Management of vestibular schwannomas in young patients-comparison of clinical features and outcome with adult patients.
ABSTRACT Vestibular schwannomas (VS) in young patients are rare. They are regarded to have different biological characteristics. Our objective is to analyze a series of such patients, with respect to their clinical presentation, treatment, and outcome and to compare the results to a matched series of adult patients.
Retrospective analysis of 20 patients under 21 years of age. All patients underwent surgery via the retrosigmoid approach. The analysis included: age, gender, tumor size, clinical, and neurological pre- and postoperative status including cochlear and facial nerve function, and complications. Statistical comparison of the young patient's data with a series of 200 adult patients previously published by the authors.
The current study demonstrates that the outcome after surgical management in patients harboring VS does not show any significant difference between young and adult patients.
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ABSTRACT: Management options for patients with vestibular schwannoma include observation, surgical resection, stereotactic radiosurgery (SRS), and stereotactic radiation therapy. In younger patients, resection is often advocated because of concern regarding the long-term effects of radiation. We studied tumor response and clinical outcomes after SRS in such patients. We reviewed long-term outcomes in 55 patients with vestibular schwannomas. Patients were 40 years of age or younger, underwent gamma knife (GK) SRS between 1987 and 2003, and were followed up for a minimum of 4 years. The median patient age was 35 years (range, 13-40 years). Forty-one patients had Gardner-Robertson class 1 to 4 hearing. Thirteen patients (24%) had undergone surgical removal. The median tumor volume was 1.7 mm. The median tumor margin dose was 13.0 Gy (range, 11-20 Gy). At a median of 5.3 years, (range, 4-20 years), 2 of 55 patients underwent GK SRS for a second time; 1 of these patients had had a recurrence after initial resection. The 5-year rate of freedom from additional management was 96%. Hearing preservation rates (i.e., remaining within the same Gardner-Robertson hearing class) were 93%, 87%, and 87% at 3, 5, and 10 years, respectively. In patients with serviceable hearing before SRS, it was maintained in 100%, 93%, and 93% of patients at 3, 5, and 10 years, respectively. Hearing preservation was related to a margin dose lower than 13 Gy (P = 0.017). At the last assessment, facial and trigeminal nerve function was preserved in 98.2% and 96.4% of patients, respectively; the only facial deficit (House-Brackmann grade III) occurred in a patient who received a tumor dose of 20 Gy early in our experience (1988). None of the patients treated with doses lower than 13 Gy experienced facial or trigeminal neuropathy. All patients continued their previous level of activity or employment after GK SRS. No patient developed a secondary radiation-related tumor. Our experience indicates that GK SRS is an effective management strategy for younger patients with vestibular schwannoma, most of whom have no additional cranial nerve dysfunction.Neurosurgery 09/2009; 65(2):294-300; discussion 300-1. · 2.53 Impact Factor
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ABSTRACT: To assess the nerve facial displacement by a vestibular schwannoma and nerve adhesion to tumor as predictive factors of facial function outcome. A prospective cohort study. Tertiary referral center. Ninety-six patients undergoing a solitary vestibular schwannoma surgery during 2005 were included. Data concerning tumor size (Stage 1, intracanalicular; Stage 2, < or =15 mm in the cerebellopontine angle [CPA]; Stage 3, 15-30 mm in the CPA; and Stage 4, >30 mm in the CPA), intraoperative facial nerve displacement (Type 1, anterior to the tumor; Type 2, anterior and superior to the tumor and separated from the cochlear nerve; Type 3, superior to the tumor; and Type 4, posterior to the tumor), degree of tumor adhesion (weak, intermediate, and strong) and postoperative facial function according to the House and Brackmann classification at days 10, 30, 90 and 180 were collected. A good facial function (Grade 1 or 2) was reported in 73% at postoperative Day 180. Univariate analysis showed that facial outcome was better in small tumors, in displacement Types 1 and 2 (Type 1, 46%; Type 2, 34%; and Type 3, 20%), and in tumors with weak and intermediate adhesion (weak, 10%; intermediate, 38%; and strong, 52%). Facial nerve displacement and adhesion were related to tumor stage. The combination of tumor stage, adhesion, and nerve displacement in a logistic regression model was highly predictive of postoperative facial function. Facial nerve displacement and nerve adhesion to tumor are significant predictive factors of facial function outcome after vestibular schwannoma surgery in addition to tumor size.Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 05/2009; 30(3):392-7. · 1.44 Impact Factor
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ABSTRACT: Vestibular schwannomas (VSs) are benign tumors arising from eighth cranial nerve and most often occur sporadically in individuals of middle age group. Sporadic VSs are rarely reported in the young population. In this study, we evaluated clinical behaviors of 12 young sporadic VSs by the statistical comparison with a matched series of 145 adult cases. We found that young tumors were characterized by an earlier onset of initial symptom, shorter duration from the first symptom to diagnosis, and larger tumor size than adult ones. Standard sequencing demonstrated the presence of NF2 mutations in eight tumors. All NF2 mutations identified were truncating mutations (nonsense, frameshift, and splicing-site mutations). Earlier formation of VSs in young patients was evidenced by the high incidence of NF2 mutations (66.7 %) far beyond our previous study in the adult case series (34.5 %). Furthermore, young tumors exhibited deficient merlin or heightened phosphorylated merlin that was subsequently demonstrated to be well correlated with increased tumor size. Finally, we compared protein levels of four pathogenesis-related molecules between young and adult group but there was no significant difference. These results led us to suggest that high frequency of NF2 mutations may play a critical role in early tumorigenesis of young VSs. Moreover, merlin deficiency or phosphorylation status of merlin was involved in their earlier development. Further study remains to fully understand the mechanism for the rapid growth of young VSs.Molecular and Cellular Biochemistry 03/2014; · 2.33 Impact Factor