Metastatic malignant peripheral nerve sheath tumor of the cauda equina

Malignant peripheral nerve sheath tumor (MPNST) of the cauda equina is rare, with only four reported patients in the literature. We present a 70-year-old woman with a history of left sciatic nerve MPNST that had been treated by left hemipelvectomy 4 years prior. She then presented with disabling right leg weakness that progressed over 7 months. Spine imaging revealed an intradural extramedullary lesion from L5 to S1 with compression of the cauda equina. She underwent an L3-S1 laminectomy with subtotal resection of the lesion, which was diagnosed as a metastatic MPNST. At the 3-month postoperative visit, her lower extremity strength had returned to baseline. The only notable postoperative complication was a superficial wound infection that resolved with antibiotic treatment and wet-to-dry dressing changes. Thus, although uncommon, the spinal canal and cauda equina should be considered as possible locations for metastatic MPNST in the appropriate clinical scenario. To our knowledge, this is the first report of a patient with a metastatic MPNST to the cauda equina.     

Spinal Leptomeningeal Hemangioblastomatosis in von Hippel-Lindau Disease: Magnetic Resonance and Pathological Findings

A 55–year–old man with von Hippel–Lindau disease presented with quadriparesis. Muitiple enhancing cervical and thoracic spinal masses were seen on magnetic resonance imaging (MRI). A rim of diffuse, nodular enhancement linking all of the discrete masses was apparent on the surface of the cervical and thoracic regions of the cord. Surgical exploration revealed multiple extramedullary–intradural and intramedullary masses, extending to and infiltrating the cord; the leptomeninges contained numerous small tumor seeds at several levels. The excised spinal masses were diagnosed as capillary hemangioblastomas, which infiltrated the pia mater. Diffuse, intense, spinal leptomeningeal enhancement on MRI associated with multiple hemangioblastomas has not been previously reported and may be referred to as spinal “leptomeningeal hemangioblastomatosis.”     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

Hemangioblastoma of the cauda equina

A very rare hemangioblastoma of the filum terminale with sciatic pain and partial cauda syndrome in a 36-year-old man is presented. The difference between solitary hemangioblastoma, hemangioblastomatosis and von Hippel-Lindau disease is stressed. The literature is reviewed and the diagnosis of cauda equina neoplasms is discussed. Even nowadays often the final diagnosis is made late and only after operation and microscopical tissue examination.     

Locally invasive giant spinal paraganglioma

Cauda equina paragangliomas are neuroendocrine tumours rarely encountered in neurosurgical practice. Large cauda equina paragangliomas with an intradural and extradural component, dense adhesion to nerve roots and high vascularity are surgically challenging and mandate meticulous operative dissection. The presence of extensive bony erosion can lead to spinal instability requiring solid instrumentation and fixation. We recommend resection of large cauda equina paragangliomas in a staged fashion with the aim of gross total resection and spinal stabilisation.     

Intradural Migration of a Sequestrated Lumbar Disc Fragment Masquerading as a Spinal Intradural Tumor

Intervertebral intradural lumbar disc herniation (ILDH) is a quite rare pathology, and isolated intradural lumbar disc herniation is even more rare. Magnetic resonance imaging (MRI) may not be able to reveal ILDHs, especially if MRI findings show an intact lumbar disc annulus and posterior longitudinal ligament. Here, we present an exceedingly rare case of an isolated IDLH that we initially misidentified as a spinal intradural tumor, in a 54-year-old man hospitalized with a 2-month history of back pain and right sciatica. Neurologic examination revealed a positive straight leg raise test on the right side, but he presented no other sensory, motor, or sphincter disturbances. A gadolinium-enhanced MRI revealed what we believed to be an intradural extramedullary tumor compressing the cauda equina leftward in the thecal sac, at the L2 vertebral level. The patient underwent total L2 laminectomy, and we extirpated the intradural mass under microscopic guidance. Histologic examination of the mass revealed a degenerated nucleus pulposus.     

