Rhabdomyosarcom

a
Purwandari (100100085)
Dyah Wijiana Heryani (100100229)
Supervisor: dr. Yazid Dimyati, Sp.A(K)

Introduction

Epidemiology
Genetic

Patophysiology
1 of 2 chromosomal translocations, namely, t(2;13) or t(1;13)

Classification

Classification
Botryoid RMS

Embryonal Rhabdomyosarcoma,

Clinical Presentation
REGION
Head and neck

SYMPTOMS
Asymptomatic mass, may mimic enlarged
lymph node

Orbit

Proptosis, chemosis, ocular paralysis, eyelid

mass

Nasopharynx

Snoring, nasal voice, epistaxis, rhinorrhea,

local pain, dysphagia, cranial nerve palsies

Paranasal
sinuses

Swelling, pain, sinusitis, obstruction,

epistaxis, cranial nerve palsies

Middle ear

Chronic otitis media, hemorrhagic discharge,

cranial nerve palsies, extruding polypoid
mass
Hoarseness, irritating cough

Larynx

Clinical Presentation
Trunk
Biliary tract
Retroperitoneum

Bladder/prostate
Female genital tract
Male genital tract
Extremity
Metastatic

Asymptomatic mass (usually)

Hepatomegaly, jaundice
Painless mass, ascites, gastrointestinal or
urinary tract obstruction, spinal cord
symptoms
Hematuria, urinary retention, abdominal
mass, constipation
Polypoid vaginal extrusion of
mucosanguineous tissue, vulval nodule
Painful or painless scrotal mass
Painless mass, may be very small but with
secondary lymph node involvement
Nonspecific symptoms, associated with the
diagnosis of leukemia

Staging : Stage

Staging : Group

Staging : Risk Group

Risk Group

Low Risk

Intermediate
Risk
High Risk

Histology

Stage

Group

Embryonal

I,II,III

Embryonal

2,3

I,II

Embryonal

2,3

III

Alveolar

1,2,3

I,II,III

Embryonal
Alveolar

or 4

IV

Treatment
a: <5 cm
b: >5 cm

Treatment
a: <5 cm
b: >5 cm

Low Risk (D9602)

Intermediate Risk (D9803)

Intermediate Risk
(D9803)

High Risk (D9802)

Vincristine
plus
Irinotecan

Case Report

Case Report
Name : M.A.M.D
Age : 12 years 6

months old
Sex : Male
Date of Admission :
8th November 2014

Chief Complaint
Chief Complaint : lump on left tight
Earlier History

History
Birth History

Spontaneous ; attended by midwives; Body Weight: 3500 gram; Body Length: 40 cm,
immediately crying.
Immunization History

Not clear, the parents was not remember

Feeding History
From birth to 6 months
: Breast milk only
From 6 months to 12 months
: Breast milk only + porridge
After 12 months
: Family menu
History of Growth and Development

Sitting
:
Walking :
Talking
:

4 months
12 months
12 months

Pregnant History
The patient is the fourth child from four children. Age of pregnant is 30 years old. There is no
history of fever, hypertension, diabetic mellitus, and consumed herbal medicine.

History of Family Disease

Physical Examination
Generalized Status

Localized Status
Head

Localized Status
Abdomen

Laboratory Findings
Complete Blood Count
Hemoglobin

12,30 gr%

(11,3 14,1 gr%)

Hematocrite

36,20 %

(37 41%)

Erithrocyte

4,50 x 106 /mm3

(4,40 4,48 x 106 /mm3)

Leucocyte

9,56 x 103 /mm3

(4,5 13,5 x 103 /mm3)

Platelet

281.000 /mm3

(217.000 497.000 /mm3)

MCV

80,40 f

(81 95f)

MCH

27,30 pg

(25 29pg)

MCHC

34,00 gr%

(29 31gr%)

RDW

13,80 %

(11,6 14,8 %)

Cell count

Neutrofil

64,10

(37-80)

Limfosit

20,90

(20-40)

Monosit

8,80

(2-8)

Eosinofil

6,10

(1-6)

Basofil

0,100

(0-1)

Immunoserology

Laboratory Findings

Autoimmune
CRP Kualitatif

Positive

Other test
Procalcitonin

0,38 ng/mL

<0,05

Chemical Clinic
Carbohydrate
metabolism
Glucose
Level
Ad
Random
Renal Function Test
Ureum
Creatinine

Electrolyte
Natrium
Kalium
Chlorida

100,90
mg/dL

<200

16,30
mg/dL
0,38
mg/dL

<50
0,31-0,47

134 mEq/L 135-155

3,3 mEq/L 3,6-5,5
100 mEq/L 96-106

Imaging
Chest X-Ray

Femur X-Ray

CTR 57%, slight cardiomegaly. Sinus

costophrenicus dextra and sinistra are sharp,
cardiac apex normal, no infiltration or
consolidation seen on lung field, intact bones
and normal joints.

Visible homogen consolidation on medial

part of left tight, solid mass with unclear
edge. Bone and joint are intact, no
destruction or abnormality visible.

CT-Scan

Mass on lesft musculus quadricep femoris, maligna (suspect Rhabdomyosarcoma?)

with size 12,3 x 13,8 x 17,8 cm, infiltrate part of subcutan and cutan regions. Os.
Femur around the mass intact, no destruction visible.

Pictures
In Pirngadi

In Adam Malik

Consul to
Rhabdomyosarcoma

Follow up
November 9

Follow up
November 11

Follow up
November 12

Follow up
November 14

Follow up
November 15

Follow up
November 16

Follow up
November 1

Follow up
November 18

Discussion
Patient admitted to hospital with history of lump

on left tight has been experienced since 1 year

ago, pain (-), immobile, skin colour didnt have
any change. Gradually, lump got bigger as 8
months ago, it was as big as baseball with
intermitten pain (+).
It is related with the theory that

rhabdomyosarcoma on extremity has

characteristic painless mass, gradually
bigger, may be very small but with secondary
lymph node involvement

Discussion
Previously patient had a small accident

while riding a motorbike with cousin then

was massaged by masseuse but not
resolved.
According to American Cancer Society,
Rhabdomyosarcoma of the trunk or
extremities often is first noticed after
trauma and initially may be regarded as a
hematoma. If the swelling does not
resolve or increases, malignancy
should be suspected.

Discussion

Discussion

Discussion
Patient

Intermediate Risk (D9803)

Intermediate Risk
(D9803)

Summary
It has been reported a case of a boy, 12 years

old with Rhabdomyosarcoma Stage III and

Group III. The diagnosis was established based
on anamnesis, clinical sign, symptoms,
physical findings, and laboratory results. The
patient is treated with regiment from IRS-V
Intermediate Risk category, started with
chemotherapy consist of Vincristine,
Actynomycin, and Cyclosphosphamid.
This patient is still having treatment in Adam
Malik General Hospital and having better
improvement.

Thank You