RMS Flashcards
Most common site of RMS
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%
It is an independent predictor of prognosis. The higher it is, the poorer is the survival
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
What is the cause of RMS?
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
Give the most common histology on the ff sites with RMS:
Bladder:
vagina:
Trunk and extremities:
Head and neck:
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.
most common site with LN mets:
Least common site with LN mets:
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.
Hematogeneous spread is most common in what sites with RMS?
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
Most common presentation of RMS
ASYMTOMATIC MASS
What are the 4 histologic subtypes of RMS? Most common?
Embryonal - most common 2/3
Botryoid subtype of embryonal
Alveolar - 2nd most common
Pleomorphic
Which consists the superior prognosis group?
The superior prognosis group, comprising of
two subsets (botryoid and spindle cell), carries a projected
5-year survival rate of 88% to 95%.
The ____ subtype, a polypoid variant of embryonal RMS, has a grapelike appearance.
botryoid
The term used for hypercellular zone of tumor cells of botryoid tumors?
cambium layer of Nicholson
In ultrastructural analysis of this tumors, which will confirm the diagnosis?
Presence of Cross-striations
LOH of chromosome ___ is specific for embruyonal subtype
11p15.5 locus
The poor prognosis group?
group with poor prognosis includes alveolar, diffuse
anaplastic, and undifferentiated sarcomas.
Most treatment failures occur within ___ years
3 years
Primary treatment for orbital RMS?
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.
Head and Neck parameningeal RMS is composed of?
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
T or F
In Parameningeal RMS, whole-brain irradiation is not necessary, even in the presence of direct intracranial tumor extension.
True
T or F?
Non parameningeal tumors may be more amenable to complete gross surgical excision compared with their parameningeal counterparts
True
T or F?
Patients with tumors arising in the bladder have significantly inferior survival compared with those with tumor confined to the prostate
False
patients with tumors arising in the prostate have significantly inferior survival compared with those with tumor confined to the bladder
Most common presentation of RMS Paratesticular tumors is?
At presentation, the tumor usually is a painless scrotal or inguinal mass that does not transilluminate.
Most common site of RMS in gynecologic tumors?
vagina
Patients with vaginal tumors are often much younger than
those with other pelvic RMS, with most girls diagnosed before
the age of ___ years.
3 years
Most common presentation for vaginal RMS is _____; botryoid morphology is common
vaginal mass or discharge
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?
T
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
alveolar or undifferentiated
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?
True
Before the era of multidisciplinary therapy, surgical ablation
resulted in a long-term survival rate of approximately ___% of those patients able to undergo resection
20
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.
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.
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?
True!
In cases of suspected RMS, the initial surgical procedure
should be an ______.
incisional biopsy
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
5 mm
In RMS, Chemotherapy is necessary in all cases
T or F
True
Several drugs have demonstrated single-agent activity measured as a percentage response rate, which has the highest?
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
In RMS, the most extensive experience in combination chemother-
apy is with ______?
VAC or VAC plus doxorubicin (VACA)
In RMS, Prophylactic lymph node irradiation is not necessary in
children with clinically negative findings who will be receiving
combination chemotherapy
T or F?
True
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.
1 cm
Patients with tumors at ______ sites have developed meningeal extension of tumor when inadequate irradiation portals were used.
parameningeal sites (middle ear, para-
nasal sinuses, nasopharynx, nasal cavity, infratemporal fossa,
and parapharyngeal area)
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.
50 to 55 Gy
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.
45 Gy
the standard of care for group III RMS continues to
be ______?
conventionally fractionated radiation with chemotherapy
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.
IRS-I study had indicated that radiotherapy
was not needed for patients whose tumors were completely
resected at diagnosis (group I)
T or F?
True
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.
alveolar or undifferentiated
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.
dactinomycin and doxorubicin
Because ________ is the most common
pattern of failure, surgery and radiotherapy play especially
important roles in the treatment of these children.
local recurrence
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?
True
Residual masses can be seen in as many as ____ of
children who are shown to be pathologically free of tumor
40%
In Intergroup Rhabdomyosarcoma Studies (IRS), primary goal is?
primary goal has been to test the efficacy of chemother-
apy and radiation therapy as a function of surgical stage
_______ had a poor prognosis, especially in extremity lesions.
alveolar histologic subset
However, those patients who do not have a complete response to chemotherapy by week __ should have early surgery or radiotherapy
9 weeks
CWS 86 study conclusions are?
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.
Both _______ and _______ are known to accentuate a “recall” of radiation injury if given during or immediately after the course of radiation therapy
dactinomycin and doxorubicin
Life-threatening late events occur in approximately ___% of sur-
vivors after treatment
9%
Side effects:
Cyclophosphamide may induce _______.
doxorubicin is implicated in ________.
Cisplatin carries a high incidence of _________.
Alkylating agents and topoisomerase inhibitors are associated with the __________.
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
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
bone sarcomas
highly malignant soft tissue
sarcoma that arises from unsegmented, undifferentiated meso-
derm or myotome-derived skeletal muscle.
RMS
