Rhabdomyosacoma

147

19

RHABDOMYOSARCOMA

Sarcoma

tissues

primitive mesenchymal cells

incomplete differentiation

Rhabdomyosarcoma (RMS)

(striated muscle)

21

5-8%

fibrous

sarcoma

soft tissue sarcoma

modalities

combined

EPIDEMIOLOGY
Soft tissue sarcomas

acute leukemias, CNS tumors, lymphomas,

neuroblastoma

RMS

Wilms tumor

(1)

undifferentiated sarcoma

(2)

70%

RMS

Rhabdomyosacoma

148

5-8%

lymphoma
histology

soft tissue sarcoma

(3,4)

RMS

Burkitts

embryonal

RMS

RMS

bladder

botryoid histology

15

alveolar

vagina

RMS

undifferentiated histology

PATHOLOGY

RMS

tissue sarcoma (STS)

RMS

soft

19.1

STS

RMS

19.1

Nonrhabdomyosarcoma Soft Tissue

Sarcomas
Tumor

Cell

Origin

Common
Sites

Common
Ages

Good

Prognos

Outc

ome

tic

Factors
Fibrosarco

Fibrobla

Excel

Rhabdomyosacoma

ma

149

sts

lent
Extremity
trunk

Most <2
yr

< 5 yr with

surge

Congenital

ry

alone
Extremity

Adult

(thigh,knee)

15 yr

5-yr

form

survi
val,

84%
5-yr

survi
val,
34-

60%
Neurofibro

Schwan

sar-coma

Extremity

Younger

Trunk,

n cell or retroperitone

in

extremi survi

with

<5

mato-sis

neurofib ut

fibroblas um

trunk

patients

ties,

neurofibro cm, no
(NFI)

ro-

53%
val

witho
NFI;

matosis 16%

Rhabdomyosacoma

150

with
NFI
Synovial
sarcoma

Mesenc

Extremities

Adolesce

Age <

cells

upper)

Young

< 5 cm II,

hymal

(lower >

nce

14 yr,

Stage
I and

adulthood calcifica 70%

tion

Stage
IIIIV,

poor
Malignant

Fibrobla Lower

Usually

5-yr

fibrous

st

10-20

survi

extremity

histiocytom

27-

53%
Extremity

Angiomato
id

form

val,

Young

children

Excel
lent

with
surge
ry

alone

Rhabdomyosacoma

19.1

151

Nonrhabdomyosarcoma Soft Tissue

Sarcomas ()
Tumor

Cell

Common

Common

Good

Outcom

Sites

Ages

Prognos

Origin

tic

Factors
Hemangio
pericyto-

Pericytes Extremit
y, re-

ma

Usually

Low

Stage I

<5 cm.

30-70%.

10-20 yrs stage

troperito
neum,

head and

with

adjuvant

Rare

therapy,

Infantile

III and

form

IV, poor
Paragang Extremit
lionic

soft part

sarcoma

5-yr

survival

neck

Alveolar

and II,

y, trunk

mesoder

Lower

m(?);

extremit

neural

y,

15-35 yr

Young

Excellent

age,

with

site

alone

orbital
< 5 cm

surgery

Rhabdomyosacoma

152

head
chest(?);

5-yr

and neck

survival,
27%-

muscle(?

59%

);

Leiomyosa Smooth

Retroperi 40-70 yr;

rcoma

to-neum, when in
Gt tract,

children,

free,

vascular

any age

33%

muscle

Epitheloid

tissue

form

< 5 cm Discase
1-5 yr,

Stomach

Excellent
with

surgery
alone
Liposarco
ma

Primitive Extremit

Two

Child,

y,

peaks in

myxoid metastasi

o-neum

childhood:

mesench retroperit
yme

0-2

yr and

2nd

decade;us
ually

type

Rarely
zes:

prognosi
s very
good
with

complete
excision

Rhabdomyosacoma

153

occurs in
6 th

decade
a
b

Low histologic grade

Carney triad:

pulmonary chondroma,

early stage

prognostic factors

gastric epitheloid leiomyosarcoma,

functioning extra-adrenal paraganglioma.

