Soft+Tissue+Sarcoma

Symptoms are often nonspecific. Average time of onset to diagnosis is approximately 4 to 6 months. The size of the tumor is site dependant. "Other presenting symptoms of site specific STS are weakness, increased pressure such as paresthesia, distal edema, or presence of warmth or distended vascularity when fixed to other structures."  5 || Detailed exam with size and mass characteristics for the limb lesion with information on the proximity to any joints. Complete Blood cell count and chemistry profile Plain radiographs of the involved site Computed tomography (CT) with contrast or a Magnetic Resonanse imaging to evaluate neurovascular compromise and possible mass fixation to bone Chest radiograph Ct scan of chest ( most common metastatic site: lung) PET scan ( metastasis) Bone scan Arteriography pr magnetic resonance angiogram Lymphangiogram Ultrasound 1 Figure 1.1 Sarcoma of the thigh. 7 || - Tumors of the trunk, head, and neck may invade adjacent structures earlier. - Lymph node metastases are uncommon, but are an ominous sign when they occur. - Skin involvement is seen in approximately 10% of patients. - Hematogenous metastases is the most common pattern of metastatic disease and occurs more frequently as the tumor enlarges. - The lung is the most common site of metastatic disease. 1 || Table 1. AJCC grading system used for soft tissue sarcoma 1 || Table 2. AJCC TNM Staging system Table 3. AJCC stage groupings 1 || Long term: Soft Tissue Sarcomas require high doses of radiation therapy to achieve local control. - Preoperative doses = 5000cGy. - Postoperative shrinking fields are used to deliver 6500 cGy with reductions at 4500 to 5000cGy and 5500 to 6000cGy. 3 - The total dose may exceed 7500cGy after boost. - 3-d planning and IMRT planning allow increased doses delivered to target volumes. - Scar should receive full dose. Electrons may be used with a bolus. - Immobilization is critical. - Total circumferential radiation of extremities should be avoided. 1-3cm strip of skin and tissue should be spared to avoid lymphedema. - Chemo is given in some cases and the radiation dose would be modified. - Brachytherapy using Ir-192 is a useful alternative for patients with recurrent extremity SRS.  3 - Catheters are placed in tumor bed at time of resection. - Figure 1.1 shows catheters placed for brachytherapy treatment of a synovial sarcoma.. 6 Figure 1.2 Brachycatheters placed after wide resection. 6
 * ​Epidemiology: || Sarcomas make up a relatively rare group of malignancies arising from the connective tissues of the body. They can occur within any organ and any anatomic location within the musculoskeletal system. The thigh is the most common subsite of origin. 1 Soft tissue sarcomas can occur at any age. Patients under the age of 40 make up 20% of all cases, and 50% are over the age of 60. 4 ||
 * Etiology: || Most soft tissue sarcomas are sporadic, with no specific identifiable etiologic agent. Almost all sarcomas due to antecedent etiologic factors are high-grade tumors, a classic example of which is a radiation-induced sarcoma. A radiation-induced sarcoma arising in soft tissue or bone can develop no sooner than 3 years after completion of a course of theraputic radiotherapy; frequently, these lesions will develop decades later. Typically, such lesions are osteosarcomas malignant fibrous histiocytoma. Phenoxy herbicide exposure in forestry workers has been linked with subsequent sarcoma development. Other agents implicated in the etiology of soft-tissue sarcoma include exposure to diozin, vinyl chloride, arsenic, and Thorotrast, a thorium-based suspension formerly used as a contrast agent in radiologic studies. Chronic lymphedema is a factor predisposing to the subsequent development of lymphangiosarcoma. Though at the time of presentation patients frequently recall a recent history of trauma, no scientific evidence directly connects such injury to the inception of soft-tissue sarcoma. Instead, it is thought that the injury draws attention to a specific body part or location, thereby increasing the likeliness of detecting an otherwise painless and frequently innocuous soft-tissue mass. Several genetic conditions are related to the development of soft-tissue sarcoma. They include neurofibromatosis, tuberous sclerosis, basal-cell nevus syndrome, Gardner's syndrome, and Li-Fraumeni syndrome. 4 ||
 * Signs & Symptoms: || <span style="color: #ff00ff; font-family: 'Times New Roman',Times,serif; font-size: 110%;">Generally, present as a painless lump that develops over a few weeks to months. <span style="color: #ff00ff; font-family: 'Times New Roman',Times,serif; vertical-align: super;">1
 * .Diagnostic Procedures: || <span style="color: #ff00ff; font-family: 'Times New Roman',Times,serif; font-size: 110%;">A detailed family history and therapeutic irradiation history is needed.
 * Histology: || <span style="color: #ff00ff; font-family: 'Times New Roman',Times,serif; font-size: 110%;">Most common Soft Tissue Sarcoma is Malignant Fibrous Histiocytoma (MFH) (28%). Other types include: leiomyosarcoma (12%), liposarcoma (15%), synovial sarcoma (10%) and malignant peripheral nerve sheath tumors. Rhabdomyosarcoma is the most common type of pediatric STS. <span style="color: #ff00ff; font-family: 'Times New Roman',Times,serif; font-size: 88%; vertical-align: super;">5 <span style="color: #ff00ff; font-family: 'Times New Roman',Times,serif; font-size: 110%;"> Over 100 types of soft tissue sarcoma described by the World Health Organization. <span style="color: #ff00ff; font-family: 'Times New Roman',Times,serif; font-size: 88%; vertical-align: super;"> 1 ||
 * Lymph Node Drainage: || <span style="color: #ff0000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">Lymphatic extension is not common. 3 ||
 * Metastatic Spread: || <span style="color: #ff0000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">- Invasion proceeds to adjacent muscle, skin, nerves, and bone.
 * Grading: || <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">[[image:softtissuesarcomagrading.jpg]]
 * Staging: || [[image:TNMstagingsofttissuesarcoma.jpg]]
 * Radiation Side Effects: || <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">Short term:
 * <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">The most common short term side effect seen is moist desquamation. This is most common when the beams are tangent to the skin and when more than 50% of the diameter of the extremity is included in the field. Another instance when moist desquamation is more common is when the patient is receiving concurrent doxorubicin.
 * <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">"Patients undergoing treatment for truncal tumors may experience nausea or thrombocytopenia."
 * <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">Approximately 10% of patients experience major wound complications after surgical resection. This may be with or without post-op irradiation. The rate increases to approximately 15% when the patient receives pre-op irradiation or brachtherapy within 5 days after their surgery.
 * <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">Long-term side effects that arise after conservative surgery and irradiation have the capability of significantly limiting the function of the preserved limb. "These sequalae include decreased range of motion and muscle strength, contracture of the joint, edema, pain, and bone fracture. Complications can be reduced b sparing a strip on normal tissue and uninvolved muscle to allow lymphatic drainage from the extremity." Physical therapy plays a very important role in helping to minimize the disabilities that arise. "Mobility of the extremity should be stressed, and patients should be placed on an exercise and range-of-motion program early in the course of therapy."
 * <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">For skin grafts, high-dose radiation doesn't appear to compromise their viability as long as proper time is allowed for healing (at least 3 weeks).
 * <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">Gonadal shields are used to reduce the dose to the testicles during treatment of lower extremity sarcomas in order to preserve fertility. <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 88%; vertical-align: super;">1 ||
 * Prognosis: || <span style="color: #008080; font-family: 'Times New Roman',Times,serif; font-size: 110%;">The most important prognostic factor in overall and diesease-free survival is the tumor grade. 1 ||
 * Treatments: || <span style="font-family: 'Times New Roman',Times,serif; font-size: 110%;"> Surgery is considered the primary treatment for soft tissue sarcomas (STS). 2 Low grade and small localized lesions with clear surgical margins can be treated with surgery alone. STS arising in areas other than extremities such as head and neck, trunk, abdomen and retroperitoneum are more challenging to manage. Surgical resection remains very important because these sites may not be amendable to curative doses of radiation therapy.

