A soft tissue tumor in membranes and often in the joints.
Sarcomas are uncommon malignant tumors that begin either in bones or in soft tissues such as muscles, cartilage, fat or connective tissue. Sarcomas make up about 1 percent of all cancers in adults and 15 percent of all cancers in children.
There are at least 56 different types of soft tissue sarcomas named according to the normal tissue from which the tumor is derived. In this manner, they are classified histologically according to the soft tissue cell of origin. Some soft tissue sarcomas arise in connective tissue (fibrosarcoma); nerve structures (neurofibrosarcoma); smooth muscles (leiomyosarcoma); fat (liposarcoma) and in membranes lining joints (synovial sarcoma).
As with many forms of malignancy, the cause of sarcomas is not known.
Because of the rarity of soft tissue sarcomas (non-rhabdomyosarcoma) in children and young adults, all patients should have their treatment planned by a multidisciplinary team of cancer specialists with experience treating children with soft tissue sarcomas.
The standard approach to the treatment of primary tumors in children is aggressive surgical therapy with total excision as assessed by pathologically-confirmed tumor-free margins (i.e., making sure the tumor is removed). When there is concern about the adequacy of the surgical margin, radiation therapy is indicated.
Because of the morbidity (complications) of high-dose radiotherapy in infants and younger children, every effort must be made to achieve local control by aggressive surgery. Although various chemotherapeutic agents possess activity against many of these tumors (e.g. doxorubicin, dacarbazine), the role of adjuvant (complementary) therapy is unclear. Therapeutic strategies must be individualized and related to age, histology (cell type), and primary site in order to maximize survival and minimize complications.
In older children, radiotherapy is administered if microscopic residual disease remains after surgery. If complete resection is successful without microscopic residual disease, it is unclear whether radiotherapy or adjunctive chemotherapy is necessary. However, if there is gross residual disease, attempts to provide curative therapy (including radiation therapy and multi-agent chemotherapy) may be considered.
Are there other tests needed to determine the disease margins?
How serious is this?
Should a specialist be consulted?
Will surgery be recommended? What is the procedure of the surgery? How much will be removed? What about post-operative care?
Will chemotherapy or radiation be given after surgery?
What is the prognosis?
Where can we find a support group?