RMS

cytogenetics,
differentiation

cross striation

immunohistochemistry
electron microscope

cell culture

oncogene

expression

HISTOLOGIC CLASSIFICATION

subtype

RMS

histologic

Rhabdomyosacoma

154

adult (pleomorphic) RMS

conventional classification

19.2

Horn and Enterline

Histologic Subtypes

Undifferentiated
Subtypes

1958

Primary Sites

(5)

Rhabdomyosarcoma

Sarcomas

Predominant

(4,6,7)

Frequen
cy

Usual

Ages

(years)

57

3-12

vagina,

0-3, 4-8

Alveolar RMS

Extremities, trunk,

19

6-21

Pleomorphic

Extremities, trunk

30-50

Embryonal
RMS
-

RMS

Head, neck, orbit

GU tract

Botryoid Bladder,

nasopharnx

RMS

Undifferentiated

perineum

Extremities, trunk

10

(EOE, Extremities, trunk

(rare in
children)
6-21

Sarcoma
(UNDS)
Other

PNET)

6-21

Rhabdomyosacoma

EOE

extraosseous Ewings sarcoma

PNET

primitive neuroectodermal tumor

Intergroup Rhabdomyosarcoma Study (IRS)

(8,9)

155

RMS

histologic subtypes

1. Anaplasia

RMS

Palmer classification

2. Monomorphous round cell pattern

3. Mixed

RMS

19.3

prognostic classification
(

19.3)

childhood

Rhabdomyosarcoma Classification

Convention

Draft

al

Classification

Classificati

(4)

Comment

on*
Botryoidal

Good prognosis(I)

Smooth muscle-like spindle

Leiomyomatous

and

Botryoidal

cell

fibrous

survival

comparable

tumor
to

with

sarcoma

Rhabdomyosacoma

156

botryoides added
to best prognosis category
Embryonal

Alveolar

Intermediate

No

Embryonal

a stroma-rich, spindle cell

prognosis (II)

Poor

alveolar

pattern

acceptable, Must be

is

tumor

prognosis Any alveolar pattern

(III)

Same cytology, no alveolar

Solid alveolar

Similar,

Alveolar

pattern

Primitive RMS

rare,

but

myogenesis

detectable mostly by

immunocytochemistry
SUH, UD, Worst
EOE

prognosis All are non-RMS tumors

(IV)

EOE
Undifferentiated
sarcomas

Defined non-RMS
sarcomas
(e.g.

synoviosarcoma)

Many are PNETs; some

may be

RMS, but must be

proven to be

included in III

Rhabdomyosacoma

SUH,

sarcoma

undifferentiated

of

157

undertermined

sarcoma;

EOE,

histology;

extraosseous

UD,

Ewings

sarcoma. (Devised by the International Soft-Tissue Sarcoma

Pathology Classification Committee)
1. Embryonal RMS

RMS

perineum

RMS

Botryoid variant

RMS

57%

RMS

19%

embryonal RMS

2. Alveolar RMS

mucosa

6%

33%

perineum

(19%)

RMS

3. Pleomorphic RMS (adult form)

(1%)

4. Undifferentiated sarcoma (UDS)

mesenchyme

Extraosseous

Ewings

extraskeletal Ewings sarcoma

(10,11)

alveolar RMS

sarcoma

retroperitoneum

(EOE)

10%

5.

embryonal RMS

Rhabdomyosacoma

158

CLINICAL PRESENTATION

(4,6)

Primary site

(65-80%)

(12)

20%

RMS

(eventual metastasis)

(4)

19.4

RMS

UDS

19.4

Rhabdomyosarcoma

(RMS)

Undifferentiated Sarcoma (UDS)