<span style="color: #0000ff; font-family: 'Times New Roman',Times,serif; font-size: 110%;">- Intraoperative electron beam radiation is another adjunctive modality for select patients. <span style="color: #0000ff; font-family: 'Times New Roman',Times,serif; font-size: 110%; vertical-align: super;">3 <span style="color: #0000ff; font-family: 'Times New Roman',Times,serif; font-size: 110%;"> This option usually delivers a dose between 1000 and 2000cGy with electron beam energies from 9-15 MeV.

Examples of sarcoma treatment fields: <span style="font-family: 'Times New Roman',Times,serif; font-size: 110%;"> Figure 1.3 Sim film of a thigh sarcoma .7 <span style="font-family: 'Times New Roman',Times,serif; font-size: 110%;"> Figure 1.4 Dose planning for a knee sarcoma. 7 <span style="font-family: 'Times New Roman',Times,serif; font-size: 110%;"> Figure 1.5 Forearm and hand treatment field photos. 3 || <span style="color: #0000ff; font-family: 'Times New Roman',Times,serif; font-size: 110%;">Muscle(adult)-6000cGy Muscle(child)-2000-3000cGy Skin5500cGy Mature cartilage and bone6000cGy Growing cartilage and bone--1000cGy || References: <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 110%;">1. <span style="color: #008000; font-family: 'Times New Roman',Times,serif; font-size: 111%;">Chao KS, Perez CA, Brady LW. //Radiation Oncology Management Decisions. 2nd edition.// Philadelphia, PA: Lippincott Williams & Wilkins. 1999, 2002; 615-621, <span style="color: #008080; font-family: 'Times New Roman',Times,serif; font-size: 110%;">617, 615. 2. Rubin P. //Clinical Oncology A Multidisciplinary Approach for Physicians and Students. 8th Edition//. Philadelphia, PA: W.B. Saunders Company. 2001; 619-620. 3. Washington CM, Leaver D. //Principles and Practice of Radiation Therapy//. Second Edition. St. Louis, Missouri: Mosby Inc. 2004; 580-584.80, 581. <span style="color: #008080; font-family: 'Times New Roman',Times,serif; font-size: 110%;">4. Lenhard RE, Osteen RT, Gansler T. The American Cancer Society's Clinical Oncology. 1st edition. Atlanta, GA: The American Cancer Society, Inc. 2001: 611-612. <span style="color: #ff00ff; font-family: 'Times New Roman',Times,serif;">5. <span style="color: #ff00ff; font-family: 'Georgia','serif';">Washington CM, Leaver DT. //<span style="font-family: 'Georgia','serif';">Principles and Practice of Radiation Therapy Practical Applications. // 3rd edition. St. Louis, Missouri: Mosby. 1997: 597. 6. Orthopedics Today web site. Available at http://www.orthosupersite.com. Accessed on Feb. 21, 2010. 7. UCLA Health System Radiation Oncology website available at http://radonc.ucla.edu/body.cfm?id=34. Accessed on Feb. 20, 2010.
 * TD5/5: || <span style="font-family: 'Times New Roman',Times,serif; font-size: 110%;"> **Organ -TD5/5** 3