What are the other Names for this Condition? (Also known as/Synonyms)

• Sclerosing Pseudovascular Rhabdomyosarcoma in Adults
• Sclerosing Rhabdomyosarcoma in Adults
• SRMS (Sclerosing Rhabdomyosarcoma)

What is Sclerosing Rhabdomyosarcoma? (Definition/Background Information)

• Sclerosing Rhabdomyosarcoma (SRMS) is an infrequent, malignant skeletal muscle tumor of the soft tissues, that are normally observed in adults
• The tumors are poorly-defined, fleshy, expanding masses that form deep within the body tissues, or below the skin surface. It may develop due to inherited or random gene mutations
• The tumor location is normally the arms, legs, and head and neck region. The signs and symptoms are dependent upon the location of the tumor
• Sclerosing Rhabdomyosarcoma may be treated using a combination of measures that include surgery, chemotherapy, and radiation therapy
• The prognosis of Sclerosing Rhabdomyosarcoma is normally poor. The cancer has been observed to metastasize (spread) to the lungs and bones

Who gets Sclerosing Rhabdomyosarcoma? (Age and Sex Distribution)

• Sclerosing Rhabdomyosarcoma is a rare tumor that is commonly present in adults, in a wide age range of 18-56 years. However, children and adolescents can also get it
• There is a slight predisposition towards the male sex
• There is no geographical, racial, or ethnic preference noticed

What are the Risk Factors for Sclerosing Rhabdomyosarcoma? (Predisposing Factors)

• The risk of Sclerosing Rhabdomyosarcoma is thought to be linked to inherited genetic defects

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Sclerosing Rhabdomyosarcoma? (Etiology)

• The cause and mechanism of Embryonal Rhabdomyosarcoma formation is unknown
• It is suspected that either inherited genetic defects or de novo (sporadic and spontaneous) genetic mutations may be the causative factors

What are the Signs and Symptoms of Sclerosing Rhabdomyosarcoma?

The presentations are based on the location of Sclerosing Rhabdomyosarcoma. The signs and symptoms include:

• In the initial growing phase of the tumors, they are normally asymptomatic
• As tumor grows rapidly, its presence may be felt by pain and a sensation of mass. The mass can cause compression of the adjacent organs affecting their function. The tumor size may range from 0.3-11.8 cm (mean size 6 cm)
• Most lesions occur in the arms and legs, followed by the head and neck (specifically in the nasal passage or nasopharynx, jaw bones, and around the orbit of the eye). It may be also found in the sacrum of the spine (mid-back region)
• Some of the symptoms noted on the head and neck region include: Bulging of the eyes, double vision, inflammation of the nasal cavity, nosebleeds, congestion, headaches, difficulty speaking and swallowing

How is Sclerosing Rhabdomyosarcoma Diagnosed?

Sclerosing Rhabdomyosarcoma may be diagnosed through the following tests and exams:

• A complete physical examination with thorough evaluation of the individual’s medical history
• Ultrasonography of the affected region
• CT, MRI scan of the affected region
• Whole-body PET scan, bone scan of affected region to check for tumor metastasis. This helps with the staging of the tumor
• Tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
• Differential diagnosis, to eliminate other tumor types is considered, before arriving at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Sclerosing Rhabdomyosarcoma?

The complications are dependent on the site and stage of the tumor. It is easier to treat the primary tumor; but if metastasis occurs, treatment can be challenging. The complications from Sclerosing Rhabdomyosarcoma could include:

• Functional impairment of organs due to mass effect
• Metastasis to the lungs and bones
• Damage to vital nerves, blood vessels, and surrounding structures during surgery to remove Sclerosing Rhabdomyosarcoma
• Side effects from chemotherapy (such as toxicity), radiation therapy (radiation fibrosis)

How is Sclerosing Rhabdomyosarcoma Treated?

Treatment measures for Sclerosing Rhabdomyosarcoma may include the following:

• Wide surgical excision of SRMS with removal of the entire tumor
• This may be followed by radiation and chemotherapy
• Arterial embolization of the tumor is used to provide temporary relief from the symptoms, and reduce blood loss during ‘tumor removal’ surgical procedure
• When the tumor is at an inaccessible location, or is unsafe for surgical intervention; non-invasive procedures are adopted
• Peripheral blood stem cell transplant (PBSCT) procedure
• Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
• Follow-up care with regular screening and check-ups are important, since the tumor can recur in some cases

How can Sclerosing Rhabdomyosarcoma be Prevented?

• Current medical research have not established a way of preventing Sclerosing Rhabdomyosarcoma
• Regular medical screening at periodic intervals with blood tests, scans, and physical examinations are mandatory; due to its metastasizing potential and chances of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Sclerosing Rhabdomyosarcoma? (Outcomes/Resolutions)

• Sclerosing Rhabdomyosarcomas are rare, but malignant cancers, and the prognosis is typically poor
• Nevertheless, the long-term prognosis may depend on a combination of factors such as; age of the individual, tumor stage at detection, size, type, and location of the tumor, and whether it has spread to surrounding areas, or to other distant locations

Additional and Relevant Useful Information for Sclerosing Rhabdomyosarcoma:

• Amongst pediatric soft tissue sarcomas (affecting children less than 15 years), rhabdomyosarcomas form the largest category of sarcomas
• Embryonic, alveolar, and pleomorphic rhabdomyosarcoma are the 3 types of rhabdomyosarcoma