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.
Highlights
Intracranial chordomas are rare tumours arising extraduraly in the clivus and midline structures of the skull base and the yearly incidence is no more than 0.1/1000.000 [35]
Twenty-one patients in this study were initially treated with microsurgical resection, while in two patients, transsphenoidal biopsy was performed as an initial diagnostic procedure, followed, by GTR in one patient
One patient experienced an aggravation of previous respiratory failure necessitating intensive care, while another experienced a postoperative haemorrhage requiring surgical intervention and intensive care treatment
Summary
Intracranial chordomas are rare tumours arising extraduraly in the clivus and midline structures of the skull base and the yearly incidence is no more than 0.1/1000.000 [35]. Histopathological features of the tumours indicate remnants of the notochord as the origin [46]. Recent gene expression studies analysing cytokeratins and brachyury proteins further strengthen this theory [52, 60]. Considered low-grade malignancies, these tumours are locally aggressive with invasive growth within mesenchymal-derived tissues and a high risk of eventual demise. Microsurgery, with the aim of gross total (GTR) or subtotal (STR) resection, followed by radiotherapy is considered the standard treatment for clivus chordomas [19, 22, 42, 49, 51]. Most chordoma patients are treated with transsphenoidal
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