The approaches and outcomes of skull base surgery for pediatric sarcoma after initial therapy.

OBJECTIVE: Surgical removal of the residual pediatric sarcoma after initial therapy is common; however, that in the skull base area still presents a formidable challenge. We reviewed the approaches and outcomes of skull base surgery for pediatric sarcoma.
METHODS: Thirteen patients with pediatric sarcoma who received skull base surgery were reviewed retrospectively. Tumor sites, surgical approach, complications, regional recurrence after surgery, and survival rate were analyzed.
RESULTS: The residual tumor sites were the infratemporal fossa (8), nasal cavity and paranasal sinus (4) and orbit with anterior skull base invasion (1). Coronal skin incision (1), that with preauricular skin incision (7) and facial dismasking flap (5) were applied to patients as the skin preparation. Following skin preparations, anterior skull base surgery with frontal craniotomy (3), infratemporal fossa approach with temporal craniotomy (5), and anterior-lateral skull base with front-temporal craniotomy (1) were performed. Tumors were removed with a sufficient surgical field in all patients. Facial nerve palsy (9), facial deformity (4), orbital complications (diplopia, decreased visual acuity, narrowing of visual field) (2) and occlusal imbalance (1) occurred. However, facial palsy, diplopia and occlusal imbalance subsided gradually. Local recurrence occurred in 6 cases and distant metastasis was observed in 4 cases. The overall 5-years survival rate was 51.9% (Kaplan-Meier method).
CONCLUSION: These tumors were safely removed with minimum morbidity. Skull base surgery is recommended to remove residual pediatric sarcoma after the initial treatment.