Surgical Resection and Adjuvant Radiation Therapy in the Treatment of Skull Base Chordomas.

Chordomas are rare tumors of notochordal origin that are known to be locally aggressive and are often treated with surgical resection followed by adjuvant radiation therapy (RT). The accepted standard of treatment for chordomas of the mobile spine, which includes en-bloc resection with wide margins, cannot be easily applied to the chordomas of the skull base because of their proximity to critical neurovascular structures. We describe our experience with the role of surgery and adjuvant RT in the treatment of chordomas over 16 years. We performed a retrospective chart review on patients with diagnoses of clival chordoma between the years 2000 and 2015 at Northwestern Memorial Hospital. We reviewed presenting symptoms, tumor location and size, extent of resection, complications, recurrence, adjuvant treatment, and follow-up duration. A total of 20 patients underwent 32 surgeries. Of the 20 initial surgeries, 80% underwent gross total resection, and 20% had subtotal resection. The mean follow-up time was 60.75 months. Mean tumor volume was 23.07 cm3. Most common presenting signs and symptoms were headaches (70%), cranial nerve palsies (45%), and diplopia (55%). Diplopia was defined as complaints of double vision without any objective evidence of a cranial nerve palsy. Median time to progression was 57 months, and median overall survival was 136 months. Initial tumor volume and the need for a second dose of RT either as sole or as adjuvant treatment of a recurrence had a statistically significant effect on progression-free survival (P = 0.009, 0.009). None of the factors studied had a statistically significant effect on overall survival. The treatment of chordomas remain challenging and requires multimodal treatment strategies spanning different specialties. Initial tumor size and need for second dose of RT for recurrence appear to play a significant role in progression-free survival. Adjuvant RT after gross total resection may play a role in improved progression-free and overall survival in patients with clival chordomas.

Sanusi O ，Arnaout O ，Rahme RJ ，Horbinski C ，Chandler JP ... -  《-》

Endoscopic Approach to Clival Chordomas: The Northwestern Experience.

Chordomas are rare primary bone tumors with a low-grade histology but an aggressive clinical behavior characterized by local invasion and recurrence. When occurring in the skull base, their treatment is limited by proximity to critical neurovascular structures. Open surgical approaches can carry high morbidity, making the development of alternative approaches desirable. We describe our experience with endoscopic endonasal approaches to clival chordomas over 13 years. We performed a retrospective chart review of patients diagnosed with clival chordomas and treated with an endoscopic endonasal approach between 2003 and 2015 at Northwestern Memorial Hospital. We reviewed presenting symptoms, tumor location and size, extent of resection, complications, recurrence, adjuvant treatment, retreatment, and follow-up duration. A total of 23 charts were reviewed, with 17 included in our final review. Mean age was approximately 48 years. The most common presenting symptom was diplopia present in 70.6% of patients. Mean tumor volume was 20.2 cm3. Gross total resection was achieved in 52.9% of patients. With a mean follow-up period of 63.4 months, 5 patients had a recurrence. The most common complication was cerebrospinal fluid (CSF) leak, which was noted in 6 patients. Tumor volume was significantly higher in patients with CSF leak. No correlation between intradural extension and postoperative CSF leak was noted. Clival chordoma are challenging entities to treat. The best outcomes are achieved with gross total resection followed by adjuvant radiotherapy treatment. We show that the endonasal endoscopic corridor is a viable alternative approach to these lesions.

Rahme RJ ，Arnaout OM ，Sanusi OR ，Kesavabhotla K ，Chandler JP ... -  《-》

Endoscopic resection of chordomas in different clival regions.

Hong Jiang W ，Ping Zhao S ，Hai Xie Z ，Zhang H ，Zhang J ，Yun Xiao J ... -  《-》

Long-term control of clival chordoma with initial aggressive surgical resection and gamma knife radiosurgery for recurrence.

Ito E ，Saito K ，Okada T ，Nagatani T ，Nagasaka T ... -  《-》

[Chordoma].

