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Ph RadOnc Residency PBRROE2-3 Review 2021 > RMS > Flashcards

RMS Flashcards

1
Q

RMS
General Treatment Paradigm for RT

Group IV (distant metastases)
-any nodal status/molecular subtype
A

Treat primary as (group I-III) + other sites of metastatic disease

Ongoing Investigations:
Treating bone metastases with stereotactic body radiation therapy.

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2
Q

RMS
General Treatment Paradigm for RT

Group III (gross residual disease)
-any nodal status/molecular subtype
A

50.4 Gy.

Ongoing Investigations:
For patients with group III disease, treating the prechemotherapy volume to 36 Gy followed by a boost to 50.4 or 59.4 Gy if the initial size is >5 cm.

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3
Q

RMS
General Treatment Paradigm for RT

Group II (positive microscopic margins or resected regional disease)

node +

A

41.4 Gy

Ongoing Investigations:
Reducing the dose to 36 Gy for patients with group II node-positive disease.

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4
Q

RMS
General Treatment Paradigm for RT

Group II (positive microscopic margins or resected regional disease)

node (–)

A

36 Gy

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5
Q

RMS
General Treatment Paradigm for RT

Group I (localized disease, completely resected with negative margins)

fusion-negative

A

No RT

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6
Q

RMS
General Treatment Paradigm for RT

Group I (localized disease, completely resected with negative margins)

fusion-positive

A

36 Gy

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7
Q

RMS

What are the most common locations of RMS (in decreasing incidence)

A 
genitourinary (31%)
parameningeal (25%)
extremity (13%)
orbit (9%)
retroperitoneum (7%)
head and neck (7%)
trunk (5%)
others (3%)

GP E ORTHO

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8
Q

RMS

RMS is more common in blacks than in whites.

TRUE or FALSE?

A

False

annual
incidence of 4.4 per 1 million whites and 1.3 per 1 million blacks

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9
Q

RMS

RMS is more common in males than females;
males may have slightly worse OS.

TRUE or FALSE?

A

False (due to second statement)

The male-tofemale
ratio is approximately 1.5 to 1.0, and males may have slightly better
overall survival

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10
Q

RMS

RMS has 2 peak age frequencies.
2 to 6 and in adolescence.

What is the most common histology for patients in the younger group?

A

embryonal

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11
Q

RMS

RMS has 2 peak age frequencies.
2 to 6 and in adolescence.

What is the most common histology for patients in the older (≥10) group?

A

alveolar

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12
Q

RMS

Age is an independent predictor of prognosis, with children <1 and >10 years having inferior survival.

TRUE or FALSE?

A

true

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13
Q

Adults with RMS have been reported to have poor
outcomes, although there is evidence that when they are treated aggressively
using pediatric-type protocols, the prognosis may be similar to that of younger
patients.

A

true

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14
Q

What is the fusion status important in prognostication and therapeutic decision-making in RMS?

A

PAX/FOXO1 fusion status

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15
Q

What is the most common histology of urinary or vaginal RMS?

A

botryoid / embryonal

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16
Q

Urinary or vaginal RMS is common to what age group?

A

infants

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17
Q

What is the most common location/histology of adolescent RMS?

A

trunk and abdominal

alveolar

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18
Q

In RMS, what are the predictors of lymph node involvement aside from tumor location?

A

large tumor size

tumor invasiveness

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19
Q

In RMS, hematogenous dissemination occurs in approximately 15% of disease at presentation.

What are the most common sites of dissemination?

A

lungs
bone marrow
bone

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20
Q

The clinical grouping classification used extensively by the Intergroup Rhabdomyosarcoma Study Group (now known as the Children’s Oncology Group Soft Tissue Sarcoma [COG STS] committee) investigators is somewhat of a misnomer because it actually requires surgical pathologic evaluation.

What is its importance?

A

for treatment selection

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21
Q

In RMS, if the IRS study group classification is used for treatment selection, what is used for prognostication?

A

TNM

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22
Q

RMS

What are the parameters of the TNM that correlates with good prognosis?

A

non invasiveness
small size
absence of metastases

site of tumor

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23
Q

RMS
IRG grouping:

Incomplete resection or biopsy with gross residual disease

A

Group III

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24
Q

RMS
IRG grouping:

Localized disease, completely resected
Infiltration outside organ or muscle of origin; regional nodes not involved

A

Group IB

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25
Q

RMS
IRG grouping:

Localized disease, completely resected
Confined to organ or muscle of origin

A

Group IA

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26
Q

RMS
IRG grouping:

Compromised or regional resection
Regional disease with involved nodes, grossly resected, but with evidence of
microscopic residual disease

A

Group IIC

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27
Q

RMS
IRG grouping:

Compromised or regional resection
Grossly resected tumor with microscopic residual disease

A

Group IIA

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28
Q

RMS
IRG grouping:

Compromised or regional resection
Regional disease, completely resected, in which nodes may be involved or extension
of tumor into adjacent organ may exist

A

Group IIB

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29
Q

RMS

Favorable sites

A

orbit
H&N (non parameningeal)
GU (non-bladder/prostate)

30
Q

RMS

Unfavorable sites

A

bladder/prostate
parameningeal
extremity
other

31
Q

RMS
Staging

T1

A

confined to organ

32
Q

RMS
Staging

T2

A

extension outside site or organ of origin

33
Q

RMS
Staging

a

A

≤5 cm

34
Q

RMS
Staging

b

A

5 cm

35
Q

RMS
Staging

Identify the stage:

Favorable, N0, T1a, M0

A

I

36
Q

RMS
Staging

Identify the stage:

