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Treatment Guidelines and Outcome

Treatment of sarcomas depends on several factors related to either the tumor (histological subtype, grade of differentiation, stage and size) or the patient (age, performance status).

Rhabdomyosarcoma, due to the local aggressiveness and to the tendency to early metastasis, must be treated with a multimodality treatment. When only surgery with or without radiotherapy were available for the treatment of this neoplasm, 5-year survival rate was around 10% (Callender et al. 1995). The advent of chemotherapeutic protocols (including vincristine, adriamycin, cyclophosphamide, dactino-mycin) in association with radiotherapy and, when required, with surgery has dramatically improved the overall and disease-free survival of the disease. According to such therapeutic guidelines, for example, Callender et al. (1995) found a 5-year local-regional control and an overall survival of 88% and 60%, respectively. Another important observation from their study was that the control of distant spreading and 10-year disease-specific survival were significantly higher (72% vs. 24% and 82% vs. 36%, respectively) in patients who had received chemotherapy for at least 1 year when compared to patients who did not (Callender et al. 1995). The high risk for nodal metastases makes comprehensive radiotherapy of the neck mandatory. Indications for surgery are quite limited and include excision of small, easily resectable residual or recurrent lesions or palliative resections.

Presence of distant metastases, meningeal extension, adult age, alveolar or unclassified subtype, incomplete resection are considered negative prognostic factors (Callender et al. 1995; Sercarz et al. 1994).

Rhabdomyosarcoma is characterized by a high, e local, regional, and distant recurrence rate. This is the reason why a "systemic" approach to the disease is mandatory also during post-treatment follow-up. The occurrence of local relapse should be accurately ruled out either clinically and by MR. When the si-nonasal tract is considered, a relatively high risk of early relapse/recurrence along meningeal planes and then into the brain parenchyma is worth being underlined. Therefore, periodic MR evaluation (every three months for the first year and then every six months) should be considered mandatory, especially for those lesions encroaching or approximating the skull base. With the specific aim of detecting meningeal or cerebral parenchyma diffusion, PET scans do not show a satisfactory diagnostic accuracy, due to the high metabolic activity of the brain. Conversely, PET is essential in revealing the presence of systemic diffusion of the disease. Cervical node metastases, another frequent pattern of failure for rhabdomyosarcoma, may be detected by US of the neck.

Chondrosarcoma is amenable to surgical excision with a high success rate (Sercarz et al. 1994). The tendency to present in the well differentiated form (Grade I) explains why chondrosarcoma is the head and neck soft tissues malignant tumor with the best prognosis. Overall 5-year survival, combined for the different grades, may vary from 44% to 81% (Downey et al. 2001).

Surgery must ensure wide clear surgical margins. In presence of small lesions without skull base or orbital involvement, especially if located at the level of nasal septum, an endoscopic resection may be safely accomplished (Matthews et al. 2002). Radiotherapy or chemotherapy should be performed whenever positive margins, which are the most important prognostic factor together with grading, are detected. In fact, the rate of relapse in presence of positive margins is as high as 65% (Mark et al. 1993). Radio- and/ or chemotherapy may also be used in case of recurrences or with a palliative intent.

Chondrosarcoma does not usually display a very aggressive behavior and local recurrence is the most common cause of death. Nevertheless, 20% of patients, especially with a high-grade lesion or mesen-chymal subtype, develop distant metastases, being the lung the most affected site. The observation of late recurrences explains the need for a prolonged follow-up.

The treatment for osteosarcoma must be based upon wide, aggressive surgical excision, with special attention paid to achieve negative margins. Positive margins, in fact, are an indeed negative prognostic factor (Kassir et al. 1997; Patel et al. 2002a). Neoadjuvant chemotherapy should be administrated to patients with high-grade tumors or with lesions unlikely to be completely excised. Adjuvant radiotherapy should be reserved to those cases in which surgery was unable to ensure a complete resection (Patel et al. 2002a). Following these therapeutic criteria, 5-year overall, disease-specific, and recurrence-free survival of 70%, 75%, and 70%, may be observed (Patel et al. 2002a). These data are consistent with those obtained at the University of Washington (Oda et al. 1997).

The most common pattern of failure for osteo-sarcoma is local recurrence, which may be more frequent in patients with positive surgical margins or with tumors greater than 4 cm in size (Patel et al. 2002a). Distant recurrence is instead rare, with rates commonly ranging between 13% and 23% (van Es et al. 1997; Bennett et al. 2000; Patel et al. 2002a).

The aggressiveness of leiomyosarcoma requires a complete surgical excision followed by radiotherapy, in spite of limited radiosensitivity (Keck et al. 2001). In presence of small, easily resectable lesions, surgery alone may be considered curative (Kuruvilla et al. 1990). Radio- and chemotherapy should also be a valid option whenever palliation is required (Batra et al. 2001). According to Batra et al. (2001), leio-myosarcoma is characterized by a poor prognosis, with a 20% 5-year overall survival; recurrences may develop in up to 55% of patients. According to some other authors, the occurrence of the neoplasm within the paranasal sinuses rather than into the nasal cavity would be associated with a higher risk of recurrence (Kuruvilla et al. 1990). Local recurrence is the most common pattern of failure.

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