RMS

Question 1

Most common site of RMS

Answer 1

most frequently involved sites are the genitourinary 31%; parameningeal, 25%;extremity, 13%;
orbit, 9%; head and neck (excluding parameningeal tumors),
7%; retroperitoneum, 7%, and
trunk, 5%; other sites 3%

Question 2

It is an independent predictor of prognosis. The higher it is, the poorer is the survival

Answer 2

Age

There are two peak age frequencies, at ages 2 to 6 and in ado-
lescence. Tumors in the younger age group are likely to be of
embryonal histology (or one of its subtypes). About 25% of
patients are ≥10 years at diagnosis and their tumors are more
commonly of alveolar histology. Age has been identified as an
independent predictor of prognosis, with children 10 years having inferior survival.3 Adults with RMS have
been reported to have poor outcomes

Question 3

What is the cause of RMS?

Answer 3

unknown

however, it is associated
with several environmental exposures including paternal ciga-
rette use, prenatal x-ray exposure, and maternal recreational
drug use.2 RMS is also associated with disorders in develop-
ment, including central nervous system, genitourinary, gastro-
intestinal, and cardiovascular anomalies, and with congenital
disorders including congenital pulmonary cysts, Gorlin basal
cell nevus syndrome, and neurofibromatosis

Question 4

Give the most common histology on the ff sites with RMS:

Bladder:
vagina:
Trunk and extremities:
Head and neck:

Answer 4

There are unexplained associations of site of primary tumor
with age at diagnosis and tumor histology.

tumors arising in the urinary bladder and vagina occur pri-
marily in infants and often are of the embryonal or botryoid
histologic type.

Tumors arising in the trunk and extremity
occur in adolescents and are often alveolar or undifferenti-
ated type.

Tumors of the head and neck area occur throughout
childhood and are commonly of the embryonal type.

Question 5

most common site with LN mets:

Least common site with LN mets:

Answer 5

overall risk of regional lymphatic spread is approxi-
mately 15%, but varies with the site of the primary lesion.
Lymph node metastases are rare in orbital tumors, but they
occur in approximately 15% of tumors at other head and neck
sites, most commonly the nasopharynx.

Question 6

Hematogeneous spread is most common in what sites with RMS?

Answer 6

Hematogenous metastases are detected at the time of pre-
sentation in approximately 15% of patients, particularly those
with truncal and extremity primary tumors. The most common
sites of hematogenous dissemination are lungs, bone marrow,
and bone

Question 7

Most common presentation of RMS

Answer 7

ASYMTOMATIC MASS

Question 8

What are the 4 histologic subtypes of RMS? Most common?

Answer 8

Embryonal - most common 2/3
Botryoid subtype of embryonal
Alveolar - 2nd most common
Pleomorphic

Question 9

Which consists the superior prognosis group?

Answer 9

The superior prognosis group, comprising of
two subsets (botryoid and spindle cell), carries a projected
5-year survival rate of 88% to 95%.

Question 10

The ____ subtype, a polypoid variant of embryonal RMS, has a grapelike appearance.

Answer 10

botryoid

Question 11

The term used for hypercellular zone of tumor cells of botryoid tumors?

Answer 11

cambium layer of Nicholson

Question 12

In ultrastructural analysis of this tumors, which will confirm the diagnosis?

Answer 12

Presence of Cross-striations

Question 13

LOH of chromosome ___ is specific for embruyonal subtype

Answer 13

11p15.5 locus

Question 14

The poor prognosis group?

Answer 14

group with poor prognosis includes alveolar, diffuse

anaplastic, and undifferentiated sarcomas.

Question 15

Most treatment failures occur within ___ years

Answer 15

3 years

Question 16

Primary treatment for orbital RMS?

Answer 16

Primary treatment typically consists of vincristine, actinomy-
cin-D, and cyclophosphamide (VAC) or vincristine and actino-
mycin-D (VA) chemotherapy with local radiotherapy beginning
between the 3rd and 12th week of treatment. Radiation doses
of approximately 50 Gy are often used, although results from
the IRS-V study suggest that 45 Gy may be sufficient when
given with a cyclophosphamide-containing chemotherapy
combination.

Question 17

Head and Neck parameningeal RMS is composed of?

