcoma
b. Kaposi’s sarcoma
c. Myxofibrosarcoma
d. Malignant fibrous histiocytoma

A

B

21
Q

It is a low-grade, well-differentiated tumor that does not invade the medullary cavity and most frequently found in the posterior aspect of the distal femur.

a. Telangiectatic sarcoma
b. Parosteal osteosarcoma
c. Periosteal osteosarcoma
d. Ewing sarcoma

A

B

Telangiectatic - lytic appearance on radiography
Periosteal - arises on the surface of the bone but has a higher metastatic spread than the parosteal type

22
Q

The following are predispositions of osteosarcoma, except:

a. Retinoblastoma
b. Li-Fraumei syndrome
c. Paget syndrome
d. two of the options
e. none of the options

A

E

include radiotherapy

23
Q

The microscopic appearance that is of a small, round, blue cell tumor is seen in:

a. Neuroblastoma
b. Lymphoma
c. Ewing sarcoma
d. Rhabdomyosarcoma
e. All of the options

A

E

24
Q

It transmits body weight from hip bone to tibia when standing.

a. fibula
b. femur
c. hip

A

B

25
Q

It is made up of the medial and lateral femoral condyles that articulate with menisci and tibial condyles to form the knee joint

a. shaft
b. proximal femur
c. distal femur
d. neck of femur

A

C

26
Q

It is a type of rhabdomyosarcoma that is present in trunk and extremities during childhood

a. Pleomorphic rhabdomyosarcoma
b. Botryoid rhabdomyosarcoma
c. Embryonal rhabdomyosarcoma
d. Alveolar rhabdomyosarcoma

A

D

Major differences: table 527.1 p.2686 Nelson

27
Q

It is also known as adult form of rhabdomyosarcoma

a. Pleomorphic rhabdomyosarcoma
b. Botryoid rhabdomyosarcoma
c. Embryonal rhabdomyosarcoma
d. Alveolar rhabdomyosarcoma

A

A

28
Q

It is characterized by tumor cells and an edematous stroma project into a body cavity like a bunch of grapes.

a. Pleomorphic rhabdomyosarcoma
b. Botryoid rhabdomyosarcoma
c. Embryonal rhabdomyosarcoma
d. Alveolar rhabdomyosarcoma

A

B

29
Q

Common sites of metastases of Ewing sarcoma include which of the following:

a. lung
b. bone
c. bone marrow
d. two of the options
e. all of the options

A

E

30
Q

Common sites of hematogenous metastases of osteosarcoma include which of the following:

a. lung
b. bone
c. bone marrow
d. two of the options
e. all of the options

A

D

lung, bone and brain