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.     

Malignant peripheral nerve sheath tumor of the cauda equina

Only one case of malignant peripheral nerve sheath tumor (MPNST) affecting the cauda equina region has been reported earlier. A 32-year-old male with congenital multiple subcutaneous swellings presented with low back pain, progressive paraparesis and bladder-bowel dysfunction. Magnetic resonance imaging (MRI) demonstrated a heterogenously enhancing intradural lesion at L2–L4. At operation, on opening the dura, multiple nodular, firm matted masses attached to the lumbosacral nerve roots were encountered. Peripheral lesions were partially excised. Histopathological exam revealed varied cellularity with necrosis and pleomorphic nuclei suggestive of MPNST. MRI features, pathophysiological characteristics and the literature are reviewed. Received: 2 February 2001 / Accepted in revised form: 13 June 2001     

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.     

Schwannoma maligno dorsal en reloj de arena embolizado e intervenido por abordaje posterior único

We report a case of a 41 years old patient complaining of chronic dorsalgia. MRI showed a well defined intradural extramedular dumbbell-shaped lesion, associated to a left paravertebral tumor at D5-D6 level. The tumor was embolizated prior to surgery. Following she underwent a D4-D6 laminotomy, left D5-D6 costotransversectomy and resection of the intracanal extradural part of the lesion with section of the left D5 nerve. Posteriorly, complete resection of the extracanal portion of the tumor was performed by a postero-lateral approach. The anatomopathologic diagnosis was a malignant schwannoma. After the surgery, the patient required adyuvant radiotherapic treatment. Malignant schwannoma is a very uncommon tumor which belongs to the malignant peripheral nerve sheath tumors (MPNST). It develops from Schwann cells. It is frequently associated with type 1 neurofibromatosis. MPNST usually present local recurrence and can metastatize.     

Calcifying pseudoneoplasm of the neuroaxis presenting with refractory seizures: Case report and literature review

Calcifying pseudoneoplasms of the neuraxis (CAPNON) are rare benign lesions that can arise anywhere within the central nervous system. The etiology of these lesions remains unknown and diagnosis is made on pathohistological analysis. We present the case of a 35-year-old male patient with a history of epilepsy since childhood who was evaluated for refractory seizures. MRI revealed a small lesion in the left-posterior temporal lobe suspected to be a cavernoma. A gross total resection of the lesion was achieved via a left temporal craniotomy and pathological analysis revealed CAPNON. At 6 months follow-up, the patient remained neurologically intact and his seizures had ceased.     

Spinal metastasis of occult lung carcinoma causing cauda equina syndrome.

Cauda equina syndrome (CES) may be caused by tumor, herniated disc, trauma and spinal infections. However, CES due to occult lung cancer has not been reported in the literature. A 50-year-old man presented with a subacute CES caused by an intradural metastasis of an adenocarcinoma of the lung to the lumbosacral cauda fibers. His lumbosacral magnetic resonance imaging (MRI), showed a well-demarcated, intradural extramedullary mass lesion resembling a neurinoma at the L4/5 level. The patient underwent an L4-L5 laminectomy. The operative findings were also suggestive of neurinoma with involvement of three nerve roots, and a well-demarcated tumor without infiltration into the subarachnoid space. Although the findings of the operation were suggestive of neurinoma, final pathological diagnosis revealed metastatic carcinoma. Immunohistochemistry revealed clear cell adenocarcinoma metastasis. Chest X-ray and high resolution contrasted pulmonary computed tomography were normal. Positron emission tomography (PET) showed a lung mass, at the left apex. The patient was treated with chemotherapy and post-operative spinal radiotherapy was also performed. The CES resolved after the operation and the patient was followed up for 2 years with no recurrence. MRI of intradural cauda equina metastasis may be similar to that of intradural nerve sheath tumor. Surgery and postoperative radiotherapy may be effective for the treatment of CES due to lung carcinoma. Definitive diagnosis is by histopathological examination with immunohistochemistry. If the primary cancer cannot be detected by conventional radiological techniques, PET may be helpful.     

