Rhabdomyosarcoma

defintion
• Rabdomiosarkoma 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,
including muscle tissue, tendons, and
connective tissue.
 epidemiology
• In general, this tumor can be said to have a
relatively rare incidence. The incidence of
rabdomiosarcoma is around 4.3 cases per one
million cases which is more common in
children.
• The average age of a child who has
rabdomiosarcoma is a child under 15, with a
male and female ratio of 5: 3.
 etiology
• The exact cause of rabdomiosarcoma in children
is still unknown. there are indications that genetic
factors appear to have an important role in the
cause.
• alveolar subtypes are always found translocation
(2; 13) (q35; q14)
• Embryonal subtypes have yet to be found
karyotypic abnormalities, however, loss of
constitutional heterozygosity is still found on
chromosome 11 p 15
 patofisiologi
• rabdomiosarcoma originates from skeletal muscle
cells
• Botrioid is a form of embryonal
rabdomiosarcoma originating from the mucosa of
the hollow area (bladder, vagina, nasopharynx
and middle ear).
• Lesions in the extremities are more alveolar
rabdomiosarcomas.
• Rabdomiosarcoma is thought to arise from the
same embryonal mesenchyme as the cross-fiber
muscle
 Gejala klinis
• Symptoms caused depend on its location.
• In the eye socket, can cause protruding eyes and
bumps in the eye
• In the ear causes pain or bleeding from the ear hole.
• In the throat causes airway obstruction, inflammation
of the sinuses (cavities around the nose), bleeding from
the nose (nosebleeds) or difficulty swallowing.
• Urinary discharge causes urinary disorders. When
attacking the muscles of the limbs, it will cause
swelling.
 diagnosis
• History
regarding the course of the disease including a history
of the tendency of cancer in the family, and patient
complaints (if the tumor is enlarged and there is pain
and discomfort).
• Physical examination determines the location and size
of tumors and regional lymph nodes
• Laboratory tests required include complete blood, liver
and kidney physiology, serum electrolytes, calcium and,
if possible, levels of magnesium, uric acid and clotting
function. Bone marrow aspiration is also needed for
the suspected RMS parameningeal
• Radiology
1. CT scan (recognizes intracranial extension
and can also show bone involvement)
2. The system program is useful for tumors in
the bladder
3. MRI
• Biopsy (The most important element in the
diagnostic procedure is examination of tumor
tissue)
 Therapy
• Primary tumor
• Surgery: Local extensive excision and radical
broad excision
• radiotherapy is given if there are residual
tumors or metastases after surgery
• Chemotherapy can be given as an adjuvant or
non-adjuvant
• Recurrent tumors
• Inadequate surgery and tumor manipulation at
the time of surgery are the causes 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
rexection for curative purposes.
• combination of vincristine, dactinomycin and
cyclophosphamide.
 prognosis
• Prognosis depends on:
• Tumor size
• Location of the tumor
• Tumor depth
• Degree of ferocity
• Cell necrosis