Non-Dura Based Intaspinal Clear Cell Meningioma

A 34-year-old female patient was presented with leg and hip pain for 6 months as well as voiding difficulty for 1 year. Magnetic resonance imaging revealed a well-demarcated mass lesion at L2-3. The mass was hypo-intense on T1- and T2-weighted images with homogeneous gadolinium enhancement. Surgery was performed with the presumptive diagnosis of intradural extramedullary meningioma. Complete tumor removal was possible due to lack of dural adhesion of the tumor. Histologic diagnosis was clear cell meningioma, a rare and newly included World Health Organization classification of meningioma usually affecting younger patients. During postoperative 2 years, the patient has shown no evidence of recurrence. We report a rare case of cauda equina clear cell meningioma without any dural attachment.     

Clear Cell Ependymoma Occurring in the Cauda Equina

The authors present a rare case of clear cell ependymoma that developed in the cauda equina. A 54-year-old man was admitted to hospital with intermittent lower back pain. A neurological examination conducted on admission revealed no sensory or motor disturbance. Deep tendon reflexes in both lower extremities were normal. Magnetic resonance images demonstrated a 1.0 cm-sized intradural mass at the filum terminale. Gross total resection was performed via total laminectomy of L1 and L2. The tumor was confirmed to be clear cell ependymoma by histopathologic examination. His symptom was relieved after surgery.     

A long-segment string of bead- like schwannoma of cauda equina: a case report

Spinal schwannomas are one of the most common intradural extramedullary spinal tumors. However, a string of bead-like intradural extramedullary schwannoma is highly uncommon. In the paper we present a case of a long-segment string of bead-like intradural extramedullary schwannoma of cauda equina from L2 to S2.The patient had been suffering progressive low back pain and sciatica with progressive neurological deficits for three years. She was misdiagnosed as lumbar disc herniation at her first time of hospitalization after a simple lumbar computed tomography (CT) scanning and a discectomy. We found her tumor by magnetic resonance imaging (MRI) and totally removed it. We therefore emphasize differentiating this almost completely curable tumor from other lumbar diseases such as lumbar disc herniation. It is suggested that a reasonable and careful strategy of surgery for these tumors is important.     

Primary intradural extramedullary hydatidosis. Case report and review of the literature

The authors report a case of cauda equina compression by intradural hydatid cyst. An 18-year-old man presented with paraparesis and sphincter dysfunction. MRI showed an intradural cystic lesion extending from L1 to L2 with low signal intensity on T1 and high signal intensity on T2. The cyst was removed after laminectomy and opening of the dural sac. Histological and parasitic examinations confirmed a diagnosis of hydatid cyst. The patient improved progressively after surgery. The similar 22 cases of intradural extramedullary hydatid disease reported in the literature were reviewed. All spinal areas were involved, with a predilection for the thoracic region. Neurological complications were usual with rapid spinal cord compression in this rare form of hydatid disease. The treatment was by surgery with a favourable outcome compared to the classic hydatid cyst of the spine.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

Cauda equina compression syndrome in a patient affected by thalassemia intermedia: complete regression with blood transfusion therapy

Surgical excision followed by radiotherapy has been reported to be successful in the treatment of spinal cord compressions due to extramedullary hematopoiesis in patients affected by thalassemia. The authors report a case of cauda equina compression in such a patient successfully treated with repeated blood transfusions.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.     

巨大脊髓圆锥血管母细胞瘤一例并文献复习

目的 探讨脊髓血管母细胞瘤的诊断、治疗及预后.方法 对1例圆锥部位巨大血管母细胞瘤的诊治进行报告,并结合文献对脊髓血管母细胞瘤进行回顾分析.结果 脊髓血管母细胞瘤可以是单独发生,也可以是Von Hippel-Lindau病的系列疾病之一,临床发病率低,最常见症状是疼痛,MRI表现为瘤结节明显强化,周围可见异常血管流空影,多伴有空洞,DSA可显示染色的瘤结节、供血动脉及引流静脉,显微神经外科手术可完整全切.结论 显微神经外科手术是治疗脊髓血管母细胞瘤的最佳方法.对于有症状患者,应尽早手术,无症状患者可以定期复查MRI,如表现为肿物生长,可视为手术指证,患者预后较好.