Myxopapillary ependymoma of the temporal lobe--report of a rare case of temporal lobe epilepsy

Myxopapillary ependymomas are a benign variant of ependymomas, occurring almost exclusively in the cauda equina region. We report an extremely rare case of myxopapillary ependymoma located in the left anterior temporal lobe. A 22-year-old man is presented with intractable seizures of 2 years duration with no focal neurologic deficits. Imaging of the brain revealed a well-circumscribed heterogeneous mass in the left anterior temporal pole with no connection to the ventricles. Imaging of the spine was normal. The patient underwent surgical removal of the tumor and at follow-up 4 months after surgery, there was improvement in his memory and speech along with complete cessation of seizures. Microscopic examination revealed the tumor to be a myxopapillary ependymoma, further confirmed by histochemical and immunohistochemical stains. To the best of our knowledge, this is the first documentation of myxopapillary ependymoma at this location and consequently, the first case to clinically present as intractable temporal lobe epilepsy.     

Paragangliomas of the cauda equina: report of two cases

Extraadrenal paragangliomas involving the spinal cord are not common and usually take the compression of the cauda equine. Two cases of paraganglioma of the cauda equina with a different presentation are reported, and the clinical and histopathology findings of this tumor, as well as diagnosis, treatment and prognosis are review. We stress the importance of the high tumor vascularization form of intradural that can make impossible achieve a complete resection. Laminotomy and intraoperatory echography are very useful in the approach to intradural tumors, such as paraganglioma.     

Discrimination of a nerve fiber that is the origin of a cauda equina tumor using acetylcholinesterase staining

Spinal nerve sheath tumors are well known to typically originate from dorsal sensory nerve roots. However, it is difficult to anatomically identify the origin in the case of cauda equina tumors. In this study, we aimed to ascertain whether a cauda equina nerve root removed with a nerve sheath tumor was a motor nerve using acetylcholinesterase (AchE) staining. Nerve rootlet sections removed with tumors were stained for AchE using the AchE Rapid Staining Kit. Additionally, we performed intraoperative motor‐evoked potential (MEP) monitoring following either transcranial electrical stimulation (TES) or electrical stimulation of nerve rootlets. The muscular strength of the lower extremities was assessed bilaterally before and after surgery using manual muscle testing. An AchE‐positive motor nerve rootlet that was the origin of a cauda equina tumor was observed in one of the 12 patients. In this patient, a MEP in the right quadriceps evoked by electrical stimulation of this rootlet was detected. TES‐MEP showed a 30% decrease in the amplitude in the right quadriceps evoked after tumor resection with this nerve rootlet. However, the motor strength in both lower extremities did not change after surgery. AchE staining and intraoperative MEP monitoring could detect the motor nerve rootlet that was the origin of a cauda equina tumor. Nerve sheath tumors originating from the motor nerve might be rare even in cauda equina.     

Epidermoid tumors of the temporal lobe as epileptogenic foci

Intracerebral epidermoid tumors of the temporal lobe are exceedingly rare. The vast majority of these slow-growing tumors remain clinically silent while only a select few are associated with overt symptomatology. We report two patients with epidermoid tumors whose unique location in the temporal lobe resulted in secondarily generalized seizures. Our first patient, a 19-year-old female, presented with a secondarily generalized tonic–clonic seizure and MRI revealed a superior temporal lobe lesion that was subsequently treated with an awake craniotomy and gross total resection. Our second patient was a 71-year-old male with a recent seizure history and known left temporal lobe lesion. The patient underwent craniotomy for gross total resection of the mass. In these two patients, we found that MRI proved diagnostic and surgical resection was curative. Our clinical experience and review of the literature indicate that gross total resection of these lesions confers control of localization-related epilepsy.     

Cauda equina syndrome due to recurrent malignant lymphoma of the spinal cord. A case report]

A 50-year-old man noticed a mass in the right cervical region and presented to our hospital. He underwent biopsy of a cervical lymph node, which revealed non-Hodgkin's lymphoma (diffuse large B cell, lymphoblastic type) histologically. He was treated with chemotherapy (CHOP) and radiation, and achieved complete remission. Two months later, he was admitted because of distal pain and extensive numbness of the lower limb as well as weakness of the left leg. Lumbar MRI showed an area of abnormal intensity in the cauda equina. Cytological examination of cerebrospinal fluid showed class V (lymphoma cells), so he was diagnosed as having recurrent malignant lymphoma of the spinal cord. He was treated with intrathecal chemotherapy and irradiation. After the treatment the mass in the cauda equina disappeared and the neurological symptoms in his legs resolved. It is rare for malignant lymphoma to recur in the spinal cord, particularly the cauda equina. It is well known that cauda equina syndrome can be caused by vertebral lesions and primary spinal cord tumors, but it is also necessary to keep malignant lymphoma of the cauda equina in mind.     

