RHABDOMYOSARCOMA

Definition
Rhabdomiosarkoma this word comes from Greek,
(rhabdo which means striated form, and myo which
means muscle).

Rabdomiosarkoma is a malignant tumor originating

from the soft tissue of the body, which is muscle
tissue, tendons and connective tissue.
Epidemiology
• Rabdomiosarkoma is a malignancy that is often
found in children. The highest incidence is at an
average age of 6 years and can be found from
infancy to young adulthood
• Rhabdomyosarcoma is more common in men
where 87% in patients under the age of 15 years
and 13% in patients aged between 15-21 years.
Very rarely occurs in young adulthood.
Etiology
The causes of rabdomiosarcoma are not yet clear but
those related to the increased prevalence of
rabdomiosarcoma are:
1. Some genetic syndromes
• Neurofibromatosis
• Li -Fraumeni Syndrome
• Rubinstein-Taybi syndrome
• Gorlin Basal cell nervus syndrome
• Backwith-Wiedemann syndrome
• Costello syndrom
Cont'
2. environmental factor

• Intrauterine exposure to X--rays

• Parental use of marijuana and cocain
• Previous exposure to alkylating agents
Classification
Histologically these tumors have 5 main categories,
namely:
1. Embryonal 84% (often found in the genitourinary or in
the head and neck)
2. Alveolar 9% (found in the extremities, perianal and or
perirectal).
3. Embryonal botryoid 4%
(usually in the oral mucosa with
tumor forms such as polopoid and grapes)
4. Rabdomiosarkoma Spindle Cells (this type often
appears
in the head and neck area and often in the peratesticular
area
5. Anaplastic Rabdomiosarkoma
Clinical manifestations
• The mass of Rabdomiosarkoma can be seen and
felt, can be felt pain or not.
• Bleeding of the nose, vagina, rectum, or mouth can
occur if the tumor is located in this area.
• Tingling, pain and movement can occur if the
tumor compresses nerves in the affected area.
• Protrusion and withered eyelids can identify a
tumor behind this area.
Physical examination
• A careful physical examination is very important to
determine tumor extension clinically and the
presence or absence of spread or distant
metastases. Enlargement of the tumor to the
surrounding tissue will form a pseudo capsule
known as pseudocapsule
Laboratory examination
• On blood tests: anemia due to the inflammatory
process / pancytopenia can be seen on bone
marrow.
• Liver function tests, including examinations of LDH,
AST, ALT, alkaline phosphatase and bilirubin levels
• Kidney function test
• Urinalysis (the presence of hematuria can identify
the involvement of GU tract in the tumor metastase
process)
• Electrolytes and blood chemistry
Radiology Examination
• In plain photos: chest photos are very helpful in
knowing the classification and involvement of
bones (primary) and to find out whether there is
metastasis in the lungs.
• Chest CT Scan: performed as an evaluation of
whether there is metastasis in the lungs
• MRI: increase the clarity of tumor invasion in body
organs, especially in the orbit, paraspinal,
parameningeal.
• Bone scanning: to find if there are metastases in
the bone
Therapy
1. Primary Tumor
a. Resectable tumors
Radical surgery is performed on tumors that are resectable on
condition that the tumor can be completely removed and the
tumor tissue free of malignant tumor incisions. There are 2
types of surgical procedures, namely:
• Wide local excision: for G1 and tumors still localized
• Broad excision of radicals: for G3 and tumors it has spread
regionally / KGB
If needed, combination therapy can be given, namely: surgery
+ radiotherapy / chemotherapy.
To prevent micrometastasis: surgery + radiation +
chemotherapy.
b. Inoperable tumors: radiation + chemotherapy
Cont'
2. Recurrent tumors
Inadequate surgery and tumor manipulation at the
time of surgery are the cause of local recurrence.
Some things to note are:
• Re-evaluate the degree of malignancy by doing
incisional biopsy
• Re-assess tumor extension in considering tumor re-
excision for curative purposes.