To review in the literature, all the epidemiological, clinical, radiological, histological and therapeutic data regarding chordomas as well as various notochordal entities: ecchordosis physaliphora, intradural and intraparenchymatous chordomas, benign notochordal cell tumors, parachordomas and extra-axial chordomas. To identify different types of chordomas, including familial forms, associations with tuberous sclerosis, Ollier's disease and Maffucci's syndrome, forms with metastasis and seeding. To assess the recent data regarding molecular biology and progress in targeted therapy. To compare the different types of radiotherapy, especially protontherapy and their therapeutic effects. To review the largest series of chordomas in their different localizations (skull base, sacrum and mobile spine) from the literature. The series of 136 chordomas treated and followed up over 20 years (1972-2012) in the department of neurosurgery at Lariboisière hospital is reviewed. It includes: 58 chordomas of the skull base, 47 of the craniocervical junction, 23 of the cervical spine and 8 from the lombosacral region. Similarly, 31 chordomas in children (less than 18 years of age), observed in the departments of neurosurgery of les Enfants-Malades and Lariboisière hospitals, are presented. They were observed between 1976 and 2010 and were located intracranially (n=22 including 13 with cervical extension), 4 at the craniocervical junction level and 5 in the cervical spine. In the entire Lariboisière series and in the different groups of localization, different parameters were analyzed: the delay of diagnosis, of follow-up, of occurrence of metastasis, recurrence and death, the number of primary patients and patients referred to us after progression or recurrence and the number of deaths, recurrences and metastases. The influence of the quality of resection (total, subtotal and partial) on the prognosis is also presented. Kaplan-Meier actuarial curves of overall survival and disease free survival were performed in the entire series, including the different groups of localization based on the following 4 parameters: age, primary and secondary patients, quality of resection and protontherapy. In the pediatric series, a similar analysis was carried-out but was limited by the small number of patients in the subgroups. In the Lariboisière series, the mean delay of diagnosis is 10 months and the mean follow-up is 80 months in each group. The delay before recurrence, metastasis and death is always better for the skull base chordomas and worse for those of the craniocervical junction, which have similar results to those of the cervical spine. Similar figures were observed as regards the number of deaths, metastases and recurrences. Quality of resection is the major factor of prognosis with 20.5 % of deaths and 28 % of recurrences after total resection as compared to 52.5 % and 47.5 % after subtotal resection. This is still more obvious in the group of skull base chordomas. Adding protontherapy to a total resection can still improve the results but there is no change after subtotal resection. The actuarial curve of overall survival shows a clear cut in the slope with some chordomas having a fast evolution towards recurrence and death in less than 4 years and others having a long survival of sometimes more than 20 years. Also, age has no influence on the prognosis. In primary patients, disease free survival is better than in secondary patients but not in overall survival. Protontherapy only improves the overall survival in the entire series and in the skull base group. Total resection improves both the overall and disease free survival in each group. Finally, the adjunct of protontherapy after total resection is clearly demonstrated. In the pediatric series, the median follow-up is 5.7 years. Overall survival and disease free survival are respectively 63 % and 54.3 %. Factors of prognosis are the histological type (atypical forms), localization (worse for the cervical spine and better for the clivus) and again it will depend on the quality of resection. Many different pathologies derived from the notochord can be observed: some are remnants, some may be precursors of chordomas and some have similar features but are probably not genuine chordomas. To-day, immuno-histological studies should permit to differentiate them from real chordomas. Improving knowledge of molecular biology raises hopes for complementary treatments but to date the quality of surgical resection is still the main factor of prognosis. Complementary protontherapy seems useful, especially in skull base chordomas, which have better overall results than those of the craniocervical junction and of the cervical spine. However, we are still lacking an intrinsic marker of evolution to differentiate the slow growing chordomas with an indolent evolution from aggressive types leading rapidly to recurrence and death on which more aggressive treatments should be applied.

George B ，Bresson D ，Bouazza S ，Froelich S ，Mandonnet E ，Hamdi S ，Orabi M ，Polivka M ，Cazorla A ，Adle-Biassette H ，Guichard JP ，Duet M ，Gayat E ，Vallée F ，Canova CH ，Riet F ，Bolle S ，Calugaru V ，Dendale R ，Mazeron JJ ，Feuvret L ，Boissier E ，Vignot S ，Puget S ，Sainte-Rose C ，Beccaria K ... -  《-》