Wu Osmond C, Shammassian Berje H, Chugh Arunit J S, Harbhajanka Aparna, Kasliwal Manish K
Department of Neurological Surgery, University Hospitals Cleveland Medical Center, Cleveland, OH, USA.
Department of Pathology, University Hospitals Cleveland Medical Center, Cleveland, OH, USA.
Case Rep Neurol Med. 2020 Jan 27;2020:1792582. doi: 10.1155/2020/1792582. eCollection 2020.
Primary intradural malignant peripheral nerve sheath tumor (MPNST) is an extremely rare diagnosis and is associated with an extremely poor prognosis. A 77-year-old man diagnosed with an intradural MPNST, more than 40 years after radiation for a testicular seminoma, is reported. Intradural MPNSTs of the spine outside the setting of neurofibromatosis is extremely rare and can masquerade as common benign nerve sheath tumors, on imaging. An older age at presentation with short duration of symptoms and prior regional radiation treatment encompassing the spine in the treatment field regardless of remoteness should alert the oncologists and neurosurgeons to the possible existence of this rare and aggressive tumor, as the management, and overall prognosis of this tumor is distinctly different compared to the usual intradural spinal tumors.
原发性硬脊膜内恶性周围神经鞘瘤(MPNST)是一种极为罕见的诊断,且预后极差。本文报告了一名77岁男性,在接受睾丸精原细胞瘤放疗40多年后被诊断为硬脊膜内MPNST。在神经纤维瘤病背景之外的脊柱硬脊膜内MPNST极为罕见,在影像学上可能会伪装成常见的良性神经鞘瘤。出现症状时年龄较大、症状持续时间短以及既往放疗区域包括脊柱(无论时间间隔多久),这些情况应提醒肿瘤学家和神经外科医生注意这种罕见且侵袭性肿瘤的可能存在,因为该肿瘤的治疗和总体预后与常见的硬脊膜内脊柱肿瘤明显不同。