Unfavorable, N0, T1a, M0

A

II

37
Q

RMS
Staging

Identify the stage:

Favorable, N1, T1a, M0

A

I

38
Q

RMS
Staging

Identify the stage:

Favorable, N0, T1b, M0

A

I

39
Q

RMS
Staging

Identify the stage:

Unfavorable, N0, T2a, M0

A

II

40
Q

RMS
Staging

Identify the stage:

Favorable, N1, T1b, M0

A

I

41
Q

RMS
Staging

Identify the stage:

Unfavorable, N0, T1b, M0

A

III

42
Q

RMS
Staging

Identify the stage:

Favorable, N1, T2b, M0

A

I

43
Q

RMS
Staging

Identify the stage:

Unfavorable, N0, T2b, M0

A

III

44
Q

RMS
Staging

Identify the stage:

Favorable, N1, T2a, M0

A

I

45
Q

RMS
Staging

Identify the stage:

Unfavorable, N1, T1a, M0

A

III

46
Q

RMS
Staging

Identify the stage:

Unfavorable, N1, T1b, M0

A

III

47
Q

RMS

What histologies are considered to be in the superior prognosis group?

A

botryoid - commonly in vagina, urinary bladder, middle ear, biliary tree, and nasopharynx.

spindle - commonly in paratesticular

48
Q

RMS

What histology is considered to be in the intermediate prognosis group?

A

embryonal - commonly found in orbit, head and neck, and genitourinary sites

49
Q

RMS Histology

Identify:

  • a polypoid variant of embryonal RMS
  • has a grapelike appearance
  • stroma consists of loose cellular tissue with a myxoid appearance.

Under the superficial stroma is a hypercellular zone of tumor cells called the cambium layer of Nicholson.

A

botryoid

50
Q

RMS Histology

Identify:

  • consists of blastemal mesenchymal cells that tend to differentiate into cross-striated muscle cells.
  • have eosinophilic cytoplasm, which is positive by periodic acid–Schiff staining.
  • actin- or desmin-positive reactions.
  • evidence of myogenesis with the presence of thick and thin cytoplasmic intermediate filaments or Z-band material.

Ribbon or strap-shaped cells and tadpole cells are characteristic.
The presence of cross-striations confirms the diagnosis.

A

embryonal - commonly found in orbit, head and neck, and genitourinary sites

51
Q

RMS

What is the genetic abnormality in the embryonal subtype?

A

consistent loss of heterozygosity at the chromosome 11p15.5 locus

52
Q

RMS

What histologies are considered to be in the poor prognosis group?

A

alveolar - commonly found in adolescents with truncal, retroperitoneal,
and extremity tumors

anaplastic

undifferentiated

53
Q

RMS Histology

Identify:

Approximately 75% of children with this histology exhibit a characteristic translocation involving chromosomes 2 and 13, t(2;13)(q35;q14), and occasionally a 1;13 translocation.

These translocations correspond to abnormal fusion genes involving PAX3-FKHR and PAX7-FKHR, respectively, and are probably the initial oncogenic events in these tumors.

Immunohistochemical presence of AP2-β and P-cadherin is also highly specific for this histology.

A

alveolar

54
Q

RMS
orbital RMS

What is the role of surgery?

A

histologic confirmation only for initial treatment

and as a salvage exenteration and enucleation for management of posttreatment ocular complications

55
Q

RMS
orbital RMS

What is the primary treatment?

A

chemo with VAC or VA

vincristine, actinomycin-D, cyclophosphamide

56
Q

RMS
orbital RMS
(from inservice bank)

When is RT administered after chemotherapy?

A

after 12 weeks

57
Q

RMS
orbital RMS

RT dose?

A

50 Gy.

45 Gy is associated with unacceptable local control rates

D9602 and ARST0331

58
Q

RMS

What sites are considered parameningeal?

A 
nasopharynx
paranasal sinuses
middle ear
pterygopalatine fossa
infratemporal fossa
59
Q

RMS

What RT technique is used for parameningeal RMS with meningeal dissemination?

A

CSI

60
Q

RMS

What RT dose is given for the primary parameningeal tumor as definitive treatment?

A

50.4/28

61
Q

RMS

What is the role of RT for nonparameningeal H&N tumors?

A

PORT

62
Q

RMS
Gynecologic

What is the most common site?

A

Vagina

63
Q

RMS
Gynecologic

Treatment

A

Surgery + RT +/-chemotherapy

RT for all microscopic or macroscopic tumor.

analysis of data
from the IRS-IV and IRS-V studies have revealed high rates of local failure for
vaginal tumors when local control with surgery or radiotherapy is omitted

64
Q

RMS
Extremity

Treatment

A

avoid disfiguring surgical procedures and recommend limb-salvage procedures including irradiation and chemotherapy

65
Q

RMS
Extremity

RT of involved lymphatics is mandatory

TRUE or FALSE?

A

True

66
Q

RMS
RT

CTV?

A

prechemo volume + surgical sites and biopsy tracts

+ 1 cm

67
Q

RMS
RT

cone-down volume after microscopic dose has been delivered.

A

gross posttreatment volume

68
Q

RMS
RT

Dose for gross residual disease?

A

standard: 50.4/1.8/28
hfrt: 59.4/1.1 bid

69
Q

RMS
RT

Dose for microscopic residual disease associated with a lymph node involvement?

A

41.4 Gy

70
Q

RMS
RT

Dose for microscopic residual disease “not” associated with a lymph node involvement?

A

36 Gy

71
Q

RMS

Which IRS study/studies introduced and omitted WBRT for patients with parameningeal tumors?

A

II and (III-IV) respectively

III for with intracranial
IV for all patients

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

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