Yeole Ujwal, Rao K V L Narsinga, Beniwal Manish, Sivakoti Sumitra, Santosh Vani, Somanna Sampath
Neurosurgery Services, Department of Surgical Oncology, Tata Memorial Centre and Homi Bhabha National Institute, Mumbai, Maharashtra, India.
Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
J Neurosci Rural Pract. 2021 Sep 28;12(4):770-779. doi: 10.1055/s-0041-1735325. eCollection 2021 Oct.
Malignant peripheral nerve sheath tumor (MPNST) arises from nerve sheaths, mostly seen in peripheral nerves but rare in craniospinal nerves. The information available in the literature to build up treatment strategy and improve clinical outcomes is scarce. We are reviewing cases from our institute, with emphasis on radiological features for early differentiation from its benign variants. We analyzed pathologically diagnosed cases retrospectively from January 2007 to December 2018 at our institute. Clinicoradiological details and treatment parameters were collected from medical records for evaluation. Each case was contacted telephonically for final clinical follow-up at the time of writing the manuscript. A total of seven cases of MPNST were diagnosed in the last 10 years. It included four intracranial and three spinal cases. The mean age for the cohort was 34.3 years, with five females. We could achieve gross total resection (GTR) and subtotal resection in four (57.1%) and two (28.6%) cases, respectively. We could achieve an overall survival of 57.1% in the average follow-up of 28.2 months (range: 8-84 months). MPNST is a rare tumor with a bad prognosis. Radical surgical resection is the mainstay of the treatment, but it is not always possible to achieve it because of the inaccessible location and large size of lesions. Preoperative diagnosis is challenging; however, few radiological findings may give a clue toward it. As a disease entity overall, it has a poor outcome with a high rate of fatality.
恶性周围神经鞘膜瘤（MPNST）起源于神经鞘膜，多见于周围神经，而在颅脊神经中罕见。文献中可用于制定治疗策略和改善临床结局的信息匮乏。我们正在回顾我院的病例，重点关注其影像学特征，以便早期与良性变体进行鉴别。
我们回顾性分析了我院2007年1月至2018年12月病理诊断的病例。从病历中收集临床放射学细节和治疗参数进行评估。在撰写本文时，通过电话联系每个病例进行最终临床随访。
在过去10年中，共诊断出7例MPNST。其中包括4例颅内病例和3例脊髓病例。该队列的平均年龄为34.3岁，女性5例。我们分别在4例（57.1%）和2例（28.6%）病例中实现了全切除（GTR）和次全切除。在平均28.2个月（范围：8 - 84个月）的随访中，我们的总生存率为57.1%。
MPNST是一种罕见的肿瘤，预后不良。根治性手术切除是主要治疗方法，但由于病变位置难以到达和体积较大，并非总是能够实现。术前诊断具有挑战性；然而，一些影像学表现可能会提供线索。作为一种整体疾病实体，其预后较差，死亡率较高。