Primary Site

Relative

Frequency(

Regional
spread

Distant

Metastatic

%)
Head
neck

Orbit

and

Sites

40
10

Nodes

Lung

Rhabdomyosacoma

Paramening
eal

Other

Genitourinary
tract

Bladder-

20
10
20
12

prostate

Vagina-

uterus

ar

Paratesticul

159

infrequent

(infrequent)

Nodes

Lung

CNS-50%

Lung, bone

infrequent

Nodes

Lung,

infrequent

(mainly

from

prostate
lesions)

Nodes

Retroperitoneal

infrequent
6

marrow

nodes

(mainly

from uterus)

Retroperitone Lung, bone

al

lymph nodes
= 30%
Extremities

20

Nodes

Trunk

10

Node

Lung, bone

Other

10

Nodes

Lung,

20%

infrequent

= Lung,

marrow,

bone,CNS

bone,

Rhabdomyosacoma

160

infrequent

paratesticular

nodes

regional nodes

biopsy

RMS

regional nodes (33%) ,

4 (10%)

(chemosis)

regional

(33%),

(58%)

(22%),

(20%)

(13)

Head and Neck Region

1

liver

ocular paralysis

40%

RMS

proptosis

( 19.5)

19.5

Rhabdomyosarcoma

(6)

Location
Head

Neck
- Orbit

Clinical Presentations

and
- Proptosis, chemosis, strabismus, ocular
paralysis, conjunctival
mass, blindness

- Nasopharynx: simusitis, local pain and

Parameningeal swelling, epistaxis,

Rhabdomyosacoma

161

dysphagia.

- Nose: simusitis, nasal discharge, local

pain and swelling,
epistaxis.

Middle

ear:

Chronic

polypoid mass in external

- neck
GU tract

- Bladder and
prostate

otitis

media,

canal, peripheral facial nerve palsy.

- Soft tissue mass, hoarseness, dysphagia.

- Urinary obstruction, hematuria, dysuria,
recurrent urinary

tract infections.

- Vagina and - Vaginal bleeding, grapelike mass.

uterus

- Testicle

- Painless paratesticular mass

Trunk

- Asymptomatic mass.

obstruction.

Extremities

- Asymptomatic mass.

Retroperitoneu Pelvis

Abdominal

mass,

pain,

intertinal

- Constipation, GU obstruction

Parameningeal

RMS

paranasal sinus, middle ear

mastoid

nasopharynx,

Rhabdomyosacoma

162

discharge

CNS involvement
GU tract

25-35%

RMS

cranial nerve palsies

prostate

polypoid

vagina

botryoid

mucosanguinous discharge
Extremities

DIAGNOSTIC EVALUATION

1. Complete history
2. Physical examination

3. Complete blood count

4. Urinalysis

5. Serum electrolytes, BUN, creatinine, SGOT, SGPT,

LDH, and alkaline

phosphatase

tomography (CT) of

primary lesion

6. Magenetic resonance imaging (MRI) and computed

7. Chest CT scan

8. Skeletal survey (including long bones, pelvis, skull,

and spine)

Rhabdomyosacoma

163

9. Bone scan

10. Bone marrow aspiration and biopsy

11. Biopsy of suspicious lymph nodes

19.6

RMS

1. Parameningeal head and neck

a. Ear, nose, and throat examination under

anesthesia

b. Ophthalmologic examination
c. Cerebrospinal fluid cytology
d. Brain and head CT scan
e. MRI

f. Surgical intervention: incisional biopsy; gross

removal usually not feasible

Rhabdomyosacoma

164

2. Genitourinary, abdominal, pelvic

a. Cystoscopy, vaginoscopy
b. Ultrasonography

c. CT scan of retroperitoneal nodes

d. MRI

e Surgical intervention: incisional or cystoscopic

biopsy, radical inguinal

orchiectomy, retroperitoneal lymph node biopsy

(for paratesticular
3. Thorax

rhabdomyosarcoma)

a. Chest radiograph and CT

b. MRI
4. Extremities

a. Bone radiographs
b. MRI

c. Surgical intervention: biopsy of regional lymph

node, wide local excision if

possible

5. Trunk

a. MRI (or myelogram) if lesion is paraspinal

possible

b. Surgical intervention: complete excision if

Rhabdomyosacoma

165

6. Gastrointestinal
a. Liver scan

b. Surgical intervention: complete excision if

possible

STAGING

Staging system

Surgical-Pathologic system

(14-16)