Answer 17

Nonorbital  RMS  of  the  head  and  neck  is  grouped  into  para-
meningeal sites (nasopharynx, nasal cavity, paranasal sinuses, 
middle  ear,  pterygopalatine  fossa,  and  infratemporal  fossa) 

PPINN Me

Question 18

T or F

In Parameningeal RMS, whole-brain irradiation is not necessary, even in the presence of direct intracranial tumor extension.

Answer 18

True

Question 19

T or F?

Non parameningeal tumors may be more amenable to complete gross surgical excision compared with their parameningeal counterparts

Answer 19

True

Question 20

T or F?

Patients with tumors arising in the bladder have significantly inferior survival compared with those with tumor confined to the prostate

Answer 20

False

patients with tumors arising in the prostate have significantly inferior survival compared with those with tumor confined to the bladder

Question 21

Most common presentation of RMS Paratesticular tumors is?

Answer 21

At presentation, the tumor usually is a painless scrotal or inguinal mass that does not transilluminate.

Question 22

Most common site of RMS in gynecologic tumors?

Answer 22

vagina

Question 23

Patients with vaginal tumors are often much younger than
those with other pelvic RMS, with most girls diagnosed before
the age of ___ years.

Answer 23

3 years

Question 24

Most common presentation for vaginal RMS is _____; botryoid morphology is common

Answer 24

vaginal mass or discharge

Question 25

In gynecologic RMS, Current guidelines from the COG STS committee call for radiotherapy in all patients with post- surgical microscopic or macroscopic tumor T or F?

Answer 25

T

Question 26

Tumors arising in the extremity are often of the _____ subtypes, large, deeply invasive, and associ- ated with a high probability of lymphatic and hematogenous metastasis

Answer 26

alveolar or undifferentiated

Question 27

Hematogenous or distant lymph node metastasis at the time of diagnosis is an ominous finding, although not all these children do poorly. true of false?

Answer 27

True

Question 28

Before the era of multidisciplinary therapy, surgical ablation resulted in a long-term survival rate of approximately ___% of those patients able to undergo resection

Answer 28

20

Question 29

It involves removal of the bulk of tumor with maximal conservation of anatomic structures, including preservation of _________ in patients with tumors of genitourinary origin; ________ in patients with extremity tumors; and ________ in patients with head and neck tumors.

Answer 29

It involves removal of the bulk of tumor with maximal conservation of anatomic structures, including preservation of bladder, bowel, and sexual function in patients with tumors of genitourinary origin; limb function in patients with extremity tumors; and vision, voice, deglutition, and appearance in patients with head and neck tumors.

Question 30

IRS surgical grouping system is used, patients with tumor amenable to complete excision fare better than those who have subtotal resection or biopsy alone. T or F?

Answer 30

True!

Question 31

In cases of suspected RMS, the initial surgical procedure should be an ______.

Answer 31

incisional biopsy

Question 32

Normal tissue margins of at least ___ mm around the tumor are usually required to consider the resection complete (IRS group I), although this is sometimes not feasible in some anatomic sites and smaller margins may suffice

Answer 32

5 mm

Question 33

In RMS, Chemotherapy is necessary in all cases T or F

Answer 33

True

Question 34

Several drugs have demonstrated single-agent activity measured as a percentage response rate, which has the highest?

