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Ph RadOnc Residency PBRROE2-3 Review 2021 > Ewing Tumor > Flashcards

Ewing Tumor Flashcards

1
Q

Ewing Tumor

Ewing tumor is the most common primary bone tumor in childhood.

TRUE or FALSE?

A

False

Osteosarcoma

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2
Q

Ewing Tumor

Ewing tumor is the most common soft tissue sarcoma in childhood.

TRUE or FALSE?

A

False

RMS

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3
Q

Ewing Tumor

What translocation is present in approximately 95% of tumors.

A

t(11;22)(q24;q22)
EWS22 and FLI1

or
t(21;22)(q22;12)
EWS22 and ERG21

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4
Q

Ewing Tumor

Describe IHC staining patterns of EwT.

A

PAS+
vimentin+
CK+

(-) for lymphoma, NB, or RMS

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5
Q

Ewing Tumor

What is the most common presenting symptom in patients with ESFT?

A

Localized pain

reported in about 90% of cases

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6
Q

Ewing Tumor

Differentiate osteosarcoma and ewing tumor based on the location of tumor involvement of long bones.

A

osteosarcoma - metaphysis

ewing tumor - diaphysis

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7
Q

Ewing Tumor

Whole-body MRI has also been reported to be superior to bone scan in
detecting bone metastases in patients with Ewing tumors

TRUE or FALSE?

A

True

particularly diffusion-weighted
images or short-time inversion recovery (STIR) images.

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8
Q

Ewing Tumor

What transcript can sometimes be detected in the peripheral blood or bone marrow, where it may indicate residual occult disease?

A

EWS-FLI1

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9
Q

Ewing Tumor

What are the two most frequent metastatic sites?

A

lungs and bones

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10
Q

Ewing Tumor

patients with only ____ metastasis have a
better clinical outcome than those with metastases in other sites (EFS 29% to
52%)

A

lung

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11
Q

Ewing Tumor

In metastatic disease, what are the prognostic factors identified by the EE99-R3 study that formed the basis for an up-front risk scoring system?

A

diagnosis at >14 years

primary tumor volume >200mL

> 1 bone metastatic site

BM mets

additional lung mets

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12
Q

Ewing Tumor

In localized disease, what are the favorable prognostic factors?

A

distal/peripheral site

tumor volume <200 mL (and/or tumor size <8 cm)

normal LDH levels

younger age

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13
Q

Ewing Tumor

An R0 resection negates the adverse risk conferred by an incomplete response to neoadjuvant chemotheray.

TRUE or FALSE?

A

False.

Poor histologic/radiologic response to chemotherapy has also been identified
as a major adverse prognostic factor, even when chemotherapy was followed by
R0 resection

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14
Q

Ewing Tumor

Central (pelvic) tumors are more likely to metastasize than extremity tumors.

They also have lower survival rates compared to those with lesions in distal bones of the extremities.

TRUE or FALSE?

A

True

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15
Q

Ewing Tumor

What are the predictors of poor response to chemotherapy?

A 
VEGF
or
p53, p16/p14ARF alterations
or
presence of CCN3
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16
Q

Ewing Tumor
General Management

Intralesional resection or debulking procedures followed by RT offer increased LC or DFS over definitive RT alone.

TRUE or FALSE?

A

Intralesional resection or debulking procedures followed by RT do not offer increased LC or DFS compared with definitive RT and should be avoided

17
Q

Ewing Tumor
Exclusive RT

What is the standard RT dose for gross disease?

A

55.8 to 59.40 Gy (1.8)

For gross disease, standard treatment is a total dose of 55.8 to 59.40 Gy and 1.8
Gy/day.

A dose of 45 to 50.4 Gy is usually recommended, although 36 Gy may
be adequate for the initial field, including microscopic disease.

18
Q

Ewing Tumor
Adjuvant RT

RT improves LC for all the patients, even after
good response to induction chemotherapy.

TRUE or FALSE?

A

True

a recent analysis of the
EE99 trial showed that adjuvant RT improves LC for all the patients, even after
good response to induction chemotherapy.

With a median follow-up of 6 years,
8-year local relapse incidence was 12%. A statistically significant reduction of
local relapse was shown in patients treated with surgery and RT compared to
surgery alone (P = .02). The benefit of RT was particularly marked for tumors
larger than 200 mL at diagnosis and 100% necrosis. A nonsignificant trend for
benefit associated with postoperative RT for DFS, EFS, and OS was reported.

19
Q

Ewing Tumor
Adjuvant RT

What is the RT dose given by COG for postoperative RT?

A

(45 to 54 range)

Doses in the range of 30 to 44.8 Gy at 1.8
Gy a day have also been reported to be effective for subclinical disease.

20
Q

Ewing Tumor
Adjuvant RT

What is the RT dose for post intralesional resection with gross residual?

A

Same as definitive RT alone dose (up to 59.4 Gy) (1.8 to 2)

21
Q

Ewing Tumor
Adjuvant RT

What is the RT dose for post intralesional resection with microscopic involvement?

A

36 to 50.4 Gy (1.8 to 2)

Total of initial and boost

22
Q

Ewing Tumor
Neodjuvant RT

What is the total dose for preoperative RT? (range and per fraction)

A

45 to 50.4 Gy (1.8)

23
Q

Ewing Tumor
RT after HDCT

What is the total RT dose prescribed if the patient received HDCT-BuMel?

A

45 Gy

24
Q

Ewing Tumor
RT after HDCT

What is the dose constraint for the spinal cord if the patient received HDCT-BuMel?

A

no more than 30 Gy

25
Q

Ewing Tumor
RT after HDCT

When do you administer RT after HDCT? (EE2012 protocol)

A

at least 10 weeks after

26
Q

Ewing Tumor

What is the difference between CTV1 and 2 in the RT of Ewing tumor?

A

CTV1 includes drain sites and scars.

27
Q

Ewing Tumor

What are the independent risk factors for survival in localized tumor of the rib?

A

pleural effusion at diagnosis

incomplete resection

28
Q

Ewing Tumor
RT

What is the dose for vertebral lesions?

A

45 Gy

29
Q

Ewing Tumor
RT

What is the dose for sacral lesions?

A

full dose

30
Q

Ewing Tumor

Adding ________ to VAC produced superior results compared to VAC in terms of LC and EFS.

A

Doxorubicin / VACA or VACD

IESS-I trial

31
Q

Ewing Tumor

What study showed that induction chemotherapy is a viable option, as confirmed by SJCRH and has been since the standard of care.

A

IESS-II

32
Q

Ewing Tumor

THe addition of combined ____&____ to VACD showed an increase in 5-year EFS in patients particularly those with primary tumors of the pelvis.

A

ifosfamide and etoposide (IE)
/VACD-IE

(Int-0091) based on phase II POG-CCG study

33
Q

Ewing Tumor

Drug/drug combination that has been reported with interesting response rates in the relapsed setting.

A

topotecan–cyclophosphamide,

temozolomide–irinotecan,

gemcitabine–docetaxel,

and high dose ifosfamide.

34
Q

Ewing Tumor

There are reports of pathologic fracture as a sequelae of treatment.

What is the most common site?

A

Proximal femur

35
Q

Ewing Tumor

Secondary malignancies are common as a late sequelae of treatment.

What is the most common?

A

Osteosarcoma

36
Q

Ewing Tumor

Standard chemotherapy in Europe

A

VIDE

37
Q

Ewing Tumor

Standard chemotherapy in North America

A

VDC-IE

Ph RadOnc Residency PBRROE2-3 Review 2021 (57 decks)

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