Functional Preservation After Planned Partial Resection Followed by Gamma Knife Radiosurgery for Large Vestibular Schwannomas.

OBJECTIVE: The treatment goal for vestibular schwannomas (VS) has been changed from total removal of the tumor to functional preservation with long-term tumor growth control. The small- to medium-sized VS can be treated by stereotactic radiosurgery, but large VS require surgical decompression for the relief of cerebellar dysfunction and increased intracranial pressure. We have been performing planned partial surgical resections followed by gamma knife radiosurgery (GKS) for large VS. Here, we evaluate a recent series of such cases from the standpoint of functional outcomes. METHODS: From January 2000 to May 2013, we treated 40 patients with large unilateral VS (maximum tumor diameter at least 25 mm) with planned partial tumor removal followed by GKS for functional preservation. The median maximum diameter of the tumors was 32.5 mm (range 25-52 mm). All patients underwent surgery via the retrosigmoid approach, and tumors situated on the ventral and in the internal auditory canal intentionally were not removed, thus preserving cranial nerve functions. GKS was performed 1-12 months after surgical resection (median interval 3 months). The median tumor volume at GKS was 3.3 cm(3) (range 0.4-10.4 cm(3)) and the median prescribed dose was 12 Gy (range 10-12 Gy). The median follow-up period after GKS was 65 months (18-156 months). RESULTS: At the final follow-up, facial nerve preservation (House-Brackmann grade I-II) was achieved in 38 patients (95%; House-Brackmann grade I: 92.5%, II: 2.5%). Among the 14 patients with preoperative pure tone average (PTA) less than 50 dB, 6 of them (42.9%) maintained PTA less than 50 dB at the last follow-up. Two patients improved from severe hearing loss to PTA less than 50 dB (1 patient after surgery and 1 patient one and half years after GKS). Five- and 10-year tumor growth control occurred in 86% of patients. Four patients (10%) required salvage surgery; the predictive factor was tumor volume greater than 6 cm(3) at GKS (P = 0.01). CONCLUSIONS: Planned partial removal of large VS followed by GKS achieved a high rate of facial nerve and hearing preservation. To achieve long-term tumor growth control, the tumor volume at GKS after planned partial surgical resection should be smaller than 6 cm(3). Our results revealed that patients with hearing preservation postoperatively have a chance of maintaining hearing function, even though the possibility exists of deterioration by long-term follow-up after surgical intervention and GKS. Furthermore, some patients with severe hearing loss before treatment have the chance of hearing improvement, even those with large VS.

[A case of a large neonatal pharyngeal teratoma].

The congenital teratoma is a rare malformation. We present herein on the case of a large teratoma that arose from the epipharynx in a neonate. The obstructive mass caused maternal polyhydramnios and was identified prenatally by ultrasonography at another hospital. The mother was transferred to our hospital for delivery. We explained about the ex utero intrapartum treatment (EXIT) because of the risk of airway obstruction at birth, but EXIT was refused by the parents. Delivery was accomplished by cesarean section at the 35 th week. Since the newborn had a severe respiratory disorder due to a large intraoral tumor and intratracheal intubation was very difficult, a tracheostomy was done under local anesthesia for 7 min. The tumor was removed successfully on the 9th day and 44th day of age.

[A case report on cervical neuroblastoma with ataxia].

We present a case of pediatric primary cervical neuroblastoma (NB), which is extremely rare. A 3-year-old girl with ataxia but no nasal obstruction, dysphagia, or stridor was diagnosed with cervical NB. Diagnostic results including clinical chemistry, full blood count, and serology, were normal. Ataxia worsened within a few days after onset and was not cured by steroids or intravenous immunoglobulin, delaying a definite diagnosis until a tumor was detected. Opsoclonus-myoclonus syndrome is typified by opsoclonus with myoclonus and ataxia, primarily associated with neuroblastoma. Brain stem cell and cerebellum dysfunction is thought to be due to an autoimmune mechanism. Following chemotherapy and selective neck dissection, the girl has had no recurrence or adverse sequelae. Ataxia disappeared during chemotherapy. We suggest that neuroblastoma should be considered in any child with unexpected by prolonged ataxia.