(clinical group) system

Study (IRS)

1972

Surgical-Pathologic

Staging

Intergroup Rhabdomyosarcoma

19.7)

19.7

Clinical Grouping System

Rhabdomyosarcoma Study (IRS)

Grou

Intergroup

(16)

Definition

p
I.

A. Localized, completely resected, confined to site

of origin

B. Localized, completely resected, infiltrated beyond

site of origin

Rhabdomyosacoma

166
II.

A. Localized, grossly resected, microscopic residual

B.

Regional

disease,

completely resected

involved

lymph

nodes,

C. Regional disease, involved lymph nodes, grossly

resected with

microscopic residual
III.

A. Local or regional grossly visible disease after

biopsy only:

B. Grossly visible disease after

50%

primary tumor
IV.

Distant metastasis present at diagnosis

TNM (tumor, node, metastasis)

(widest diameter)

19.8

adult tumor staging

pediatric sarcoma

resection of

(15,17)

X-ray

histologic subtype

TNM Pretreatment Staging System (IRS-IV)

(4,6)

Stage

Sites

Invasiven

Size

Rhabdomyosacoma

167

ess
I.

Orbit
Head

T1 or T2 a or NO or N1 M
and

or Nx

or Nx

neck T1 or T2 a or NO or N1 M

(excluding

parameningeal)

Genitourinary
(nonbladder/

T1 or T2 a or NO or N1 M
b

or Nx

nonprostate)
II.

Bladder/prostate

T1 or T2 a

NO or Nx

Extremity

T1 or T2 a

NO or Nx

Cranial

T1 or T2 a

NO or Nx

(includes T1 or T2 a

NO or Nx

N1

parameningeal
Other

trunk,
etc)
III.

O
O
O
O

retroperitoneum,

Bladder/prostate
Extremity
Cranial
parameningeal

T1 or T2 a
T1 or T2 b
T1 or T2 b

NO or N1 M
or Nx

or Nx

NO or N1 M

Rhabdomyosacoma

168
Other

(includes T1 or T2 b

trunk
etc)
IV.

NO or N1 M
or Nx

retroperitoneum,

All

T1or T2

a or NOor N1

T. Tumor

N. Regional nodes

M. Metastasis
T1. Confined to anatomic site of origin
involved

NO. Not clinical

MO. No distant metastasis

T2. Extension

N1. Clinically involved

M1. Distant metastasis present a. 5 cm in diameter

NX. Clinical status unknown
b. >5 cm in diameter

* Distant metastatic disease

marrow, brain, distant muscles

abdominal fluid

nodes.

lung, liver, bones, bone

PROGNOTIC CONSIDERATION

CSF pleural

stage IV

Rhabdomyosacoma

1. Clinical group

169

group I

(18)

2. Primary site

(19)

3. Histopathologic type
RMS

UDS

4.Response to treatment

alveolar

embryonal RMS

(20)

(21)

TREATMENT

RMS

residual tumor

IRS

1. Surgery

aggressive surgery

ablative

Rhabdomyosacoma

170

vagina

(22)

biopsy

regional nodes

(33%)

(40%)

2. Radiotherapy

RMS

(15%)

GU (20%)

wide excision

local recurrence

75%

(23)

RMS

4,000 cGy

4,000 cGy

1.
RMS

IRS

UDS

cGy

3.