Answer 34

``` ifosfamide (86%) vincristine (59%) cyclophosphamide (54%) topotecan (46%) mitomycin-C (36%) dactinomycin (24%) , cisplatin (15% to 21%), dacarbazine (11%), etoposide (15% to 21%), , irinotecan (23%), and ```

Question 35

In RMS, the most extensive experience in combination chemother- apy is with ______?

Answer 35

VAC or VAC plus doxorubicin (VACA)

Question 36

In RMS, Prophylactic lymph node irradiation is not necessary in children with clinically negative findings who will be receiving combination chemotherapy T or F?

Answer 36

True

Question 37

A clinical target volume of ___ cm is added and can be modified to account for anatomic barriers to tumor spread (such as the bony orbit in primary orbital tumors) or to account for regression of “pushing” the tumor border after chemotherapy, such as may occur in large pelvic tumors that initially displace contents of the peritoneal cavity.

Answer 37

1 cm

Question 38

Patients with tumors at ______ sites have developed meningeal extension of tumor when inadequate irradiation portals were used.

Answer 38

parameningeal sites (middle ear, para- nasal sinuses, nasopharynx, nasal cavity, infratemporal fossa, and parapharyngeal area)

Question 39

Radiation is necessary to ensure local tumor control in patients who are unable to undergo complete surgical resection. Local control of gross disease in most anatomic sites requires doses of ____ Gy.

Answer 39

50 to 55 Gy

Question 40

Data from the IRS-V D9602 study support a somewhat lesser dose of ___ Gy for gross tumor at orbital sites, especially if cyclophosphamide is included in the systemic therapy regimen.

Answer 40

45 Gy

Question 41

the standard of care for group III RMS continues to | be ______?

Answer 41

conventionally fractionated radiation with chemotherapy

Question 42

They also sug-gest that local tumor control is greater for tumors ___ cm in diameter than for larger lesions, supporting the adult experience with soft tissue sarcomas.

Question 43

IRS-I study had indicated that radiotherapy was not needed for patients whose tumors were completely resected at diagnosis (group I) T or F?

Answer 43

True

Question 44

the IRS-I to IRS-III studies suggested that the subset of group I patients with _______ histology had improved overall and failure-free survival when radiother- apy was given to the primary tumor site.

Answer 44

alveolar or undifferentiated

Question 45

Interaction between radiation and some of the commonly used chemotherapeutic drugs can produce undesir- able early and late effects. This is particularly true of ______ and _____. Radiation therapy given concurrently with these agents is usually avoided.

Answer 45

dactinomycin and doxorubicin

Question 46

Because ________ is the most common pattern of failure, surgery and radiotherapy play especially important roles in the treatment of these children.

Answer 46

local recurrence

Question 47

There is evidence that salvage is more successful if radiotherapy was not used in the initially treatment and can be maximized for salvage therapy. T or F?

Answer 47

True

Question 48

Residual masses can be seen in as many as ____ of | children who are shown to be pathologically free of tumor

Answer 48

40%

Question 49

In Intergroup Rhabdomyosarcoma Studies (IRS), primary goal is?

Answer 49

primary goal has been to test the efficacy of chemother- | apy and radiation therapy as a function of surgical stage

Question 50

_______ had a poor prognosis, especially in extremity lesions.

Answer 50

alveolar histologic subset

Question 51

However, those patients who do not have a complete response to chemotherapy by week __ should have early surgery or radiotherapy

Answer 51

9 weeks

Question 52

CWS 86 study conclusions are?

Answer 52

Conclusions from this study were: 1. Duration of chemotherapy can be reduced to as little as 16 weeks for the most favorable patients. 2. Ifosfamide gives improved response rates compared with cyclophosphamide. 3. Hyperfractionated accelerated radiotherapy concurrent with chemotherapy as used in the study is tolerable and provided acceptable local control.

Question 53

Both _______ and _______ are known to accentuate a “recall” of radiation injury if given during or immediately after the course of radiation therapy

Answer 53

dactinomycin and doxorubicin

Question 54

Life-threatening late events occur in approximately ___% of sur- vivors after treatment

Answer 54

9%

Question 55

Side effects: Cyclophosphamide may induce _______. doxorubicin is implicated in ________. Cisplatin carries a high incidence of _________. Alkylating agents and topoisomerase inhibitors are associated with the __________.

Answer 55

Cyclophosphamide may induce hemorrhagic cystitis doxorubicin is implicated in late myocardiopathies. Cisplatin carries a high incidence of hearing impairment. Alkylating agents and topoisomerase inhibitors are associated with the development of secondary neoplasms, particularly acute myeloid leukemia

Question 56

Late radiation effects are related to the irradiated site, the dose of radiation, and the age of the child at the time of treat- ment. Effects include bone and soft tissue growth disturbances, dental abnormalities, cataract, hypopituitarism, gonadal dys- function, induction of second malignant tumors (particularly ____________), and chronic organ dysfunction

Answer 56

bone sarcomas

Question 57

highly malignant soft tissue sarcoma that arises from unsegmented, undifferentiated meso- derm or myotome-derived skeletal muscle.

Answer 57

RMS