Multidisciplinary management of clival chordomas; long-term clinical outcome in a single-institution consecutive series

Abstract

ObjectiveChordomas of the skull base have high recurrence rates even after radical resection and adjuvant radiotherapy. We evaluate the long-term clinical outcome using multidisciplinary management in the treatment of clival chordomas.MethodsBetween 1984 and 2015, 22 patients diagnosed with an intracranial chordoma were treated at the Karolinska University Hospital, Stockholm, Sweden. Sixteen of 22 were treated with Gamma Knife radiosurgery (GKRS) for tumour residual or progression during the disease course. Seven of 22 received adjuvant fractionated radiotherapy and 5 of these also received proton beam radiotherapy.ResultsFifteen of 22 (68%) patients were alive at follow-up after a median of 80 months (range 22–370 months) from the time of diagnosis. Six were considered disease free after >10-year follow-up. The median tumour volume at the time of GKRS was 4.7 cm3, range 0.8–24.3 cm3. Median prescription dose was 16 Gy, range 12–20 Gy to the 40–50% isodose curve. Five patients received a second treatment with GKRS while one received three treatments. After GKRS patients were followed with serial imaging for a median of 34 months (range 6–180 months). Four of 16 patients treated with GKRS were in need of a salvage microsurgical procedure compared to 5/7 treated with conventional or proton therapy.ConclusionAfter surgery, 7/22 patients received conventional and/or photon therapy, while 15/22 were treated with GKRS for tumour residual or followed with serial imaging with GKRS as needed upon tumour progression. With this multidisciplinary management, 5- and 10-year survivals of 82% and 50% were achieved, respectively.

Salvage Therapy for Local Progression following Definitive Therapy for Skull Base Chordomas: Is There a Role of Stereotactic Radiosurgery?

“…Most recently, Förander et al demonstrated the role of GKSRS in the management of residual disease after no previous radiation and management of progression after previous SRS. 11 With dosing parameters similar to that in our study, a tumor control rate of 50% at 15 years for first time GKSRS patients was noted in the setting of an ARE rate of 18%; notably, a majority of the recurrences were considered out of field. In the repeat SRS subcohort of six patients, a prominent effect on tumor control was also noted.…”

“…Interestingly, location of the treated recurrence relative to the prior radiation field did not limit delivery of an effective and safe radiation dose, with median maximum and marginal doses of 28 and 15 Gy, respectively; these dosing schemes are similar to those reported by other GKSRS series in the literature. [10][11][12][13][14][15] As discussed in the following, it is likely that the judicious use of surgery helped radiation outcomes for patients with infield/ marginal recurrences adjacent to the brainstem. The largest radiosurgery series to date reported outcomes for 71 patients, 20 of whom were treated for recurrence following prior radiation.…”

“…Other centers have reported high rates of local control using radiosurgery to treat local residual or recurrent disease. [10][11][12][13][14][15] However, these studies do not exclusively address the management of recurrent disease after a previous charged particle therapy. Most recently, Förander et al demonstrated the role of GKSRS in the management of residual disease after no previous radiation and management of progression after previous SRS.…”

Anesthetic Considerations in a Case With Massive Recurrent Clivus Chordoma

“…1 Treating chordomas can be challenging due to their proximity to various vital structures and their high recurrence rate, therefore, en bloc resection may not be possible, and microsurgery with the aim of gross total resection or subtotal resection followed by adjuvant therapy such as radiotherapy is considered to be the treatment of choice. 1,2,4 Most chordoma patients treated with the transsphenoidal approach. 8 However, more extensive tumors treated with the transbasal or transpetrosal approach.…”

“…8 However, more extensive tumors treated with the transbasal or transpetrosal approach. 2 Other approaches include trans mandibular, transcochlear, transcondylar, subtemporal-preauricular, and extreme lateral. 1 Chordomas are often resistant to conventional radiotherapy.…”