Long-term quality of life outcomes in patients undergoing microsurgical resection of vestibular schwannoma.

Background: While previous studies have assessed patient reported quality of life (QOL) of various vestibular schwannoma (VS) treatment modalities, few studies have assessed QOL as related to the amount of residual tumor and need for retreatment in a large series of patients. Objective: To assess patient reported QOL outcomes following VS resection with a focus on extent of resection and retreatment. Methods: A retrospective chart review was performed using single-center institutional data of adult patients who underwent VS resection by the senior authors between 1989-2018 at Loyola University Medical Center. The Penn Acoustic Neuroma Quality of Life (PANQOL) survey was sent to all patients via postal mail. Results: Fifty-five percent of 367 total patients were female with a mean age of 61.6 years (SD 12.63). The mean period between surgery and PANQOL response was 11.4 years (IQR: 4.74-7.37). The median tumor size was 2 cm (IQR: 1.5-2.8). The mean total PANQOL score was 70 (SD 19). Patients who required retreatment reported lower overall scores (µdiff = -10.11, 95% CI: -19.48 to -0.74; p = 0.03) and face domain scores (µdiff = -20.34, 95% CI: -29.78 to -10.91; p < .001). There was no association between extent of resection and PANQOL scores in any domain. Conclusion: In an analysis of 367 patients who underwent microsurgical resection of VS, extent of resection did not affect PANQOL scores in contrast to previous reports in the literature, while the need for retreatment and facial function had a significant impact on patient-reported outcomes.

Is There a Plateau to the Learning Curve for Acoustic Neuroma Resection?-Experience and Outcomes from a Single Interdisciplinary Team Over Thirty Years.

Objective The evolution of acoustic neuroma (AN) care continues to shift focus on balancing optimized tumor resection and control with preservation of neurological function. Prior learning curve analyses of AN resection have demonstrated a plateau between 20 and 100 surgeries. In this study of 860 consecutive AN surgeries, we investigate the presence of an extended learning curve tail for AN resection. Methods A retrospective cohort study of AN resections by a single interdisciplinary team between 1988 and 2018 was performed. Proportional odds models and restricted cubic splines were used to determine the association between the timing of surgery and odds of improved postoperative outcomes. Results The likelihood of improved postoperative House-Brackmann (HB) scores increased in the first 400 procedures, with HB 1 at 36% in 1988 compared with 79% in 2004. While the probability of a better HB score increased over time, there was a temporary decrease in slope of the cubic spline between 2005 and 2009. The last 400 cases continued to see improvement in optimal HB outcomes: adjusted odds of HB 1 score were twofold higher in both 2005 to 2009 (adjusted odds ratio [aOR]: 2.11, 95% confidence interval [CI]: 1.38-3.22, p < 0.001) and 2010 to 2018 (aOR: 2.18, 95% CI: 1.49-3.19, p < 0.001). Conclusion In contrast to prior studies, our study demonstrates the steepest growth for learning, as measured by rates of preservation of facial function outcomes (HB 1), occurs in the first 400 AN resections. Additionally, improvements in patient outcomes continued even 30 years into practice, underlining the importance of lifelong learning.

Occipital Neuralgia following Acoustic Neuroma Resection.

Background While postoperative outcomes of acoustic neuroma (AN) resection commonly consider hearing preservation and facial function, headache is a critical quality of life factor. Postoperative headache is described in the literature; however, there is limited discussion specific to occipital neuralgia (ON) following AN resection. Objective The aim of this study is to investigate the effectiveness of conservative management and surgery. Methods We conducted a retrospective review of 872 AN patients who underwent resection at our institution between 1988 and 2017 and identified 15 patients (1.9%) that met International Classification of Headache Disorders criteria for ON. Results Of the 15 ON patients, surgical approaches included 13 (87%) retrosigmoid (RS), one (7%) translabyrinthine (TL), and one (7%) combined RS + TL. Mean clinical follow-up was 119 months (11-263). Six (40%) patients obtained pain relief through conservative management, while the remaining nine (60%) underwent surgery or ablative procedure. Three (38%) patients received an external neurolysis, four (50%) received a neurectomy, one (13%) had both procedures, and one (13%) received two C2 to 3 radio frequency ablations. Of the nine patients who underwent procedural ON treatment, seven (78%) patients achieved pain relief, one patient (11%) continued to have pain, and one patient (11%) was lost to follow-up. Of the six patients whose pain was controlled with conservative management and nerve blocks, five (83%) found relief by using neuropathic pain medication and one (17%) found relief on nonsteroidal anti-inflammatory drug. Conclusion Our series demonstrates success with conservative management in some, but overall a minority (40%) of patients, reserving decompression only for refractory cases.