A case of post-irradiation lumbosacral radiculopathy successfully treated with corticosteroid and warfarin]

A 33-year-old man underwent post-operative radiation therapy for the left testicular anaplastic seminoma. One year later, the patient developed muscle weakness and sensory disturbance in the left lower extremity, and muscle weakness in the right lower extremity. MRI demonstrated linear and focal gadolinium (Gd) enhancement of the anterior portion of the lumbosacral roots within the cauda equina. The neurological symptoms improved after administration of corticosteroid and warfarin. Radiation myelopathy of this type was classified as "selective anterior horn cell injury or amyotrophy" by Reagan, and the site of the lesion was considered to be the lower motor neurons. However, based on the clinical and MRI findings, we proposed that the disease process was injury to the spinal nerve roots rather than the lower motor neurons. Recent neuropathological studies of this syndrome have demonstrated degeneration of the proximal spinal nerve roots. We consider that primary lesions of this syndrome occur in spinal nerve roots rather than in lower motor neurons, and "lumbosacral radiculopathy" is a more appropriate term for this condition.     

Thoracic exophytic ependymoma masquerading as a benign extra-axial tumor

Spinal tumors are conventionally differentiated based on location in relation to the spinal cord. Benign spinal tumors such as schwannomas and meningiomas are typically extra-axial (intradural extramedullary) lesions, whereas more aggressive primary spinal tumors such as ependymomas are typically intramedullary masses. Rarely, ependymomas can have both intramedullary and extramedullary components (typically referred to as exophytic ependymomas). We report a case of a spinal exophytic ependymoma that radiographically masqueraded as a benign intradural extramedullary lesion causing cord compression and neurologic deficit in a 47-year-old man. The diagnosis of exophytic ependymoma was made intra-operatively, with resultant gross total resection of the extramedullary portion and subtotal resection of the intramedullary portion. Histopathological examination confirmed ependymoma with World Health Organization grade II/IV. Pre-operative suspicion of an exophytic ependymoma influences operative planning and clinical management. We review the literature and discuss clinical management strategies for these interesting spinal tumors.     

Malignant peripheral nerve sheath tumor of the spine after radiation therapy for Hodgkin's lymphoma

We report the development of a malignant peripheral nerve sheath tumor (MPNST) in 2 patients after irradiation for Hodgkin's lymphoma. Clinicians should be aware of this uncommon, but important fatal complication of radiation therapy. The first case is a 37-year-old man who was diagnosed with nodular sclerosing (NS) Hodgkin's lymphoma and underwent successful mantle radiation. He presented to our neurosurgery service with a left C6 radiculopathy 6 years later. The second case is a 30-year-old female diagnosed with NS Hodgkin's lymphoma. She did well with extensive radiotherapy until 5 years later when she developed severe right arm and chest pain secondary to recurrent lymphoma. After aggressive radio- and chemotherapy, she presented to the neurosurgery service with a right Horner's syndrome, right C6 radiculopathy, and weakness of her right triceps and wrist extensors. Both patients obtained magnetic resonance imaging revealing intradural extramedullary cervical nerve root associated mass lesions. Two years after radiation therapy for his Hodgkin's lymphoma, the first patient underwent a C6 laminectomy at an outside institution for resection of a benign neurofibroma. Four years later, he underwent a posterior C5-7 laminectomy with lateral mass plate fusion and partial excision of a recurrent mass diagnosed as a MPNST. The second patient underwent a C5-6 hemilaminectomy and partial resection of a tumor also pathologically consistent with MPNST. We present 2 case reports of patients who developed neurofibrosarcomatous tumors with malignant transformation after undergoing radiation therapy for Hodgkin's lymphoma. Despite prompt surgical resection, these tumors exhibited aggressive behavior. Numerous cases of soft tissue tumors have been described to arise in areas of prior radiation therapy; however, there have been rare reports of de novo MPNST after radiation therapy, especially in the setting of Hodgkin's lymphoma. Postirradiation MPNST should be considered in the differential diagnosis of a painful, enlarging mass in a previously irradiated area.     

A rare case of metastatic renal cell carcinoma resembling a nerve sheath tumor of the cauda equina.

