James Greg, Crocker Matthew, King Andrew, Bodi Istvan, Ibrahim Ahmed, Chitnavis Bhupal P
Department of Neurosurgery, Barts & The London National Health Service Trust, London, United Kingdom.
J Neurosurg Spine. 2008 Jun;8(6):567-73. doi: 10.3171/SPI/2008/8/6/567.
Malignant triton tumors (MTTs) are malignant peripheral nerve sheath tumors with rhabdomyosarcomatous differentiation. Malignant triton tumors affecting the spine are rare but present special challenges to the neurosurgeon. The authors report on 2 new cases of spinal MTTs, and analyze the 8 previously reported cases found via database search. The patients include a 15-year-old girl with thoracic MTT, and a 24-year-old man with lumbosacral MTT; both patients underwent radical resection with spinal stabilization. When these cases were combined with the literature results there was a male/female ratio of 6:4, and an age range of 15-67 years (median 37.5). Nine patients presented with symptoms related to the spinal cord, cauda equina, or nerve root compression. Four patients had undergone previous radiotherapy, and 2 had neurofibromatosis Type 1. Five cases of MTT were lumbosacral, 4 were thoracic, and 1 was cervical. Seven patients had intradural extension of tumor. All patients underwent some form of surgery, with 8 having total macroscopic excision. However, 6 patients required subsequent operations. In the 8 patients for which survival data was available, median time from diagnosis to death was 12 months (range 3-16). Malignant triton tumors are rare but should be included in the differential diagnosis of spinal tumors, particularly in patients who have undergone previous radiotherapy or who have neurofibromatosis. The authors suggest roles for radical surgery and multidisciplinary management.
恶性蝾螈瘤（MTTs）是具有横纹肌肉瘤分化的恶性周围神经鞘瘤。影响脊柱的恶性蝾螈瘤罕见，但给神经外科医生带来特殊挑战。作者报告了2例脊柱MTTs新病例，并通过数据库搜索分析了之前报告的8例病例。患者包括一名患有胸椎MTT的15岁女孩和一名患有腰骶部MTT的24岁男性；两名患者均接受了根治性切除并进行了脊柱稳定手术。将这些病例与文献结果相结合后，男女比例为6:4，年龄范围为15 - 67岁（中位数为37.5岁）。9例患者出现与脊髓、马尾或神经根受压相关的症状。4例患者曾接受过放疗，2例患有1型神经纤维瘤病。5例MTT位于腰骶部，4例位于胸部，1例位于颈部。7例患者肿瘤有硬膜内扩展。所有患者均接受了某种形式的手术，8例实现了肉眼下全切。然而，6例患者需要后续手术。在可获得生存数据的8例患者中，从诊断到死亡的中位时间为12个月（范围3 - 16个月）。恶性蝾螈瘤罕见，但在脊柱肿瘤的鉴别诊断中应予以考虑，特别是在曾接受过放疗或患有神经纤维瘤病的患者中。作者提出了根治性手术和多学科管理的作用。
