Faculty Bibliography

OBJECTIVES/HYPOTHESIS:: Medulloblastoma is the most common pediatric malignant tumor of the central nervous system in children. Treatment includes surgical excision, external beam radiation, and multiagent chemotherapy. Otologic sequelae are common and may result from radiation and/or chemotherapy. Profound sensorineural hearing loss (SNHL) is a known complication of neuro-oncologic treatment and may render these patients eligible for cochlear implantation (CI). Issues of CI in this population, including diagnosis, treatment of preoperative middle ear disease, operative and postoperative course, performance data, and long-term tumor surveillance are highlighted and reviewed. STUDY DESIGN:: Retrospective chart review. METHODS:: Three patients treated for pediatric medulloblastoma with surgical resection, postoperative hyperfractioned craniospinal radiotherapy, and multiagent adjuvant chemotherapy who underwent cochlear implantation were identified. Details of neuro-oncologic treatment and associated otologic complications are presented and analyzed. Primary outcome assessment includes treatment of middle ear pathology, perioperative cochlear implant course, and postimplantation performance data. RESULTS:: Each patient required surgical treatment of chronic ear disease 4 to 16 years after chemoradiation. All progressed to profound SNHL and were implanted 8 to 17 years post-neuro-oncologic treatment. There were no intraoperative complications, and full insertion of the cochlear implant electrode array was achieved in each patient. One patient developed postoperative wound dehiscence requiring operative closure. Postimplantation performance data support significant benefit in all patients. CONCLUSIONS:: Patients treated for pediatric medulloblastoma develop otologic sequelae, including profound SNHL, and may require cochlear implantation. Successful management of middle ear and mastoid pathology involves consideration of potential future cochlear implantation. Postoperative performance data supports cochlear implantation in this population. Laryngoscope, 2009

INTRODUCTION: ACNS 0122 aimed to improve event-free survival (EFS) and OS (overall survival) for intracranial NGGCT, by increasing response rate (complete [CR] and partial [PR]) with neoadjuvant carboplatin/VP-16, alternating with ifosfamide/VP-16, followed by craniospinal irradiation (CSI) plus involved field boost. In patients not obtaining CR/PR by neuro-imaging and tumor marker response after neoadjuvant chemotherapy, second-look surgery was recommended. Patients with persistent radiographic disease or positive markers underwent myeloablative chemotherapy (thiotepa/VP-16) prior to CSI. OBJECTIVES: 1) To determine response rate following three cycles of neoadjuvant chemotherapy; 2) to determine EFS and OS; and 3) To determine whether additional CR can be achieved with high-dose thiotepa/VP-16 for patients not achieving CR/PR. RESULTS: 104 patients enrolled from 1/04-7/08. Median age was 12 (range 3-23) years. 76% were male,. No toxic deaths occurred. Among 84 evaluable patients, response rate was reported as 70% (33 CR, 26 PR). With central imaging review (58/84 patients reviewed to date) response rate was 90%. Nineteen patients underwent second-look surgery, 2 secondary to progression; reviewed pathology in 11 was malignant teratoma or mature teratoma (8), fibrosis (1), and NGGCT (2). Median follow-up for patients without events is 1.9 years (range 0.06-4.9). Fifteen patients have experienced recurrence or progression to date with 6 subsequent deaths. Two-year EFS and OS are 84.4%+/-4% and 93% +/-3%, respectively. Further stratification of responses will be presented. CONCLUSION: Neoadjuvant chemotherapy for NGGCT demonstrates a very high response rate and when administered before CSI may increase survival