Rhabdomyosarcoma and Soft Tissue Sarcomas

Soft tissue sarcomas account for about 8% of all childhood cancers. These cancers arise from soft tissues and can occur almost anywhere in the body. The most common type of soft tissue sarcoma is Rhabdomyosarcoma and the other types are often referred to as Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS).

Our dedicated team of oncologists, surgeons, radiation oncologists, and support staff work closely together and meet weekly to discuss the best care for each child, adolescent, and young adult. State-of-the-art clinical care is provided to all of our patients through access to the most current national trials, best evidence-based therapies, as well as supportive and experimental therapies.

Soft tissue sarcomas form in the blood veins, lymph system, muscles, nerves, tendons and tissue in the body. They can be approached in two main categories: rhabdomyosarcoma and Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS).

Rhabdomyosarcoma is an aggressive cancerous tumor of the muscle. It can occur throughout childhood and adolescence and has even been seen at birth. Approximately two thirds are seen in children less than 6 years of age. It usually begins in the muscles around the head, near the brain, in the urogenital tract or in muscles in the arms or legs.

Non-Rhabdomyosarcoma Soft Tissue Sarcomas (NRSTS) include numerous types of sarcomas that can develop anywhere in the body but most commonly in arms and legs. Types include clear cell sarcoma, malignant peripheral nerve sheath tumor, fibrosarcoma, leiomyosarcoma, synovial sarcoma, epitheliod sarcoma, malignant fibrous histiocytoma, liposarcoma, and undifferentiated sarcomas. NRSTS are more commonly seen in infants less than one year and in adolescents. Certain families may have a genetic predisposition for sarcomas and people who have received prior radiation therapy.

Soft tissue sarcomas are often asymptomatic until the mass is noticeable and palpable. The symptoms for these sarcomas vary depending on the type of disease, but most often will include one or many of the following:

Pain at the tumor location
Swelling or a lump at the location of the tumor
Unexplained weight loss
Fatigue
Respiratory symptoms if spread to lung
Tumors in nose may cause congestion, bleeding, or neurological symptoms
Tumors around eye may cause swelling or bulging of eye, or vision changes
Tumors in bladder, prostate and urogenital tract may cause difficulty or inability to urinate or stool.

In order to determine the how to treat your child's cancer, tests must be done to determine what type of soft tissue sarcoma your child has and what stage the cancer is. The doctor will ask about symptoms and medical history. A physical exam will be done. Addtional tests may include:

Blood and urine tests
Sample of the tumor tissue. This can be done with a biopsy or if possible resection of tumor.
Depending on type and location may need bone marrow evaluation and spinal tap (lumbar puncture).
Images of bodily structures. This can be done with:
- X-ray
- Computed tomography (CT) scan
- Magnetic resonance imaging (MRI) scan
- Bone Scan
- PET scan

After cancer is diagnosed, staging tests are performed to find out if the cancer has spread and, if so, to what extent. Treatment depends on the type, stage, and location of the cancer. Your doctor will talk with you about the best treatment plan for your child.

Rhabdomyosarcoma will be treated with chemotherapy after biopsy or removal of tumor. Depending on initial tumor size, histology (alveolar versus embrynal), and spread of tumor, radiation therapy and/or additional surgery may be needed.

NRSTS treatment and responses vary widely depending on type and location. Treatment usually involves upfront surgical resection if possible and if not chemotherapy and/or radiation may be used to shrink the tumor.

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Rhabdomyosarcoma and Soft Tissue Sarcomas

What are soft tissue sarcomas?

What are symptoms of sarcoma?

Testing and diagnosis

Treatment

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