Sarcoma treatment is best delivered by experienced providers who are part of a multidisciplinary team including surgery, medical oncology, radiation oncology, pathology and radiology. Any particular patient with a sarcoma, will have treatment recommended depending on the tumor size, histologic grade (growth rate) and presence of metastasis. Metastasis are cells that spread to a second location usually involving lung, lymph nodes, bone or other soft tissues. A patient initial evaluation should involve imaging of the tumor mass (MRI with or without plain x-ray) and CT of the lung if the tumor is large or high grade. Patients should not have their tumor surgically excised or removed until imaging and a biopsy have been carried out and interpreted by an experienced pathologist. Surgical treatment is determined primarily by the size, location and grade of the tumor at its "primary site". Limb sparing surgery is preferred as a general rule in 90% of patients and the initial patient evaluation required adequate imaging, usually including an MRI and a biopsy prior to resection to determine tumor grade.
Tumor excision is frequently advised after consideration for either chemotherapy of radiation therapy prior to the tumor resection. Pre-operative chemotherapy or radiation therapy allows better tumor control and the possibility of longer survival. A recurrence rate at the tumor primary site depends on tumor size, grad and location, but typically do not exceed 15-25%.
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Radiation therapy is given to improve local tumor control and may be given before or after surgery. It usually requires approximately six weeks to deliver and results in inflammation at the tumor site with some extremity stiffness or swelling. Radiation therapy does not usually produce nausea or vomiting or significant fatigue for extremity tumors.
Chemotherapy involves drugs that control rapidly growing tumor cells. Chemotherapy is recommended for high grade sarcomas with a risk of metastasis. It is more effective in patients under age 2 years and is associated with greater effectiveness with certain tumor subtypes. Patients with high or intermediate grade tumors are larger than 5cm and should e considered for chemotherapy. Chemotherapy can be given before or after tumor resection with the latter being attractive because it allows early treatment and an early assessment of effectiveness.
Adriamycin (Doxorubicin) is the most common and effective against for sarcomas and is usually given in combination with other drugs (Ifosfamide, etc.). Patient survival has only a limited gain of (10-20%) from chemotherapy which is dependent on the tumor subtype (MFH vs leiomyosarcoma vs epithelioid sarcoma). High risk subtypes are less likely to be affected by chemotherapy. Chemotherapy is the only treatment able to control the disease that is systemic or at risk for metastasis, because chemotherapy affects rapidly growing cells. It can also make patients nauseated, anemic (affecting red blood cells) at risk of infections (affecting white blood cells) and temporarily loss of their hair.
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