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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