Malignant Transformation of Acoustic Neuroma/Vestibular Schwannoma 10 Years after Gamma Knife Stereotactic Radiosurgery
Malignant Transformation of Acoustic Neuroma/Vestibular Schwannoma 10 Years after Gamma Knife Stereotactic Radiosurgery
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DOI：
10.1055/s-0030-1253576
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发表时间：
2010-09-01
影响因子：
--
通讯作者：
Hardwidge, Carl
中科院分区：
文献类型：
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作者：
Demetriades, Andreas K.;Saunders, Nicholas;Hardwidge, Carl
仅报道了少数前庭蜗神经新发恶性肿瘤病例。更罕见的是先前组织学诊断的良性前庭神经鞘瘤恶变。我们介绍了一名年轻成年人的病例，他接受了手术/伽玛刀联合治疗良性前庭神经鞘瘤，并于 2 年后进行了进一步手术。他在最初诊断出面部麻木和共济失调 10 年后，MRI 显示肿瘤复发。根治性切除后，组织学显示恶变为恶性周围神经鞘瘤。 3个月内，脑干受压迅速、侵袭性复发，需要进一步手术进行脑干减压。组织学证实进一步去分化为间变性肉瘤。在等待放疗期间，肿瘤再次复发，患者死亡。该患者没有 2 型神经纤维瘤病的特征。文献中还有 13 例恶性前庭神经鞘瘤病例。只有六人接受了放射治疗，其中只有两人在放射治疗前进行了良性病变的组织学确认。他们都没有神经纤维瘤病。另外三例在放射手术后有恶性肿瘤的组织学证据，但没有放射治疗前的组织学证据；其中，两人神经纤维瘤病呈阳性。讨论了前庭神经鞘瘤的肿瘤生物学以及恶性转化背景下的放射生物学。
Only a handful of cases of de-novo malignancies of the vestibulocochlear nerve have been reported. Even rarer is the malignant transformation of a previously histologically diagnosed benign vestibular schwannoma. We present the case of a young adult who had combined operative/Gamma knife treatment for a benign vestibular schwannoma, followed by further surgery 2 years later. He represented 10 years after original diagnosis with facial numbness and ataxia, MRI showing gross tumor recurrence. After radical resection, histology showed malignant transformation to a malignant peripheral nerve sheath tumor. Within 3 months there was rapid, aggressive recurrence with brainstem compression, requiring further surgery for brainstem decompression. Histology confirmed further de-differentiation to an anaplastic sarcoma. While awaiting radiotherapy the tumor recurred again, the patient succumbing. The patient had no features of neurofibromatosis type 2. In the literature there are 13 other cases of malignant vestibular schwannomata. Only six had radiotherapy and of these only two had histological confirmation of a benign lesion preradiotherapy. Neither of these had neurofibromatosis. Three other cases had histological proof of malignancy postradiosurgery, but with no preradiotherapy histology; of these, two were positive for neurofibromatosis. The tumor biology of vestibular schwannomata as well as the radiobiology in the context of malignant transformation is discussed.