8-13%

32%

(4)

19.9)

group I

IV

nonalveolar

alveolar

group III

(13)

group I ()

group II
6

local recurrence

4,140 cGy

2.

alveolar

4,500-5,040

Rhabdomyosacoma

cranial parameningeal RMS

recurrence

19.9

171

mucositis

(24)

local

Primary RMS

Clinical

Age(yr);

Radiation

Group (IRS),

Tumor

Dose

Site

(cm)

Histology,

size

(cGy)

UDS

(4)

Treatme

Total Norm

nt

Dose

al

Duratio (cGy) Tissu

n

(wk)

Margi
n

(cm)

I, nonalveolar,
any site

II, any type;

Any

None

Any

180/day, 5

4-5

4140

172

Rhabdomyosacoma

I,

d/wk

alveolar,

any site

III + IV, any

type,

<6 yr; <5 180/day, 5

any

except

site cm

d/wk

<6 yr; 5 180/day, 5

special cm

pelvic sites

yr; <5 180/day, 5

yr;

cm
cm

III,

d/wk

bladder,

sites-

4140

4500

4500

5-6

5040

5-6

5040

180/day, 5
d/wk

(above)

d/wk

IV

prostate;

vagina,

uterus*
*

d/wk

special As for III 180/day, 5

pelvic

4-5

normal tissue tolerance

IRS

Rhabdomyosacoma

2,400 cGy

173

1,200 cGy

150 cGy

1,800 cGy

External-beam megavoltage

radiation implant

vagina

fibrosis

(25)

Wilms tumor

RMS

neuroblastoma

3. Chemotherapy

(26)

microscopic residual tumor

IRS

RMS

regimens

regimen

histology ( group I
histology ( group I

19.10

II

II)

alveolar)

RMS

Clinical Group,

Operative

Radiation

Histology, Site

Management*

Therapy

I,

nonalveolar, Complete

any site

excision,

None

unfavorable

group III

UDS

favorable

IV

(4)

Chemotherapy**
AMD x 5, VCR x
6; six 9-wk courses

Rhabdomyosacoma

174

margins clear

in 54 wk (Reg. 31)

I, alveolar, any As for group I,

site

nonalveolar

Begin
14;

day VCR,

CPDD,

ADR,

( 19.9)

CTX,

AMD, x 1 yr

II, nonalveolar, Grossly complete As for group Randomize:

any site

excision with (A) I,

Reg.32 (same as

residual,

VCR, and ADR

microscopic
regional

alveolar

(B)

31)

vs

(total

AMD,

of

360

extension

mg/m ) x 1 year

excised, or

(Reg. 33)

(C) both A and

B

II, alveolar, any As for group II,

site
II

nonalveolar

As for group Reg. 38

I,

alveolar

III, Grossly complete As for group Reg. 32

nonalveolar,

(II) or

meningeal

excision

cranial nonpara- incomplete

II + III + IV, As
any

(III)

II or
III

for Begin

nonparameningeal day 0 if

RT Group II:

Randomize: Reg.

histology,

meningeal

32 vs Reg. 33

parameningeal

present;

vs. Reg. 35

cranial

signs

otherwise

Group III: Reg. 34

vs Reg. 36

Rhabdomyosacoma

175

begin day 14
(II) or

42 (III, IV)

19.10

RMS

UDS

(4)

()

Clinical Group,

Operative

Radiation

Chemotherapy*

Histology, Site

Management*

Therapy

III,

pelvic

special Incisional

cystoscopic

(bladder,

uterus)

VCR,

bladder trigone ADR,

biopsy

prostate,
vagina,

or Start wk 6 for
and

cervix,

CPDD,
CTX,

AMD x 2 yr,

prostate with RT at wk

lesions; start
wk

20

6 (Reg. 37-B)

if or with optional

tumor present

RT at wk 20

after excisional (Reg. 37-A)

biopsy
of

lesion

arising in

vagina-uterus

or

bladder dome

III,

histology,
other sites

any Incomplete
all excision

incisional

biopsy only

or

( 19.9)

Randomize:

repetitive pulse

VAC x

2 yr (Reg. 34)

Rhabdomyosacoma

176

vs Reg.

38 drugs x 2

yr (Reg.

35) vs Reg.

35 +VP-16

for 2 yr (Reg.

