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Connective Tissue Oncology Society

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Posters— Surgical Treatment of Sarcomas

CHORDOMA: NATURAL HISTORY AND TREATMENT RESULTS IN 55 PATIENTS

Gronchi A, Azzarelli A, Baratti D, Lozza L, Bertulli R, Casali PG, Pennacchioli E, Rasponi A, Dileo P, Pilotti S (Istituto Nazionale Tumori, Milan, Italy)


Intervention: Chordoma is a rare neoplasm with poor prognosis as already observed by several authors. Our experience reports one of the largest series in the litterature and confirms the need of new adjuvant treatments.

Patients: We observed at our institution 55 patients affected by chordoma. The neoplasm was located in the sacrum in 49 cases, at the base of the skull in 3 and in the vertebral bodies in 3. The median age at the time of the diagnosis was 58 (range 2-77); 34 patients were male and 21 female; median diameter was 13 cm. (range 2-30). During the seventies advances in preoperative staging, more reliable surgical techniques and a clear definition of oncological adequacy were the basis of a new rationale treatment of sacral chordoma. Since 1977 patients underwent surgery with intent of radicality. The technique of operation performed in 29 pts. was high amputation of the sacrum by posterior approach, with the aim to perform an uncontaminated removal of the entire lesion and surrounding normal tissue. Radiotherapy was considered in case of marginal or intralesional operations.

Results: Surgical margins were rated as wide in 9 patients, marginal in 17 and macroscopically intralesional in 3. Postoperative radiation therapy was performed in 9 patients with marginal/intralesional surgery; one patient underwent preoperative RT. Sacral chordoma recurred in 17 out of these 29 recent operated patients. Local failure, mainly located in the ischiorectal area, developed in 13 cases, distant isolated metastases (lung) in one patient and both local and distant failure in 3, with involvement of lung in 2 cases and distant subcutaneous dissemination in 1. As regard to resection margins definition, local + distant failure developed in 6 out 9 (66.7%) patients with wide margins and in 11 out of 17 (64.7%) patients with marginal operation.

Conclusions: Although chordoma is considered a low grade lesion, surgical treatment to obtain effective local control is difficult, due to late diagnosis and anatomical site. In the present study wide surgical margins were obtained only in 31% of the operated patients and recurrence rate was high. Distant metastases, isolated or concurrent to local failure, were detected only in 4 out of 29 patients (13.7%). Therefore strong effort in achieving coplete surgical excision is mandatory, as well as in developing more effective adjuvant therapies.


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