Embryonal rhabdomyosarcoma is the most common type of rhabdomyosarcoma. Around 60-70% of all diagnosed cases of rhabdomyosarcoma is of this type [1, 2].
What is Embryonal Rhabdomyosarcoma?
Rhabdomyosarcoma is known as the malignancy of striated muscles. There are 5 known types of this condition depending on the cellular changes that occur on the tumor. In embryonal rhabdomyosarcoma, the tissues have high variability in their cytology and may represent several stages of the morphogenesis of the skeletal muscles.
The cells of the tumor may have the characteristics similar to developing muscle cells of a 6-8 week-old embryo. Tumors most usually develop in the head and neck, vagina, bladder and the prostate. Although it is the most common type, embryonal rhabdomyosarcoma is more commonly found in children who are less than 5 years of age. The 5-year survivability for children who are less than 10 years old is around 60% [1, 2, 3]. Figure 1 shows an example of embryonal rhabdomyosarcoma of the vagina.
Figure 1- Embryonal rhabdomyosarcoma of the vagina
Staging
The tumor, node and metastasis staging for embryonal rhabdomyosarcoma is as follows [1]:
Stage I
The tumor is localized and involves the head and neck region. The mass should not be in the genitourinary area [1].
Stage II
The primary tumor is equal or less than 5cm in diameter. The mass is localized and its location is not included in the stage I category [1].
Stage III
Location is similar to a stage II type but its size is greater than 5cm in diameter. The lymph nodes near the mass may also be involved [1].
Stage IV
During the time of diagnosis, the tumor has already metastasized to other organs [1].
Causes and Risk Factors
The direct cause of embryonal rhabdomyosarcoma is not yet identified but there are several factors which may increase the possibility of developing this type of tumor. The presence of inherited medical conditions that cause changes in the DNA may increase the possibility of having embryonal rhabdomyosarcoma. Examples of these conditions include Beckwith-Wiedemann syndrome, neurofibromatosis type 1, Noonan syndrome, Costello syndrome and Li-Fraumeni syndrome [1, 4].
Signs and Symptoms
The signs and symptoms that can be experienced depend on the location of the tumor. Most of the time, the first symptom that can be experienced is the development of lump or swelling. Less than half of the patients would complain of pain associated with the tumor growth.
Embryonal rhabdomyosarcoma of the eye may cause bulging of the eye and affect the vision of the child. If the tumor grows in the ear or nasal sinuses, it may also cause headache, earache and congestion of the sinuses. Involvement of the bladder may lead to presence of blood in the urine. Tumors that grow in the abdominal or pelvic area may cause vomiting, constipation or abdominal pain [1, 2, 4].
Diagnosis
Health history and physical examination
This condition can be detected early because the location of the tumor makes them noticeable. Aside from the lump that is developing, the physician will have to identify all the other symptoms the patient is experiencing [1, 2].
Biopsy and imaging tests
Performing a biopsy will aid the physician to identify the type of malignancy that is present. Imaging tests such as CT scans and MRI will not only help in evaluating the presence of metastasis but it will also assist in planning for a surgical resection of the primary tumor. After these tests has been performed on the patient, the physician will be able to establish the diagnosis of embryonal rhabdomyosarcoma and provide the stage of the malignancy [1, 2].
Treatment
Surgery
If the tumor growth is still localized, the physician may perform an excision of the tumor along with a margin of healthy cells surrounding the tumor. This process is done to ensure that all cancer cells are removed from the body. If the malignancy have already spread to other parts of the body, the effectiveness of the surgical procedure in removing the mass decreases [1, 2].
Chemotherapy
This mode of treatment uses anti-cancer drugs to get rid of the cancer cells but before initiating the therapy, the patient will be assessed due to the risks associated with this therapy. The prognosis depends on the stage and location of the tumor [1, 2].
Radiation
Radiation therapy utilizes high energy beams to kill the cancer cells. This therapy is performed in combination with chemotherapy to achieve the best outcome. A patient may also undergo radiation therapy or chemotherapy after undergoing a surgical resection of the tumor to ensure that all cancer cells are eliminated [1, 2].
References
- National Cancer Institute. (2015, August 5). Childhood Rhabdomyosarcoma Treatment (PDQ®)–Health Professional Version. Retrieved from National Cancer Institute: http://www.cancer.gov/types/soft-tissue-sarcoma/hp/rhabdomyosarcoma-treatment-pdq#link/_224_toc
- Drake, A. F. (2016, March 9). Pathology – Rhabdomyosarcoma. Retrieved from Medscape: http://emedicine.medscape.com/article/873546-overview#a5
- Chen, E. (2013, April 19). Types of Childhood Cancer: Embryonal Rhabdomyosarcoma. Retrieved from St. Baldrick’s Foundation: http://www.stbaldricks.org/blog/post/types-of-childhood-cancer-embryonal-rhabdomyosarcoma/
- American Cancer Society. (2014, November 21). Rhabdomyosarcoma. Retrieved from American Cancer Society: http://www.cancer.org/cancer/rhabdomyosarcoma/detailedguide/rhabdomyosarcoma-risk-factors
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