We present a rare case of solitary metastasis to the cauda equina from the kidney. The patient was a 68-year-old man with a two-year history of low back pain. His past medical history revealed a renal cell carcinoma diagnosed seven years earlier. His lumbosacral MR imaging showed a well-demarcated, intradural extramedullary mass at the L3 level. He underwent an L2-4 laminectomy. The operative findings of the tumor quite resembled that of a nerve sheath tumor. It did not infiltrate into the subarachnoid space and involved only one spinal nerve. Pathology of the tumor was a metastasis of the renal cell carcinoma. Only 10 cases with such a metastasis to the cauda equina have been reported in the English literature. We added the 11th and reviewed the literature with reference to tumor pathologies, clinical findings and route of metastasis to the cauda equina.     

Paraganglioma of the sacral spinal canal

Paragangliomas are tumors arising in the paraganglia. Involvement of the spine is less common, and usually takes the form of intradural compression of the cauda equina. We report here a case of a 60-year-old man with recurrent and progressive pain of his sacral and perineal area, accompanied by occasional rod and perineal hypoesthesia on admission. He underwent laminectomies of the vertebral bodies S1 and S2, and an en bloc resection of the tumor. Postoperative histopathological examination revealed a paraganglioma. Postoperative staging showed no pathological abnormalities, and no tumor recurrence after one year. Even though rare, the paraganglioma of the sacral spinal canal should be considered in the differential diagnosis of tumors occurring in the spine.     

脊髓圆锥马尾区畸胎瘤合并终丝牵张型脊髓拴系综合征的手术治疗

目的 探讨脊髓圆锥马尾区畸胎瘤合并终丝牵张型脊髓拴系综合征（TCS）的临床特点、手术方法及其疗效。方法 回顾性分析2007年2月～2022年3月手术治疗的50例脊髓圆锥马尾区畸胎瘤合并终丝牵张型TCS的临床资料。结果 畸胎瘤内容物及囊性部分内壁剥离切除45例,囊壁次全切除加电灼5例;50例终丝均分离、切断。术后病理均为成熟囊性畸胎瘤及内终丝结构。术后随访6个月～14.5年,中位数75个月,按Hoffman脊髓功能评分,脊髓功能状态好转27例,不变22例,恶化1例;1例畸胎瘤内膜次全切除术后复发再次手术,其余49例未见肿瘤复发,无再拴系。结论 椎管内畸胎瘤多分布于脊髓圆锥马尾区,以慢性神经压迫为表现;对合并终丝牵张型TCS,一期行畸胎瘤切除术及终丝切断脊髓栓系松解术,疗效满意。     

Concurrent spinal nerve root schwannoma and meningioma mimicking single‐component schwannoma

We present a first case of concurrent tumors consisting of schwannoma and meningioma arising at the same spinal level in a patient without neurofibromatosis. A 49‐year‐old man without clinical evidence of neurofibromatosis presented with a 5‐month history of right neck pain. MRI demonstrated an extradural tumor involving the right‐sided C2 nerve root with a small intradural component. T1‐ and T2‐weighted and contrast‐enhanced MRI could not differentiate the intradural tumor as different from the extradural tumor. Total removal of the tumors was performed. No contiguity of the extradural tumor with the intradural tumor was seen. The intradural tumor attached strongly to the dura mater around the C2 nerve root exits. Intraoperative pathological diagnosis confirmed the extradural tumor as schwannoma and the intradural tumor as meningioma. We therefore thoroughly coagulated the dura mater adjacent to the intradural tumor and resected the dura mater around the nerve root exits together with the tumor. Pathological examination revealed that the resection edge of the extradural component consisted of a spinal nerve with thickened epineurium and was free of neoplastic cells. No schwannoma component was evident in the intradural tumor. No obvious transition thus existed between the extra‐ and intradural tumors. Distinguishing these tumors prior to surgery is critical for determining an optimal surgical plan, as schwannoma and meningioma require different surgical procedures. We therefore recommend a careful review of preoperative imaging with the possibility of concurrent tumors in mind.     

Invasive character of an intradural spinal meningioma in early childhood

The authors report the case of a 2-year-old baby girl with an intradural meningotheliomatous meningioma of the cauda equina which recurred three times. Despite four operative procedures and localized radiotherapy, the lesion kept on growing to reach the retroperitoneal space. Extensive laminectomy and associated radiotherapy were probably responsible for a spinal dislocation which had to be operated on. The child was left paraplegic with major bladder dysfunction after all procedures. This is the first reported case of well-documented malignant evolution of a spinal meningioma.