Surgery With or Without Radiation Therapy

In a retrospective study of 20 children with skull-based chordomas, the median age at diagnosis was 12 years. The most common presenting symptoms were diplopia, headache, and swallowing difficulties.[11] Five patients had locally recurrent tumors. Twelve patients underwent surgery with an endoscopic endonasal approach alone, and eight patients underwent other procedures. All but two patients received radiation therapy. Fourteen patients had gross-total resections, ten of whom developed surgical complications.

In a retrospective study of 20 children with skull-based chordomas, the median age at diagnosis was 12 years. The most common presenting symptoms were diplopia, headache, and swallowing difficulties.[11] Five patients had locally recurrent tumors. Twelve patients underwent surgery with an endoscopic endonasal approach alone, and eight patients underwent other procedures. All but two patients received radiation therapy. Fourteen patients had gross-total resections, ten of whom developed surgical complications.

No differences in recurrence rates were seen between patients who presented with a new diagnosis and patients who had recurrent disease or between patients who underwent a gross-total resection and patients who underwent a near-total resection.

In a retrospective study of 20 children with skull-based chordomas, the median age at diagnosis was 12 years. The most common presenting symptoms were diplopia, headache, and swallowing difficulties.[11] Five patients had locally recurrent tumors. Twelve patients underwent surgery with an endoscopic endonasal approach alone, and eight patients underwent other procedures. All but two patients received radiation therapy. Fourteen patients had gross-total resections, ten of whom developed surgical complications.

Of patients who received postoperative radiation therapy, none had a recurrence.

In a retrospective study of 20 children with skull-based chordomas, the median age at diagnosis was 12 years. The most common presenting symptoms were diplopia, headache, and swallowing difficulties.[11] Five patients had locally recurrent tumors. Twelve patients underwent surgery with an endoscopic endonasal approach alone, and eight patients underwent other procedures. All but two patients received radiation therapy. Fourteen patients had gross-total resections, ten of whom developed surgical complications.

Comparatively, of the 11 patients who either did not receive radiation therapy or were treated preoperatively, 4 had a recurrence (P = .09).

In a retrospective study of 20 children with skull-based chordomas, the median age at diagnosis was 12 years. The most common presenting symptoms were diplopia, headache, and swallowing difficulties.[11] Five patients had locally recurrent tumors. Twelve patients underwent surgery with an endoscopic endonasal approach alone, and eight patients underwent other procedures. All but two patients received radiation therapy. Fourteen patients had gross-total resections, ten of whom developed surgical complications.

Three patients developed distant metastases, and three patients died of disease.

In a retrospective study of 20 children with skull-based chordomas, the median age at diagnosis was 12 years. The most common presenting symptoms were diplopia, headache, and swallowing difficulties.[11] Five patients had locally recurrent tumors. Twelve patients underwent surgery with an endoscopic endonasal approach alone, and eight patients underwent other procedures. All but two patients received radiation therapy. Fourteen patients had gross-total resections, ten of whom developed surgical complications.

A high Ki-67 index was more prevalent among patients with dedifferentiated chordomas. Two of the three patients who died had an elevated Ki-67 index.

Pediatric patients with base-of-skull chordomas were treated with proton-beam therapy or a combined proton/photon approach (proton-based; most received 80% proton/20% photon) at the Massachusetts General Hospital from 1981 to 2021. Of 204 patients, the median age at diagnosis was 11.1 years (range, 1–21 years).[12]

Pediatric patients with base-of-skull chordomas were treated with proton-beam therapy or a combined proton/photon approach (proton-based; most received 80% proton/20% photon) at the Massachusetts General Hospital from 1981 to 2021. Of 204 patients, the median age at diagnosis was 11.1 years (range, 1–21 years).[12]

The chordomas presented in the upper and/or middle clivus in 59% of the patients, lower clivus in 36%, craniocervical junction in 4%, and nasal cavity in 1%.

Pediatric patients with base-of-skull chordomas were treated with proton-beam therapy or a combined proton/photon approach (proton-based; most received 80% proton/20% photon) at the Massachusetts General Hospital from 1981 to 2021. Of 204 patients, the median age at diagnosis was 11.1 years (range, 1–21 years).[12]

The median overall survival (OS) was 26 years, and the median progression-free survival (PFS) was 25 years. The 5-, 10-, and 20-year OS rates were 84%, 78% and 64%, respectively. The 5-, 10-, and 20-year PFS rates were 74%, 69%, and 64%, respectively.

Pediatric patients with base-of-skull chordomas were treated with proton-beam therapy or a combined proton/photon approach (proton-based; most received 80% proton/20% photon) at the Massachusetts General Hospital from 1981 to 2021. Of 204 patients, the median age at diagnosis was 11.1 years (range, 1–21 years).[12]

In multivariable actuarial analysis, prognostic factors associated with worse OS included poorly differentiated subtype, radiographical progression prior to radiation therapy, larger treatment volume, and lower clivus location.