RMS Flashcards by Mario Luna

RMS
General Treatment Paradigm for RT

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

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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RMS
General Treatment Paradigm for RT

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

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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RMS
General Treatment Paradigm for RT

Group II (positive microscopic margins or resected regional disease)

node +

41.4 Gy

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

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RMS
General Treatment Paradigm for RT

Group II (positive microscopic margins or resected regional disease)

node (–)

36 Gy

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RMS
General Treatment Paradigm for RT

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

fusion-negative

No RT

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RMS
General Treatment Paradigm for RT

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

fusion-positive

36 Gy

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RMS

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

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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RMS

RMS is more common in blacks than in whites.

TRUE or FALSE?

False

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

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RMS

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

TRUE or FALSE?

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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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?

embryonal

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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?

alveolar

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RMS

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

TRUE or FALSE?

true

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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.

true

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

PAX/FOXO1 fusion status

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

botryoid / embryonal

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Urinary or vaginal RMS is common to what age group?

infants

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

trunk and abdominal

alveolar

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

large tumor size

tumor invasiveness

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

What are the most common sites of dissemination?

lungs
bone marrow
bone

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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?

for treatment selection

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

TNM

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RMS

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

non invasiveness
small size
absence of metastases

site of tumor

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RMS
IRG grouping:

Incomplete resection or biopsy with gross residual disease

Group III

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RMS
IRG grouping:

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

Group IB

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RMS IRG grouping: Localized disease, completely resected Confined to organ or muscle of origin

Group IA

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

Group IIC

RMS IRG grouping: Compromised or regional resection Grossly resected tumor with microscopic residual disease

Group IIA

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

Group IIB

RMS Favorable sites

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

RMS Unfavorable sites

bladder/prostate parameningeal extremity other

RMS Staging T1

confined to organ

RMS Staging T2

extension outside site or organ of origin

RMS Staging a

≤5 cm

RMS Staging b

5 cm

RMS Staging Identify the stage: Favorable, N0, T1a, M0

RMS Staging Identify the stage: Unfavorable, N0, T1a, M0

RMS Staging Identify the stage: Favorable, N1, T1a, M0

RMS Staging Identify the stage: Favorable, N0, T1b, M0

RMS Staging Identify the stage: Unfavorable, N0, T2a, M0

RMS Staging Identify the stage: Favorable, N1, T1b, M0

RMS Staging Identify the stage: Unfavorable, N0, T1b, M0

III

RMS Staging Identify the stage: Favorable, N1, T2b, M0

RMS Staging Identify the stage: Unfavorable, N0, T2b, M0

III

RMS Staging Identify the stage: Favorable, N1, T2a, M0

RMS Staging Identify the stage: Unfavorable, N1, T1a, M0

III

RMS Staging Identify the stage: Unfavorable, N1, T1b, M0

III

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

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

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

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

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.

botryoid

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.

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

RMS What is the genetic abnormality in the embryonal subtype?

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

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

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

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.

alveolar

RMS orbital RMS What is the role of surgery?

histologic confirmation only for initial treatment and as a salvage exenteration and enucleation for management of posttreatment ocular complications

RMS orbital RMS What is the primary treatment?

chemo with VAC or VA | vincristine, actinomycin-D, cyclophosphamide

RMS orbital RMS (from inservice bank) When is RT administered after chemotherapy?

after 12 weeks

RMS orbital RMS RT dose?

50 Gy. 45 Gy is associated with unacceptable local control rates D9602 and ARST0331

RMS What sites are considered parameningeal?

``` nasopharynx paranasal sinuses middle ear pterygopalatine fossa infratemporal fossa ```

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

CSI

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

50.4/28

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

PORT

RMS Gynecologic What is the most common site?

Vagina

RMS Gynecologic Treatment

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

RMS Extremity Treatment

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

RMS Extremity RT of involved lymphatics is mandatory TRUE or FALSE?

True

RMS RT CTV?

prechemo volume + surgical sites and biopsy tracts + 1 cm

RMS RT cone-down volume after microscopic dose has been delivered.

gross posttreatment volume

RMS RT Dose for gross residual disease?

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

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

41.4 Gy

RMS RT Dose for microscopic residual disease "not" associated with a lymph node involvement?

36 Gy

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

II and (III-IV) respectively III for with intracranial IV for all patients