Rhabdomyosarcoma is a malignant soft-tissue tumour derived from skeletal muscle. The skeletal muscle tumours are of two types- that is either the rhabdomyomas which are the benign type or the rhabdomyosarcomas which are the malignant type. It is the third most common solid tumour seen in childhood, the first two being the neuroblastoma and the Wilms’ tumour.
What exactly do we mean by skeletal muscle ?
The body is seen consisting of 3 basic muscles. These are –
- The Skeletal (voluntary) muscles: These muscles are basically the voluntary muscles of the body. They move parts of our body, as per our will. For example, if you want to lift your hand, the skeletal muscles in your forearm get to work.
- The Smooth (involuntary) muscles: Smooth muscle is your involuntary muscle in the body that does all its automatic functions, without you even knowing. For instance, the smooth muscles that are present in the stomach and your intestines do the process of food propelling and digesting, in an auto mode.
- The Cardiac muscle: As the name suggests, this is the main muscle type responsible for functioning of the heart.
Where does rhabdomyosarcoma occur ?
The Rhabdomyosarcoma basically originates from precursor cells called rhabdomyoblasts (which later on give rise skeletal muscles). As rhabdomyoblasts are embryonal or immature cells, it is a far more common tumour in children, as compared to adults. Common sites of primary disease include the head and neck region, genitourinary tract such as the bladder and the limbs.
How is rhabdomyosarcoma caused ?
Though most cases of Rhabdomyosarcoma are sporadic, the disease has been linked to familial syndromes such as neurofibromatosis and the Li-Fraumeni syndrome (LFS). Rhabdomyosarcoma has been associated with mutations of the p53 tumour suppressor gene.
The two histologic subtypes of RMS, embryonal and alveolar, have been found to have distinctive genetic alterations that may play a role in the pathogenesis of these tumours.
What are the clinical features of rhabdomyosarcoma ?
It is found occurring in multiple primary sites. It usually presents as an asymptomatic mass. Otherwise it causes site-specific symptoms. If it occurs in the eye area, it causes orbital symptoms like proptosis (eyeball appearing to pop out) and ophthalmoplegia (paralysis of the eye muscles). Parameningeal sites include middle ear, nasal cavity and paranasal sinuses. Therefore, parameningeal symptoms are nasal or sinus obstruction and/or headache. If it occurs in the genitourinary tract, symptoms seen are hematuria (blood in urine), blockage of urinary flow or stool movement (constipation).
How is rhabdomyosarcoma diagnosed ?
A complete physical examination of the body is mandatory. Laboratory studies should include a complete blood count and extensive diagnostic work-up of serum ions such as phosphorus, magnesium and calcium. Serum creatinine, uric acid and nitrogen also should be assessed.
Baseline coagulation studies should be performed.
Radiologic evaluation should include plain radiographs of the primary site along with the more advanced computed tomography (CT) scan of the main tumour and the surrounding structures. In some cases, extent of the disease can be aided by magnetic resonance imaging (MRI) or ultrasonography.
Histologically, by light microscopy, the tumour rhabdomyosarcoma will exhibit skeletal muscle tissue or rhabdomyoblasts.
What are the variants of rhabdomyosarcoma ?
There are 2 chief types of Rhabdomyosarcoma, along with some less common types.
- Embryonal rhabdomyosarcoma: Embryonal rhabdomyosarcoma (ERMS) usually affects infants, occurring usually in the urinary bladder and vagina. It is seen to be the most frequent type of rhabdomyosarcoma at all ages.
There are other two subtypes of embryonal rhabdomyosarcoma- the botryoid rhabdomyosarcoma and the spindle cell rhabdomyosarcomas, besides the conventional one. These are inclined to have a better prognosis than the more common conventional form. - Alveolar rhabdomyosarcoma: The Alveolar rhabdomyosarcoma (ARMS) is seen in older children, adolescents and teens. It is seen to occur most often in the trunk, the arms and the legs. It is usually the aggressive variant, and is seen to undergo metastasis, as well. It therefore, requires more aggressive treatment.
- Anaplastic rhabdomyosarcoma and undifferentiated sarcoma : Anaplastic rhabdomyosarcoma is an uncommon type that occurs more often in adults but is rare in children.These uncommon cancers are aggressive and pleomorphic. They tend to enlarge quickly and usually require intensive therapy.
How are rhabdomyosarcoma treated ?
- First the tumours have to be staged. This has to be done both locally and systemically, after analyzing the spread of the tumour.
- Secondly, biopsy has to be done to confirm rhabdomyosarcoma. It is the confirmatory test.
- The chief types of treatment that can be used for rhabdomyosarcoma include:
Surgery
Chemotherapy
Radiation therapy
High-dose chemotherapy and bone transplant by stem cells (done extremely rarely). - Majority of cases of rhabdomyosarcoma are to be treated with surgery to get rid of the tumour, if major damage or disfigurement does not ensue.
- If surgery is not possible, the next line of therapy is to use chemotherapy and/or radiation therapy to possibly shrink the tumour. Once it shrinks, the surgery can then be performed. The main goal of surgery is to remove the tumour totally and completely, but this is not always achievable.
- In cases of metastasis or aggressive disease, radiation therapy/ chemotherapy can also be given.
- All of these treatments do have their side effects, but many of these side effects can be managed or alleviated by your oncology team of doctors and nurses.
How does a rhabdomyosarcoma behave ?
Despite aggressive treatment using all the approaches such as surgery, dose-intensive combination chemotherapy, and radiation therapy, the result for patients with metastatic disease remains reduced.
What is the care to be taken after the removal of a rhabdomyosarcoma ?
Metastatic diagnostic workup and monitoring post-treatment has to include imaging and scanning of the limbs, thorax, chest, retroperitoneum, and abdomen with radiography, MRI or CT scans. Adjuvant chemotherapy or radiotherapy may be required in cases wherein excision cannot be done completely.
Can rhabdomyosarcoma be prevented ?
The risk of many adult cancers can be definitely reduced with healthy lifestyle choices (such as healthy diet or quitting tobacco habits), but so far, we do not know how to prevent these cancers in children. The major risk factors for rhabdomyosarcoma (RMS) include age, gender, and certain inherited conditions cannot be changed or modified. However, most children with rhabdomyosarcoma can be successfully treated.
How to find and reach cancer specialists for rhabdomyosarcoma treatment ?
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Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.