IV,

any Excise

histology,

any site except

possible

cranial

parameningeal

if

( 19.9)

36)

Randomize:
repeti-

tive

pulse

VAC x

2 yr (Reg. 34)

vs

Reg. 38 drugs

x2

yr (Reg. 35)

vs

Reg. 35 + VP-

16

for 2 yr (Reg.

36)

Reg = regimen; AMD = actinomycin, VCR = vincristine;

CPDD = cisplatin;

ADR adriamycin (doxorubicin); CTX = cyclophosphamide;

VP-16 = etoposide.

Rhabdomyosacoma

177

(Phenomenon of multidrug resistance)

VAI

VAC (vincristine, actinomycin D, cyclophosphamide),

(vincristine,

actinomycin

D,

ifosfamide)

(vincristine, ifosfamide etoposide) regimen

VAC
IV

(27)

repetitive pulse VAC

(28)

1-2

(4)

IRS

etoposide (VP16)

19.11

tinum

High-Dose Chemotherapy

ifosfamide,

G-CSF

(6)

Dose

Carbopl 450
a-

Course #1
Drugs

carboplatinum ( 19.11)

neutropenia

relapse

autologous bone marrow transplantation

IRS-

group IV

VIE

Days
1, 2

mg/M /d

Course # 2
Dose
600

Days
1, 2

mg/M /d

Course # 3
Dose
750

Days
1, 2

mg/M /d

Rhabdomyosacoma

178
VP 16

1,200

Ifosfa-

3 g/M

mide

ABMT
G-CSF

4, 5

mg/M /d
3.5

g/M

2,000

4, 5

4 g/M

mg/M /d
2

4, 5, 6

8
5-16

g/kg/d

IV

1,500

SC
a

mg/M /d

Rest

or

5-16

g/kg/d

SC
IV

or

5-16

g/kg/d

SC

or

IV

G-CSF (granulocyte-colony-stimulating factor) 24

autologous

bone marrow transplant (ABMT).

TREATMENT RESULTS AND PROGNOSIS

(4)

IRS

Rhabdomyosacoma

1.

19.12)

group I

19.12 Three-Year Survival

Clinical Group

3-Year Survival (%)

IRS-I

IRS-II

87

88

III

57

68

II

74

IV
2.

77

23

32

179

19.13 Three-Year Survival

perineum

GU tract ( 19.13)

(IRS-II)
Primary Site

% Alive

Orbit

93

GU tract (except group III SP)*

80

Cranial parameningeal

71

GU tract (mainly group III SP)*

Other head and neck

64
69

Rhabdomyosacoma

180
Trunk

57

Retroperitoneum-Pelvis

46

Extremity

56

* SP = Special pelvic sites (bladder, prostate, vagina, cervix,

uterus)

3.

Histology

nonalveolar RMS

19.14

(IRS-II)

alveolar

RMS

undifferentiated sarcoma ( 19.14)

Three Year Survival

Histologic Type

(4)

Histologic Type

% Alive

Embryonal

69

Alveolar

56

Other

4. Recurrence

IRS-II

group

17%

IV

5%

group I

66

8%

relapse

relapse

33%, group II 28%, group III 20%

group

Rhabdomyosacoma

181

COMPLICATIONS OF THERAPY

arabinoside

intrathecal methotrexate

(31)

ascending myelitis

fibrosis

(second solid tumor

cranial parameningeal RMS

leukoencephalopathy

(29)

(35,36)

(32)

cytosine

multimodality therapy

growth hormone deficiency

cyclophosphamide
(34)

(30)

cyclophosphamide

(33)

acute myeloid leukemia)

(33,37)

FUTURE PERSPECTIVES

RMS

IRS-IV

20

staging system

hyperfractionated radiation

REFERENCES

182

Rhabdomyosacoma

1. Kramer S, Meadows AT, Jarrett P, Evans AE. Incidence

of childhood cancer: Experience

of a decade in a population-based registry. J Natl

Cancer Inst 1983;70:49-55.

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