PEDIATRIC

RHABDOMYOSARCOMA
AMAL HALIM
RHABDOMYOSARCOMA (RMS) IS THE MOST COMMON SOFT TISSUE SARCOMA IN CHILDREN . IN
PATIENTS WITH LOCALIZED DISEASE, OVERALL 5-YEAR SURVIVAL RATES HAVE IMPROVED TO
MORE THAN 80% WITH THE COMBINED USE OF SURGERY, RADIATION THERAPY, AND
CHEMOTHERAPY. HOWEVER, IN PATIENTS WITH METASTATIC DISEASE, LITTLE PROGRESS HAS
BEEN MADE IN SURVIVAL RATES, WITH A 5-YEAR, EVENT-FREE SURVIVAL RATE OF LESS THAN 30%.
CAUSES

• Genetic syndromes
• Enviromental exposure to cocaine,alkylating agents,and xrays
AGE

• Approximately 87% of patients are younger than 15 years, and 13% of

patients are aged 15-21 years.
CLINICAL PICTURE
PRESENTS AS AN EXPANDING MASS CAUSING CLINICAL EFFECT
ACCORDING TO ITS SITE.FOR EXAMPLE PROPTOSIS AND PAIN IN THE
EYE,HEMATURIA IN URINARY BLADDER,ETC,------
SITES OF
RHABDOMYOSARCOMA
THE MOST COMMON SITES ARE THE HEAD AND NECK (28%),
EXTREMITIES (24%), AND GENITOURINARY (GU) TRACT (18%). OTHER
NOTABLE SITES INCLUDE THE TRUNK (11%), ORBIT (7%), AND
RETROPERITONEUM (6%).
INVESTIGATIONS
LAB STUDIES AS CBC,KIDNEY AND LIVER FUNCTIONS ,FISH ,RT-PCR.
IMAGING STUDIES AS PLAINX RAY,CT SCAN,MRI,BONE SCAN.
BIOPSY FROM THE LESION.
BONE MARROW ASPIRATION AND BIOPSY.
HISTOLOGY
RHABDOMYOSARCOMA IS ONE OF THE SMALL, ROUND BLUE-CELL TUMORS OF CHILDHOOD. OCCASIONALLY,
THESE TYPES OF TUMORS CAN BE DIFFICULT TO DIFFERENTIATE. RHABDOMYOSARCOMA CELLS TEND TO HAVE
VARIABLE DIFFERENTIATION ALONG THE MYOGENESIS PATHWAY AND MAY APPEAR AS STRAP CELLS OR
MYOTUBES THAT SOMETIMES CONTAIN MUSCLE CROSS-STRIATIONS. RHABDOMYOSARCOMA CELLS MAY
DEMONSTRATE POSITIVE IMMUNOHISTOCHEMICAL RESULTS FOR MUSCLE-SPECIFIC MARKERS, SUCH AS
MYOGLOBIN, ACTIN, AND DESMIN.
TYPES:BUTRYOID,ALVEOLAR,UNDIFFERENTIATED.
TREATMENT

• It depends on many factors as group staging (next slide)

IT DEPENDS ALSO ON STAGING(BELOW)
PM(PARAMENINGEAL –SITES)
NASOPHARYNX, MIDDLE EAR, PARANASAL SINUSES, INFRATEMPORAL
AND PTERYGOPALATINE FOSSA)
RISK CATEGORIES IN
RHABDOMYOSARCOMA
SURGERY

• Complete resection of the primary tumor is needed with 1-2cm safety

margin.
• The lymph nodes should be sampled but no radical surgery of lymph nodes.
INDICATIONS OF CHEMOTHERAPY AND
RADIOTHERAPY

• Low risk cases:Vincristine +dactinomycin.

• Intermediate risk cases:VAC +radiotherapy(topotecan may be used).
• High risk cases:ifosfamide and etoposide based regimens may be added to
the standard VAC+radiotherapy.Stem cell transplantation may be used as
well.
• The location and extent of disease after surgical management largely determine the doses for radiation
treatment. In general, a margin of 2 cm around the tumor and involved nodes is the guideline for the treatment
volume.

• Chemotherapy is typically administered for 2-3 months before the start of radiation therapy. Radiation
treatment is then administered for approximately 5-6 weeks. The only exception to this rule involves patients
with parameningeal disease and evidence of meningeal spread. In this circumstance, radiation is started at the
time of diagnosis. During radiation treatment, doses of chemotherapy are altered to avoid the use of
radiosensitizing agents (eg, dactinomycin, doxorubicin). Patients with meningeal extension of parameningeal
rhabdomyosarcoma should receive whole-brain irradiation in addition to radiotherapy of the primary tumor.
NB :CHEMOTHERAPY PROTOCOLS OF RHABDOMYOSAROMA RANGES
FROM 6-12 MONTHS BUT THE CHEMOTHERAPY SHOULD START 3
MONTHS BEFORE THE START OF RADIOTHERAPY
• In general, irradiation is good for patients with evidence of gross or microscopic residual disease after resection.
Patients in group I (complete resection) typically do well without irradiation. However, radiation therapy may be
of some benefit in patients with alveolar histology, who may otherwise have a less favorable prognosis.

• Patients in clinical group II typically receive total radiation doses of 4100 cGy. Individuals in group III receive
approximately 5000 cGy. Although this dose is associated with a relapse rate of more than 30%, the long-term
toxic effects of increasing the total dose make more aggressive treatments unfeasible. Current investigators are
examining hyperfractionization and brachytherapy as alternatives to current treatment methods. In addition,
newer studies indicate that improved risk stratification enables decreased therapy intensity for selected patients
without compromising survival.
• A study by Clement et al indicated that long-term survivors of pediatric head
and neck rhabdomyosarcoma are at a significantly increased risk of
developing pituitary dysfunction, owing to treatment with radiation therapy.
• A study by Mattos et al indicated that chemoradiotherapy in children with
head and neck rhabdomyosarcoma can significantly impact dental and
craniofacial features.
OTHER TREATMENT OPTIONS
MAINTENANCE CHEMOTHERAPY.
MOLECULARTARGETED DRUGS AND IMMUNOTHERAPY
A QUESTION
A BOY OF 6 YEARS ,PRESENTS WITH A MASS IN THE UPPER THIGH AND A LYMPH NODE IN THE
INGUINAL NODE.BIOPSIES OF BOTH ,THE STAGE REVEALED ALVEOLAR
RHBDOMYOSARCOMA.
DETERMINE THE GROUP,THE STAGE,THE RISK.
DETERMINE THE TREATMENT