Characteristics and Outcomes of Pediatric Vestibular Schwannomas.

Abstract

To review the demographics, treatment modalities, and survival of children with vestibular schwannomas. Analysis using the Surveillance, Epidemiology, and End Results (SEER) database. Pediatric patients from birth to 18 years in the SEER database were included from 2004 to 2014 based on a diagnosis of vestibular schwannoma using the primary site International Classification of Diseases (ICD) O-3 code of C72.4: acoustic nerve and the ICD O-3 histology codes of 9540/1: neurofibromatosis, Not Otherwise Specified (NOS); 9560/0: neurilemoma, NOS; or 9570/0: neuroma, NOS. One hundred forty-eight pediatric vestibular schwannomas (VSs) cases were identified. The mean age at diagnosis was 13.9 years (range, 4.0-18.0). Eighty-five (57.4%) patients were women. Seventy-seven (52.0%) patients had isolated unilateral VSs while 71 (48.0%) patients had either bilateral VSs or unilateral VSs with other brain, spinal cord, or cranial nerve tumors. Eighty two (55.4%) patients received surgical resection only, 45 (30.4%) received no treatment, 6 (4.1%) received radiation only, and 12 (8.1%) received surgery and radiation. The median tumor size for patients who received no treatment was 9.5 mm (interquartile range [IQR]: 8.0) compared with 33.5 mm (IQR: 23.0) for patients who received surgical care and 41.0 mm (IQR: 1.5) for patients who received both surgery and radiation (p < 0.001). The 5-year overall survival rate was 97%. Pediatric VSs tend to be diagnosed in adolescence. No men or women predominance was appreciated. Treatment varied according to tumor size. Survival rates for children with vestibular schwannomas are excellent. These data may assist healthcare providers when counseling children with vestibular schwannomas and their families.