gms | German Medical Science

Objective: Clival and craniocervical chordomas are slow growing but clinically malignant neoplasms. The proximity of these tumors to vital structures may allow a gross total resection but virtually never a free margin. Therefore, we hypothesized, that patients would benefit from adjuvant radiation therapy, preferentially protons or ions, even if gross total resection was achieved. A small series is presented in which this clinical practice was implemented.

Methods: Between 2006 and 2016, 15 patients (10 female/5 male, median age 52y, range 19/79) with clivul and craniocervical chordomas underwent 25 resections. 9 of these patients presented with double vision due to abducens or oculomotor palsy, 1 with deterioration of visual acuity and field as well as pituitary insufficiency, 1 with nasal obstruction, 2 with unilateral palsy of the caudal cranial nerves and craniocervical instability, 1 with vertigo, 1 incidental. 3 patients were biopsied before. The maximal tumor diameter ranged from 14 to 72 mm. A pure endoscopic transnasal approach was primarily applied, in 1 patient an anterior cervical approach was added. 7 patients received proton beam, 4 heavy ions, 2 photon, 1 none, 1 scheduled for protons.

Results: Gross total resection was achieved in 13 and subtotal resection in 2 cases during primary surgery. 8/8 abducens and 1/1 oculomotor palsies resolved, visual acuity and field improved and pituitary insufficiency remained, breathing improved, caudal cranial nerve palsies remained unchanged, neck pain resolved after dorsal craniocervical fusion. In 1 patient, aggravation from caudal CN palsy to plegia was encountered. One patient complains prolonged nasal discomfort with crusting. 1 patient died due to mayor head trauma after second resection of a regrowth and second radiation. The remaining are progression free after a median follow-up of 56 months, range 2-121. 2 patients presented with irradiation induced necrosis of the mesial temporal lobe, 1 was surgically excised. 1 patient developed a probably radiation induced sarcoma of the condyle/petrous bone and died 6 months after its diagnosis.

Conclusion: The low mortality rate of 2/15 after a median follow-up of 52 months allows the recommendation for radiation following radical surgery. But all effective therapy has morbidity, 1 surgically induced caudal CN palsy, and radiation induced 1 sarcoma and 2 temporal necrosis.