PDF(Pubmed)
Abstract：
BACKGROUND: Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous syndrome that causes multiple central and peripheral nerve sheath tumors. People with NF1 have a 10% chance of developing malignant peripheral nerve sheath tumors (MPNSTs). Here we report a unique instance of a malignant schwannoma that has remained free of metastasis since its initial removal a decade ago. The malign schwannoma has been infrequently documented in the literature, and remarkably, no instances of such an extensive postoperative time without metastases have ever been described.
METHODS: A 46-year-old male patient with NF had multiple neurofibromas in different parts of his body, underwent surgery about 10 years ago (2013), and was diagnosed histopathologically as MPNST.
METHODS: He was admitted to our institution with a recurrent mass in the posterior third of the proximal thigh and severe pain radiating to the left lower extremity, which presented as sciatic pain (2021). A magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography examination revealed that the tumor was likely malignant.
METHODS: Surgical excision was performed.
RESULTS: A 10-year follow-up revealed no metastases or neurologic impairment.
CONCLUSIONS: When articles about benign schwannomas are placed in a separate category, little is written about NF-1-related malignant schwannomas of the sciatic nerve. MPNSTs are high-grade, aggressive sarcomas with a high risk of local recurrence (40%-65%) and metastasis to other body parts. Therefore, among the various benign peripheral nerve sheath tumors in NF-1 patients, the diagnosis of MPNST is crucial.Orthopedic surgeons should be aware that neurofibromas in NF-1 have a significant risk of developing MPNSTs. This study reports the successful treatment of a giant malignant sciatic nerve schwannoma with a long follow-up period without metastasis.
METHODS: A 46-year-old male patient with NF had multiple neurofibromas in different parts of his body, underwent surgery about 10 years ago (2013), and was diagnosed histopathologically as MPNST.
METHODS: He was admitted to our institution with a recurrent mass in the posterior third of the proximal thigh and severe pain radiating to the left lower extremity, which presented as sciatic pain (2021). A magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography examination revealed that the tumor was likely malignant.
METHODS: Surgical excision was performed.
RESULTS: A 10-year follow-up revealed no metastases or neurologic impairment.
CONCLUSIONS: When articles about benign schwannomas are placed in a separate category, little is written about NF-1-related malignant schwannomas of the sciatic nerve. MPNSTs are high-grade, aggressive sarcomas with a high risk of local recurrence (40%-65%) and metastasis to other body parts. Therefore, among the various benign peripheral nerve sheath tumors in NF-1 patients, the diagnosis of MPNST is crucial.Orthopedic surgeons should be aware that neurofibromas in NF-1 have a significant risk of developing MPNSTs. This study reports the successful treatment of a giant malignant sciatic nerve schwannoma with a long follow-up period without metastasis.
摘要：
背景：1型神经纤维瘤病（NF1）是一种常染色体显性遗传的神经皮肤综合征，可引起多发性中枢和外周神经鞘瘤。NF1患者有10％的机会发生恶性周围神经鞘瘤（MPNSTs）。在这里，我们报告了恶性神经鞘瘤的独特实例，自十年前首次切除以来一直没有转移。恶性神经鞘瘤在文献中很少有记载，值得注意的是，从未描述过如此广泛的术后时间没有转移的情况。
方法：一名46岁的男性NF患者在身体不同部位有多个神经纤维瘤，大约10年前（2013年）接受了手术，并在组织病理学上被诊断为MPNST。
方法：他因大腿近端后三分之一的肿块和严重的疼痛放射到左下肢而入院。表现为坐骨神经疼痛（2021年）。磁共振成像和氟脱氧葡萄糖-正电子发射断层扫描检查显示肿瘤可能是恶性的。
方法：进行手术切除。
结果：10年的随访显示无转移或神经损害。
结论：当关于良性神经鞘瘤的文章放在一个单独的类别中时，关于NF-1相关的坐骨神经恶性神经鞘瘤的报道很少。MPNST是高级的，侵袭性肉瘤具有高的局部复发风险（40％-65％）和转移到其他身体部位。因此,在NF-1患者的各种良性周围神经鞘瘤中，MPNST的诊断至关重要。骨科医生应该意识到NF-1中的神经纤维瘤具有发生MPNSTs的巨大风险。本研究报告成功治疗巨大的恶性坐骨神经神经鞘瘤，随访期长，无转移。
方法：一名46岁的男性NF患者在身体不同部位有多个神经纤维瘤，大约10年前（2013年）接受了手术，并在组织病理学上被诊断为MPNST。
方法：他因大腿近端后三分之一的肿块和严重的疼痛放射到左下肢而入院。表现为坐骨神经疼痛（2021年）。磁共振成像和氟脱氧葡萄糖-正电子发射断层扫描检查显示肿瘤可能是恶性的。
方法：进行手术切除。
结果：10年的随访显示无转移或神经损害。
结论：当关于良性神经鞘瘤的文章放在一个单独的类别中时，关于NF-1相关的坐骨神经恶性神经鞘瘤的报道很少。MPNST是高级的，侵袭性肉瘤具有高的局部复发风险（40％-65％）和转移到其他身体部位。因此,在NF-1患者的各种良性周围神经鞘瘤中，MPNST的诊断至关重要。骨科医生应该意识到NF-1中的神经纤维瘤具有发生MPNSTs的巨大风险。本研究报告成功治疗巨大的恶性坐骨神经神经鞘瘤，随访期长，无转移。