Pediatric vestibular schwannomas: case series and a systematic review with meta-analysis.

OBJECTIVE: Sporadic unilateral vestibular schwannomas are rare in the pediatric population. Little has been reported in the literature on the presentation, tumor size, response to surgical treatment, and recurrence rates in these younger patients. The authors' goal was to describe their institutional experience with pediatric sporadic vestibular schwannomas and to conduct a meta-analysis of the existing literature to provide further insight into the presentation, tumor characteristics, and surgical outcomes for these rare tumors to help direct future treatment strategies. METHODS: The authors performed a retrospective review of all patients 21 years of age or younger with unilateral vestibular schwannomas and without neurofibromatosis type 2 who underwent resection by the senior authors between 1997 and 2019. A systematic review of the literature and meta-analysis was also performed by entering the search terms "pediatric" and "vestibular schwannoma" or "acoustic neuroma," as well as "sporadic" into PubMed. Presentation, treatment, clinical outcomes, and follow-up were analyzed. RESULTS: Fifteen patients were identified at the authors' institution, ranging in age from 12 to 21 years (mean 16.5 years). Common presenting symptoms included hearing loss (87%), headache (40%), vertigo (33%), ataxia (33%), and tinnitus (33%). At the time of surgery, the mean tumor size was 3.4 cm, with four 1-cm tumors. Four patients had residual tumor following their first surgery, 3 (75%) of whom had significant radiographic regrowth that required further treatment. The literature review identified an additional 81 patients from 26 studies with patient-specific clinical data available for analysis. This resulted in a total of 96 reported patients with an overall average age at diagnosis of 12.1 years (range 6-21 years) and an average tumor size of 4.1 cm. CONCLUSIONS: Pediatric vestibular schwannomas present similarly to those in adults, although symptoms of mass effect are more common, as these tumors tend to be larger at diagnosis. Some children are found to have small tumors and can be successfully treated surgically. Residual tumors in pediatric patients were found to have a higher rate of regrowth than those in their adult counterparts.

Caudal Homeobox Gene-2 Staining Defines Intracranial Mature Teratoma with Differentiation to Colonic Adenocarcinoma.

BACKGROUND: Malignant transformation of a mature intracranial teratoma into an adenocarcinoma is an extremely rare event and portends a poor prognosis. The clinical progression, radiographic changes, histopathological findings, and immunohistochemistry findings from these unique cases might provide clues regarding this transformation. Caudal homeobox gene-2 (CDX-2) is a specific and robust marker for colonic adenocarcinomas and can also be used to identify differentiation of mature intracranial teratoma into colonic-type adenocarcinoma. To the best of our knowledge, this is the first case report of the use of this specific marker for an intracranial malignancy. CASE DESCRIPTION: We present the case of a 55-year-old right-handed Hispanic-American woman with progressive headaches who was found to have a left posterior parietotemporal tumor with intraventricular extension. Surgical debulking was performed, and the pathologic examination revealed a mature teratoma. Despite surgical resection and radiotherapy, the teratoma progressed to a malignant mature intracranial teratoma both radiographically and histologically. Histological analysis of the third specimen revealed a moderately differentiated adenocarcinoma. The tumor cells were positive for CDX-2 and cytokeratin 20 and negative for cytokeratin 7 and thyroid transcription factor-1, consistent with an enteric/colonic-type adenocarcinoma, demonstrating progressive atypia and malignancy. CONCLUSIONS: Malignant transformation of a mature intracranial teratoma portends a poor prognosis. The exact histopathological diagnosis can facilitate treatment of these patients. CDX-2 is a specific robust marker for identifying differentiation of a mature intracranial teratoma into a colonic adenocarcinoma. This positive staining can also be observed in primary colonic and other adenocarcinomas. To the best of our knowledge, this is the first report of the use of CDX-2 in the diagnosis of an intracranial malignancy. The triangulation of clinical progression, radiographic findings, and histopathological and immunohistochemistry findings provided clues regarding this unique transformation.