Neurofibromatosis type 2

神经纤维瘤病 2 型
  • 文章类型: Systematic Review
    背景:前庭神经鞘瘤(VS)是桥小脑角最常见的肿瘤。放射学测试的更大可及性增加了其诊断。考虑到肿瘤的特点,病人的症状和年龄,已经提出了三种治疗策略:观察,手术或放疗。为每位患者选择最合适的药物是争议的常见来源材料和方法:本文包括与VS相关的详尽文献综述,可作为治疗这些病变患者的临床指南。演示文稿以临床医生通常会问自己的问题的形式进行定向,答案已由SEORL-CCC耳科委员会咨询的国家和国际专家小组撰写和/或审查。
    结果:已编制了一份清单,其中包含关于VS管理的13个最具争议的主题块,形式为50个问题,并通过系统的文献综述(1992年至2023年在PubMed和Cochrane图书馆发表的与每个主题领域有关的文章)寻求所有这些问题的答案。三十三位专家,由SEORL-CCC耳学委员会领导,分析和讨论了所有的答案。在附件1中,可以找到分为4个主题领域的14个其他问题。
    结论:本关于VS管理的临床实践指南为有关该肿瘤的最常见问题提供了一致的答案。缺乏足够的前瞻性研究意味着受试者的证据水平通常是中等或较低的。这一事实增加了专家编写的此类临床实践指南的兴趣。
    BACKGROUND: Vestibular schwannoma (VS) is the most common tumour of the cerebellopontine angle. The greater accessibility to radiological tests has increased its diagnosis. Taking into account the characteristics of the tumour, the symptoms and the age of the patient, three therapeutic strategies have been proposed: observation, surgery or radiotherapy. Choosing the most appropriate for each patient is a frequent source of controversy.
    METHODS: This paper includes an exhaustive literature review of issues related to VS that can serve as a clinical guide in the management of patients with these lesions. The presentation has been oriented in the form of questions that the clinician usually asks himself and the answers have been written and/or reviewed by a panel of national and international experts consulted by the Otology Commission of the SEORL-CCC.
    RESULTS: A list has been compiled containing the 13 most controversial thematic blocks on the management of VS in the form of 50 questions, and answers to all of them have been sought through a systematic literature review (articles published on PubMed and Cochrane Library between 1992 and 2023 related to each thematic area). Thirty-three experts, led by the Otology Committee of SEORL-CCC, have analyzed and discussed all the answers. In Annex 1, 14 additional questions divided into 4 thematic areas can be found.
    CONCLUSIONS: This clinical practice guideline on the management of VS offers agreed answers to the most common questions that are asked about this tumour. The absence of sufficient prospective studies means that the levels of evidence on the subject are generally medium or low. This fact increases the interest of this type of clinical practice guidelines prepared by experts.
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  • 文章类型: Journal Article
    What surgical approaches for vestibular schwannomas (VS) are best for complete resection and facial nerve (FN) preservation when serviceable hearing is present?
    There is insufficient evidence to support the superiority of either the middle fossa (MF) or the retrosigmoid (RS) approach for complete VS resection and FN preservation when serviceable hearing is present.
    Which surgical approach (RS or translabyrinthine [TL]) for VS is best for complete resection and FN preservation when serviceable hearing is not present?
    There is insufficient evidence to support the superiority of either the RS or the TL approach for complete VS resection and FN preservation when serviceable hearing is not present.
    Does VS size matter for facial and vestibulocochlear nerve preservation with surgical resection?
    Level 3: Patients with larger VS tumor size should be counseled about the greater than average risk of loss of serviceable hearing.
    Should small intracanalicular tumors (<1.5 cm) be surgically resected?
    There are insufficient data to support a firm recommendation that surgery be the primary treatment for this subclass of VSs.
    Is hearing preservation routinely possible with VS surgical resection when serviceable hearing is present?
    Level 3: Hearing preservation surgery via the MF or the RS approach may be attempted in patients with small tumor size (<1.5 cm) and good preoperative hearing.
    When should surgical resection be the initial treatment in patients with neurofibromatosis type 2 (NF2)?
    There is insufficient evidence that surgical resection should be the initial treatment in patients with NF2.
    Does a multidisciplinary team, consisting of neurosurgery and neurotology, provides the best outcomes of complete resection and facial/vestibulocochlear nerve preservation for patients undergoing resection of VSs?
    There is insufficient evidence to support stating that a multidisciplinary team, usually consisting of a neurosurgeon and a neurotologist, provides superior outcomes compared to either subspecialist working alone.
    Does a subtotal surgical resection of a VS followed by stereotactic radiosurgery (SRS) to the residual tumor provide comparable hearing and FN preservation to patients who undergo a complete surgical resection?
    There is insufficient evidence to support subtotal resection (STR) followed by SRS provides comparable hearing and FN preservation to patients who undergo a complete surgical resection.
    Does surgical resection of VS treat preoperative balance problems more effectively than SRS?
    There is insufficient evidence to support either surgical resection or SRS for treatment of preoperative balance problems.
    Does surgical resection of VS treat preoperative trigeminal neuralgia more effectively than SRS?
    Level 3: Surgical resection of VSs may be used to better relieve symptoms of trigeminal neuralgia than SRS.
    Is surgical resection of VSs more difficult (associated with higher facial neuropathies and STR rates) after initial treatment with SRS?
    Level 3: If microsurgical resection is necessary after SRS, it is recommended that patients be counseled that there is an increased likelihood of a STR and decreased FN function.  The full guideline can be found at: https://www.cns.org/guidelines/guidelines-management-patients-vestibular-schwannoma/chapter_8.
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